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How common are brain tumours?

16th most common adult cancer, and 2nd most common children cancer



Presentation of a brain tumour?

symptoms of raise ICP: headache, reduced conscious level, nausea and vomiting

progressive neurological deficit and epilepsy


Features of a raised ICP headache?

worst on waking from sleep, increased by coughing, straining and bending forwards, can be relieved by vomiting


What is the cardinal physical sign or a brain tumour?



What causes papilloedema?

obstruction of venous return from the retina

caused by loss of crisp optic nerve head margins, venous engorgement, retinal oedema, haemorrhages, raise ICP, intraneuronal ischemic damagee


What types of focal neurological deficits are there?

motor, sensory, speech, visual, deafness, deteriorating memory, personality change


What type of tumours are focal siezures more common in?

motor, sensory and temporal lobe


Are primary or secondary brain tumours more common?



Treatment of secondary brain tumours?

surgery, radiotherapy, chemotherapy, best supportive care


When would surgery be considered for a brain tumour?

absent or controlled primary disease, younger than 75, good performance status


Where is the main origin for primary brain tumours to occur from?

glial cells


What is the main type of primary brain tumour?

astrocytoma (85-90%)
oligondendroglioma (5%)


What is WHOs grading for gliomas?

1 - pilocytic astrocytoma (paediatric tumour)
2 - benign premalignant tumour
3 - anaplastic astrocytoma
4 - glioblastoma multiforme (THE MOST COMMON PHENOTYPE)


If given enough time, what will all gliomas progress to?

glioblastoma multiforme (GBM) with the exception of pilocytic astrocytoma


What is the common pathway to a malignant glioma?

initial genetic error in glucose glycolysis, mutation of isocitrate dehydrogenase 1 (IDH1), resulting in excessive build up of 2-hydroxyglutarate, triggering genetic instability in glial cells and subsequent inappropriate mitosis


What is the less common pathway to a malignant glioma?

no IDH mutation, catastrophic genetic mutation, poor prognosis


Survival of GBM?

20% survive more than 18 months, even with aggressive therapy


Good prognostic factors for a glioblastoma?

under 50, aggressive surgical therapy, good performance post surgery, secondary GBM, MGMT mutatnt (as they means they should respond to chemo)


Bad prognostic factors for a glioblastoma?

over 50, poor neurological function post surgery, non radical surgery treatment, primary GBM, MGMT wild type as means they wont respond to chemo


Traditional vs updated glioblastoma therapy?

debulking surgery, radiotherapy

respective surgery, adjuvant chemo with temozolomide with RT, 6 cycles of teozolomide chemo


How does temozolomide chemo treat brain tumours?

a prodrug, activated by HCl, crosses the blood brain barrier and then methylates guanine in DNA to make replication impossible at the base site


Which tumour is resistant to Temozolomide?

MGMT, by reversing guanine methylation


When does Temozolomide treatment work best?

any age, if debulk first, if performance status if low, no wild type MGMT


What is WHOs performance status for any cancer patient?

1 - capable of light work
2 - self caring >50% of day
3 - limited self care


How does avastin treat brain tumours?

it Is an anti angiogenic agent which shuts down the tumours blood supply


Side effects of avastin?

hypertension, GI haemorrhage, expensive


How does gene therapy treat brain tumours?

inoculation of tumour with replication deficient HSV-1 retrovirus so wild type HSV-1 replicates exponentially and causes encephalitis

modified HSV-1 fails to replicate in normal brain but replicates rapidly in GBM, causing oncolysis and release of progeny virus

tumour cells killed and normal brain cells unaffected


How does dexamethasone treat brain tumours?

powerful synthetic steroid, given orally, improving brain performance, reduces tumour inflammation and oedema


In who is a benign glioma commonly seen?

in the young, rare over 45


What is the first symptoms of a benign glioma?



What causes detioration in a benign glioma?

tumour transformation into a malignant phenotype, progressive mass effect due to slow tumour growth, progressive neurological deficit from destructional tumour


Prognosis of an oligodenroglioma?

10-15yrs, can transform into anaplastic or GBM


Treatment for oligodendroglioma?



What genetic deficits are in an oligodendroglioma?

chromosomes 1 and 19, IDH1 mutation positive


What causes neurological deficit in brain tumours?

mass effect of tumour and surrounding cerebral oedema, deficit depends on tumour location


What are the most common focal neurological signs seen from raised ICP in brain tumours?

3rd and 6th cranial palsy from stretching of the nerves by downward displacement of the temporal lobes


DD of brain tumour?

intercranial mass lesions e.g. cerebral abscess, tuberculoma, subdural haematoma, intercranial haematoma, stroke, benign intracranial hypertension


Investigations of a brain tumour?

imaging with CT and MRI, MR angiography, PET scan, CXR for metastases, avoid LP


Risk factors of brain tumours?

ionising radiation, genetics, male, Africa, age


What is MS?

multiple sclerosis - an autoimmune, inflammatory, demyelinating disease specific to the CNS which causes chronic neurological disability


What is the mean onset of MS?

30 years


Pathophysiology behind MS?

inflammation in white matter of the CNS, producing antibodies against EBV nuclear antigens which then attack CNS myelin due to molecular mimicry, causes demyelinations and axonal loss, causing MS plaques


Risk factors for MS?

Caucasians due to less vit D, women, predisposition to EBV in children


What is relapsing remitting MS?

random attacks over a number of years, mainly in first 3-4 years, recovery varies between patients and between attacks, disabilities accumulate with each attack


What is chronic progressive MS?

slow decline in neurological functions either from the onset (primary) or following a relapse (secondary)


What is benign MS?

few relapses and little disability


Features of active MS?

demyelination (breakdown products present), variable oligodendrocyte loss, hypercellular plaque edge due to infiltration of tissue with inflammatory cells, perivenous inflammatory infiltrate, extensive blood brain barrier disruption, older active plaques have central gliosis


Features of inactive MS?

demyelination (breakdown products absent), variable oligodendrocyte loss, hypocellular plaque, variable infllamtory infiltrate, moderate to minor blood brain barrier disruption, plaques gliosed


Common sites and symptoms of MS?

cerebral hemispheres - large variety of symptoms

spinal cord - weakness, paraplegia, spasticity, tingling, numbeness Lhermitte's sign, bladder, sexual dysfunction

optic nerves - impaired vision and eye pain

medulla and pons - dysarthria, double vision, vertigo, nystagmus

cerebellar white matter - dysarthria, nystagmus, intention tremor, ataxia


Typical symptom of MS?

optic neuritis, spasticity, sensory symptoms and signs, Lhermittes sign, nystamus, double vision, vertigo, bladder and sexual dysfunction. gait


Atypical symptoms of MS?

aphasia, hemianopia, extrapyramidal movement disturbance, severe muscle wasting, muscle fasciculation


What are the first symptoms to present in MS?

weakness, parasthesiae and visual loss


What is the essential diagnostic criteria for MS?

two or more CNS lesions, disseminated in time and space, exclude other clinical conditions


Investigations for MS?

EP, MRI, CSF electrophoresis shows IgG oligoclonal bands, neurological examination, history, symptoms


DD for MS?

SLE, autoimmune disease, lyme disease, syphilis, AIDS, adrenomyeloneuropathy, cardiac embolic event, mitchochondrial encephalopathy


Treatment of MS?

IV methylprednisolone, SC B IFN


side effects of B IFN?

injection site reaction, flu like symptoms complex, mild intermittent lymphopenia, mild to moderate rises in liver enzymes, flu like symptoms


Treatment of spasticity in MS?

oral - baclofen, diazepam
peripheral never blocks e.g. phenol, alcohol, botox
intrathecal baclofen
functional neurosurgery
remove trigger factors e.g. UTI, bed sores


Treatment of tremor in MS?

BBs, low dose barbiturates, gabapentin, isoniazid, wrist bands containing small weights, computer controlled mechanical damping devices, stereotactic thalamotomy, thalamic electro stimulation


Treatment of sexual dysfunction in MS?

sexual counselling, intracorporeal inhection of PGE, penile prosthesis, Viagra, vibrators, lubricating gels, local anaesthetics,


Psychological effects of MS?

depression, anxiety, euphoria, pathological crying/laughing, cognitive dysfunction


Characteristics of fatigue specific to MS?

fundamentally different from ordinary fatigue, exacerbating by heat, improved by cool temperatures, makes other symptoms appear worse


The cause of pain in MS?

trigeminal neuralgia, painful tonic seizures, optic neuritis, vertebral compression fractures, ulnar and peroneal palsies, haemorrhagic cystitis, dysaesthic extremity pain, painful leg spasms, chronic joint and back pain


Characteristics of paroxysmal symptoms in MS?

brief duration, occur frequently, triggered by various factors, positive symptoms, usually respond to anti convulsant therapy


What is an impairment?

any loss of abnormality or psychological or anatomical structure or function


What is a disability?

any restriction or lack of ability to perform and activity in manner or within normal range


What is a handicap?

a disadvantage for a given individual resulting from an impairment or disability that limits or prevents the fulfilment of a role that is normal for that individual


What is neurological rehabilitation?

restoration of the individual to the highest feasible functional level physically, psychologically, socially, economically with the constraints of impairemtns and disabilities and within the context of cultural mileu


Wht is involved in MS physio?

special seating, moving ad handling techniques, passive stretching, standing and weight bearing, splints, serial casting


How does GABAb baclofen treat spasticity?

reduces calcium influx, suppresses release of excitatory neurotransmitters and post synaptic increase in potassium conductance


How does Tizanidine treat spasticity?

alpha2 agonist, peaks at 2 hours


CI of Tizanidine?

hepatic impairments


SE of Tizanidine?

dry mouth, hypotension, acute hepatitis, withdrawal, weakness


How does dantrolene treat spasticity?

causes muscle relaxtion by interfering wuth calcium influx


SE of dantrolene?

diarrhoea, avoid in liver disease


How does Botox treat spasticity?

relatively localised hypertonia,


How does THC (weed) treat MS?

CB1 presynaptic receptors inhibit calcium influx, inhibits glutamate release and activates potassium channels


What causes gait in MS?

weakness, foot drop, sensory ataxia, cerebellar ataxia, spasticity, drug side effects, fatigue


Treatment of gait in MS?

treat cause, physio, fall education, walking stick, orhoptics, functional electrical stimulation, famipridine, botox


How does dalfampridine treat MS?

prevents back propagation of action potentials, inhibits postassium channels so there is better conduction along demyelinated axons


What does detrusor overactivity cause?

frequency, urgency, incontinence


What does detrusor areflexia cause?

flaccid, large bladder


What does detrusor sphincter dysnergia cause?

incomplete bladder emptying


What does sphincter over activity cause?

retention and hesitancy


What does sphincter incompetence cause?



What is the diagnosis criteria called for MS?

McDonalds criteria


Which nerves are never affecting in MS?



What percent of strokes are caused by intracerebral haemorrhages



How does an intracerebral haemorrhage present?

sudden onset headache, drowsiness, vomiting, focal deficit


What is dementia?

describing symptoms of poor memory and difficulty learning


What causes dementia?

damage to brain cells, the most common cause being Alzheimer's


What is Alzheimer's?

a neurodegenerative disease, mainly in the cortex, causing plaques and tangles, leading to dementia


What does the Amyloid Precursor Protein do?

helps neurons in the brain grow and repair itself after an injury


What enzyme breaks down the APP and what does this lead to?

alpha-secretase and gamma-secretase, and the broken up protein parts are soluble and go away


What happens when beta-secretase and gamma-secretase breaks down APP and what does this lead to?

the left over fragment is not soluble and forms the monomer amyloid beta which are chemically sticky and bind together outside the neurons, leading to beta amyloid plaques which can get between neurons and inhibit neuron signalling so memory is impaired and can cause an autoimmune response, damaging surrounding neurons, can also deposit around blood vessels, lead to amyloid angiopathy, increasing risk of haemorrhage, rupture and blood loss


What 3 things can amyloid beta lead to in Alzheimer's?

beta amyloid plaques, autoimmune inflammation and amyloid angiopathy


What are tangles in Alzheimer's?

found inside the cell


What protein holds together neurons in the brain?

tau protein


What effect do beta amyloid plaques have

inhibit neuron signally and initiates activation of kinase in the neurons, which transfer phosphate groups to the tau protein so it changes shape and then the tau protein can no longer support the microtubules, clumps together with other tau proteins and gets tangled, leading to neruofibrially tangles


What do neurofibrially tangles lead to in Alzheimer's?

signals cannot be passed along the neurons in the brain and can lead to apoptosis leading to brain atrophy, narrow gyri, wider sulci and larger ventricles in brain


What are the two types of Alzheimer's?

sporadic and familial


What is sporadic Alzheimer's?

late onset, unknown cause, due to genetic and enviromental risk factors, the most common time, risk increased with age, linked to e3 allele of apolipoprotein E gene (APOE-e4), the risk increases if more of these genes were inherited


How common is sporadic Alzheimer's?

most common Alzheimer's

1% age 60-65
50% age 85


Function of apolipoprotein E?

breaks down beta amyloid


Why does APOE-e4 genes develop Alzheimer's?

the gene is less effective at breaking down beta amyloid, leading to increased plaques


What is familial Alzheimer's?

dominant gene speeds up progression, early onset, caused by

gene mutations in PSEN1 (c14) or PSEN2 (c1) gene are linked as they encode for presenilin 1 and 2 which are subunits of gamma secretase, so will change the location of APP splitting, increasing beta amyloid
Trisomy 21 (Downs) which has an extra copy of c21 which codes for APP gene which increase amyloid plaque build up


Symptoms of Alzheimer's?

can be undetectable in early stages then leads to short term memory, loss of motor skills, language is affected, then lose long term memory, until more disorientated and bedridden


What is the most common cause of death in Alzheimer's?

infection e.g. pneumonia


How do you diagnose Alzheimer's?

brain biopsy (after death) so must just exclude other causes of dementia for diagnosis


Treatment of Alzheimer's?

no cure, few medications which help and none that hault progression


Functions of the frontal lobe?

voluntary and planned motor behaviours, motor speech area, personality, planning


What happens in fronto temporal dementia?

personality and behaviour change followed by breakdown in attention and executive function (behavioural variant FTD)


Function of the temporal lobe?

hearing, language, semantic knowledge, memory, emotional/effective behaviour


What is HM amnesic?

severe epilepsy, profound failure to create new memories


Treatment of HM amnesic?

surgery to bilateral to remove medial temporal lobes including hippocampus


What is preclinical Alzheimer's?

amnesic prodrome which memory impairment, poor performance on episodic memory tests, cognitive function is preserved, scored on cognitive/memory screening, high risk of developing Alzheimer's


What is used to assess cognitive function?



What is involved in the 6CIT?

what year is it?
what month is it?
give an address with 5 parts?
count down from 20
say months of year in reverse
repeat address


definition of functional memory disorder?

>6 months
affect personal/professional life
psychosocial burden/psychological distress
verbal memory and attentional capacity >-1.5SD
absence of organic cause of cognitive memory
absence of major psychiatric disease (severe depression)


What is the criteria called for Alzheimer's?

consensus criteria Braak
1/2 = low likelihood
3/4 = intermediate likelihood
5/6 = high likelihood

diagnosis in intermediate of high likelihood of Alzheimer's are met and patient has clinical history of dementia


What condition is Alzheimer's linked to?

parkinson's disease


Investigations for Alzheimer's?

PET and SPECT, functional MRI


Management of Alzheimer's?

prevention, support, carers, advice, medications, socially active, cognitively active, control vascular risk factors, treat mood and anxiety


What are Caerphilly healthy behaviours for Alzheimer's?

no smoking, BMI 18-25, 3 or more portions of fruit/veg, and less than 30% of calories from fat, walking 2 or more miles each day, or vigorous exercise as a regular habit,


What medications can be used for Alzheimer's?

AtCh esterase inhibitors e.g. exelon, aricept, reminyl or memanrine (anti glutamate) or NMDA receptor antagonists e.g. namenda


Screening and staging instruments for mental status?

MMSE, mental state questionnaire, clinical dementia rating, global deterioration scale, ACE-R, CERAD, CAMDEX, ADAScog, MoCA


What are the 5 diffferent cognitive areas in the MMSE?

orientation, memory, attention, language, visual processing


What tests and questions are used for Alzheimer's testing?

address, date, birth, priminister, clock drawing test, fluency test or letters and design, how many animals can you name in one minute, card sorting test, stroop test, visualspatial memory and visuocontructive abilities, constructional apraxia, closing in phenomenon, £D visuoconstructive abilities, non verbal intelligence, abstract reasoning, verbal comprehension, spontaneous speech


What preventitive drugs can be used for Alzheimer's?

manage vascular risk, statins, antioxidants, anti inflammatory drugs, oestrogen, social interventions


What is epilepsy?

recurring, unpredictable siezures


What is a seizure?

when neurons are synchronously active in the brain, it is a paroxysmal event which changes behaviours, sensations and cognitive behaviour, caused by an abnormal burst of electrical impulses or too little inhibition


What happens in epilepsy with too much excitation?

fast of long lasting activation of NMDA which causes calcium to come in


What happens in epilepsy with too little inhibition?

There is too little GABA receptors so cannot inhibit signals


Potentially causes of epilesy?

primary, or secondary to tumours, brain injury and infection


What are common outward signs seen in epilepsy?

jerking (typically beginning in corner of mouth and thumb and spreading to the limbs), moving, losing consciousness, starts on one side and then moves to the other, can be followed by a period of complete paralysis


What are common inward symptoms seen in epilepsy?

fears, strange smells


What are the 2 types of seizures?

generalized or focal/partial - depends on how much of the brain is affected


What are the two types of partial seizure?

simple and complex


What happens in a simple parital seizure?

affects small part of brain
remain conscious
strange sensations and jerking movements
can be a warning that another seizure is coming
be aware that something is happening
remember the seizure
sense of deja vu


What is a Jacksonian march?

when movements start in on e group of muscles and spreads to other groups


What happens in a complex partial seizure?

impaired consciousness/impaired awareness
may not remember
may just be small movements e.g. smacking lips, rubbing hands, moving arms


What is Todd's paralysis?

the paralysis post seizure


DD for blackouts?

syncope, non epileptic seizure, epilepsy


What is a generalized seizure?

When both hemispheres are affected (secondary if starts as a partial seizure)


What is a tonic seizure?

muscles become stiff and flex, can lead to a fall backwards


What is an atonic seizure?

muscles become relaxed and can lead to a forward fall


What is a colonic seizure?

violent muscle contractions (convulsions)


What are tonic-clonic seizures?

most common generalized seizure, have a tense phase when the patient stiffens up and then the muscles start to rapidly contract


What are myoclonic seizures?

short muscle twitches, can be just a single twitch or can be lots


What are absence seizures?

patient loses consciousness and then quickly regains it, the patient may just look spaced out


What is status eplilepticus?

seizure >5 mins, or multiple seizures without returning to normal

normally tonic clonic type seizure

medical emergency


Treatment of status epilepticus?

acutely with benzodiazepines, o2, cardiorespiratory monitoring, vitamins IV


How do benzodiazepines treat epilepsy?

enhance effect of the inhibitor GABA


What symptoms follow seizures?

confusion (post ictal confusion), paralysis of arms and legs (normally on one side but can last up to 15 hours - Todds paralysis)


What causes Todd's paralysis?

temporary severe suppression of activity in the brain which was affected by the seziure


What investigations are done in epilepsy?

MRI, CT, EEG - to find anatomical abnormalities

evaluation of seizure and eye witness accoutns


Function of EEG?

detects electrical activity in the brain


Treatment of epilepsy?

anti convulsants based on the patient

epilepsy surgery if small part of the brain can be removed

nerve stimulation influences vagus nerve to influence neurotransmitter release to reduce frequency and severity of seizures

ketogenic diet so body burns fat not carbohydrates which produces ketone bodies which are used as an energy source by the brain instead of glucose

stress relievers, avoid triggers, avoid contact sports and swimming, and must be seizure free for a year to drive

deep brain stimulation implants electrode into specific areas of the brain, reducing abnormal electricity, reducing frequency of seizures


Who do absence seizures commonly affect?

children, can appear as a stop in conversation or daydream


How long do complex partial seizures last for?

around 2-3 minutes


Triggers of epilepsy?

flashing lights, CV disease, cerebral tumours, alcohol, post trauma, genetics


What anti epileptic drugs are used for focal seizures?

Lamotrigine and Carbamarzepine

pre synaptic and stop excitary signals


What anti epileptics drugs are used for generalized seizures?

lamotrigine and sodium valproate


Side effects of anti epileptic drugs?

memory problems, hepatic enzyme inhibition, weight gain, hair loss, rashes, leucopaenia


What is the dosage of anti epileptic drugs?

start low and gradually build up, until seizures stop or until you develop side effects, aim to achieve control with minimum dosage, and there is risk of intoxication which can lead to ataxia, nystagmus and dysarthria


When should to do surgery for epilepsy?

when only a single part of the brain is causing the seizures and it can be removed without causing any significant loss of brain function


Side effects of anti epileptic drugs?

it is a shock every 5 minutes for 30 seconds, hoarseness, sore throat and cough


Symptoms of non epileptic seizures?

pelvic thrusting, no tongue biting, eyes closed, crying/speaking, change in amplitude not frequency of activity


Side effects of DBS?

bleeding on the brain, depression, memory problems


What cause a non epileptic seizure?

mental process caused by psychosocial distress, and seizure instead of the emotion, it is situational and can last 1-20 minutes


What causes syncope?

fainting, changing behaviour sensation and cognitive processes, causing by insufficient blood of O2, from standing or sitting, lasting 5-30 seconds, has less warning and a history of heart disease


What is parkinsonism?

a triad of symptoms of tremor, rigidity/increased tone and bradykinseia


How common is Parkinson's?

0.3% of population over 40
7.5 million worldwide

more common in men

most common at 65


Sign and symptoms of Parkinson's?

tremors, rigidity, bradykinesia, postural instability

hypominia, dysphagia, hypophonia, blurred vision, eyelid opening apraxia, gait (shuffling, freezing)

reduced smell, constipation, visual hallucinations, frequency, urgency, dribbling of saliva, depression, dementia


What part of the brain is mainly affected in Parkinson's?

basal ganglia - which is responsible for muscle tone and ease of movemt


What makes up the basal ganglia?

caudate nucleus, caudate putamen, globus pallidus (interna and externa), nucleus accumbens, subthalamic nuclei, substantia nigra (2 parts)


Function of the basal ganglia?

cerebral cortex sends signals to the basal ganglia to initiate movement. The basal ganglia will control skeletal muscle tone and coordination of learned movements and then it will send signals back to the cortex (motor) via the thalamus to then be initiated down the spinal cord, out of the ventral horn to have normal movement pattern


How is the basal ganglia affected in Parkinson's?

problem with the output, so no normal controlled movement pattern


Function of substantia nigra?

has dopamine neurogic neurons which release dopamine into the striatum which then binds to dopamine 1 or 2 (excitory or inhibitory)


Pathophysiology of Parkinson's?

reduced dopamine in the substantia nigra leading to apoptosis or necrosis of dopaminergic neurons

caused by protein misfolding, aggregation and toxicity, defective proteolysis, mitchondrial dysfunction, oxidative stress

demyelinataion, neuronal loss and glyosis in substantia nigra, presence of Lewy bodies

lack of dopamine mean it cannot bind to GABA neuron so the inhibitory GABA is overstimulated causing glutamate secretion, causes excessive inhibitor input to the thalamus causing suppression of thalamocorticospinal pathway, so movement is not smooth


What are Lewy bodies?

round eosinophilic intra cytoplasmic occlusions in the nuclei of neruone, made up mainly of alpha-synciclein ubiquitin and other proteins


Risk factors of Parkinson's?

familial, GBA, CNCA, LRRK2, PARK2 and PINK1, pesticides, survivors of the encephalitis lethargica


Protective factors of Parkinson's?

smoking, coffee, vigorous exercising, NSAIDs


Treatment of Parkinson's?

Levodopa, dopamine agonists, monoamine oxidase B inhibitors, catechol-o-methyltransferase inhibitors, BBs, antichloinergics, physio, dietitian, language therapist, specialist nurse, counselling, surgery, DBS


How does levo-dopa treat Parkinson's?

it is a precursor to dopamine to improve symptoms


What does levo dopa need to be prescribed with and why?

a peripheral dopadecarboyxlase inhibitor e.g. benserazide to prevent it being converted to dopamine too early in the bloodstream, and reduces it peripheral side effects (nausea and compulsive behaviours)


How do dopamine agonists treat Parkinson's and what are the side effects?

act as a dopamine agonist and do not need to be converted in the body to become active, but risk of impulsive control disorders and excessive sleepiness


How do MAO-B inhibitors treat Parkinson's?

e.g. selegiline and rasagiline inhibit levodopa and dopamine breakdown so the effects last longer


How do COMT inhibitors treat Parkinson's?

prevent levodopa breakdown, so more of the dose can reach the brain


What is a myasthenia gravis?

chronic, acquired, autoimmune neuromuscular disease


Pathophysiology of myasthenia gravis?

autoantiboies to acetylcholine receptors (anti-AChR antibodies) at the post synaptic membrane, causing depletion of working post synaptic receptor sites and interfering with the neuromuscular junction

associated with HLAB8 and DR3, or induced by drugs e.g. penicillin


When does myasthenia gravis occur?

most common in young women under 40 and old men over 60

can be passed onto neonates

15/100,000 but is underdiagnosed


What diseases is myasthenia gravis associated with?

other autoimmune disease, thymic atrophy, tumour of SLE


Signs and symptoms of myasthenia gravis?

drooping eye, slurred speech, difficulty breathing, blurred/double vision, weakness in arms and legs, change in facial expression, unable to hold a steady gaze, difficulty chewing, weakness in proximal muscles, affect diaphram and resp troubles


What exacerbates and relieves myasthenia gravis?

exacerbated in exercise but relieved by rest


Causes of myasthenia gravis?

bone marrow transplant, or IFN-a medication,


Why is the thymus large at birth?

as it is responsible for immune response and the immune system is low at birth, it decreases in size as we get older


What size is the thymus in myasthenia gravis?

abnormal and large, can people can develop thymoma,


Investigations for myasthenia gravis?

examine eyes and muscles for signs of weakness
anti AChR positive or anti MuSK positive
CT and MRI look for thymoma
nerve conduction measured - fatigue will occur quicker in myasthenia gravis
single fibre EMG measure elecrtical potential form nerve to muscle, lower in myasthenia gravis
ice test, crushed ice in glove over eye for 3 minutes, which leads to imporved ptosis in myasthenia gravis


Treatment of myasthenia gravis?

increase sleep, avoid stress and excessive heat, rest, immunosuppressants e.g. prednisolone, acetylcholinesterae inhibitors inhibitors for temporary symptomatic relief by increasing ACh availiability (but beware of cholinergic crisis), osetoporosis prophylaxis, modified diet if difficultuy swallowing, thymectomy


What is myasthenia gravis cholinergic crisis?

weakness of the respiratory muscles during a relapse which is life threatening


Treatment of myasthenia gravis cholinergic crisis?

monitor FVC, ventillatory support, plasmapheresis, IV Ig, identify triggers and treat them


Prognosis of myasthenia gravis?

5% mortality with treatment
25-30% without treatment

inter current infection and hot weather aggravate it


What is Guillain Barre syndrome?

acute inflammtory demyelinating and occasional axonal polynaruopathy


Causes of Guillain Barre syndrome?

infection leading to shared epitopes with an antigen in the peripheral nerve tissue (ganglioside GM1 and GQ1b) leading to an autoantibody mediated nerve cell damage


triggers of Guillain Barre syndrome?

infection with campylobacter jejuni, EBVm CMV, post pregnancy (risk decreases during pregnancy), malignancies, vaccines


How common is Guillain Barre syndrome?

1/100,000 a year, everyone equally likely to get it, but more men than female had it and peak ages are 15-35 and 50-75


What is the first symptom in Guillain Barre syndrome?

paraesthesia and sensory loss in lower extremities


Symptoms of Guillain Barre syndrome?

ascending symmetrical weakness, neuropathic pain in legs and pain, reduced or absent reflexes early, reduced sweating, reduced heat tolerance, bladder weakness, postural hypertension, cardiac arrhythmias, urinary hesitancy


Complications of Guillain Barre syndrome?

can lead to paralysis, and can be severe if respiratory and facial muscles are involved which can cause respiratory fialure, hypo/hypertension, cardiac arrhythmia, urinary retention, ileus, pneumonia


DD for Guillain Barre syndrome?

stroke, brianstem compression, cord compression, poliomyelitis, myasthanthia gravis, hypokalaemia, vasculitis, botulism, encephalitis


Investigations for Guillain Barre syndrome?

high ADH secretion
LP - increased CSF protein
antibodies present
nerve conduction studies
MRI to exclude other causes


Management of Guillain Barre syndrome?

plasma exchange, IV Ig, corticosteroids, DVT prophylaxis, ITU for ventilation and intubation, pain relied, physio, nasogastirc feeding if swallowing problems, counselling


Prognosis of Guillain Barre syndrome?

good, but 10% die from resp failure, pulmonary emboli, infection or prolonged paralysis

poorer prognosis if old, rapid progression of symptoms, prolonged ventilation, preceding diarrhea, muscle dysfuction


What is the Miller Fisher syndrome?

variant of Guillain Barre syndrome that affects cranial nerves to the eye muscles and presents with opthamoplegia and ataxia


What are the different subtypes of Guillain Barre syndrome?

AIDP (95%)
AMSAN (GM1, GM1b and GD1a)
acute pandysautomina


What is giant cell arteritis?

aka temporal arteritis - granulomatous arteritis with inflammed medium and large arteries leading to occlusion o the lumen leading to ischemia of the optic nerve


In who does giant cell arteritis occur?

over 60s, related to polymyalgia rheumatic in 50% of cases


Symptoms of giant cell arteritis?

fever, fatigue, headache, SCALP PAIN, headache in front or on temples, JAW PAIN and claudication, diplopia, ptosis, blurred vision, amaurosis fugax, symptoms develop quickly, tenderness of temporal artery


What are the specific symptoms for giant cell arteritis?

jaw claudication and pain, scalp pain


Diagnosis of giant cell arteritis?

biopsy of temporal artery to confirm with GRANULOMATOUS CHANGES, elevated inflammatory markers, increased platelets and alkaline phosphate, decreased Hb, normonchromic, normocytic anaemia


Treatment of giant cell arteritis?

prednisolone immediately during biopsy, high dose methylprednisolone injection then prednisolone tablets, start high dose and then decrease, low dose aspirin, PPI to protect against stomach ulcers, immunosuppressants during prednisolone reduction, gastric and bone protection


Complications of giant cell arteritis?

irreversible bilateral visual loss, CV disease


What trigeminal neuralgia?

paroxysm of intense, sudden, stabbing pain in trigeminal never distribution from compression of the trigeminal nerve where it enters the brainstem, this pressure wears away at the myelin sheath causing uncontrollable pain


Symptoms of trigeminal neuralgia?

intense, sharp jabbing pain in forehead, cheek or jaw, dental pain, pain on one side of the face


Causes of trigeminal neuralgia?

MS, tumours on the nerve, compression of the nerve by a small artery or vein as it leaves the brain stem


What are the 3 branches of the trigeminal nerve and which is the least common to get trigeminal neuralgia?

mandibular, maxially and opthalamic (least common)

the pain is usually in 1 or 2 of the divisions


Triggers of trigeminal neuralgia?

washing the affected area, shaving, eating, talking, dentist, kissing, cold breeze, head movement, vibrations, hot or cold food, caffeine


DD of trigeminal neuralgia?

root canals


Investigations in trigeminal neuralgia?

rule out other conditions with MRI and history


Treatment of trigeminal neuralgia?

anticonvulsants e.g.carbamazipine to relieve nerve pain and slow down electrical impulses in the nerves and reducing the ability to transmit pain, it becomes less effective over time

gabapentin, phenytoin

thermocoagulation of the trigeminal ganglion or section of the sensory division

microvascular decompression to seoarate vessels from nerve root

stereotactic knife surgery - effective but has complications of facial numbness, hearing loss, stroke and death

injection of glycerol into the nerve


Side effects of carbamazipine?

tiredness, dizziness, confusion, nausea, double vision, leukopenia, depression


What is anesthesia dolorosa?

rare combination of numbeness and continuous pain in trigeminal neuralgia, it is virtually untreatable


What is the ganglion called where all 3 trigeminal nerve branches meet?

Gasserian ganglion


What is Huntington's disease?

neurodegenerative disease cauign atrophy and neruonal loss of striatum and cortex, leading to depletion in GABA and ACh, also increasing lenght of CAG triplet


What is the huntington gene and what chromosome is it on?

a increased in length of a cysteine-adenosine-guanine triplet repeat in the huntington gene on c4, the longer the repeat the earlier the onset of the disease


How common is Huntington's disease?

1/10,000, it is familial, autosomal dominant, 50:50 change of passing it on


Symptoms of Huntington's disease?

clumpsy, restless, muscle twitches and spasms, become more difficult to perform tasks, CHOREA

poor communications and starting conversations and undersatnd

poor attention spam and memory

depression, apathy, change in sexual behavior, anxiety, aggressive,


When does Huntington's disease usually present?

30-60years, 6% are juvinile though, people die 10-20 years after diagnosis


Pathophysiology of Huntington's disease and the 3 types?

a normal huntington gene has 20 or less CAG repeats but in Huntington's disease there are 3 types:

1 - 27-35 repeats
2 - 36-39 repeats
3 - over 40 repeats

all the repeats code for a mutant huntington gene with long sticky tail of glutamine which all stick together and form clumps which end up in the nucleus and is thought to disrupt signals and kills cells

the more repeats, the earlier the onset


Treatment of Huntington's disease?

no treatment so aim to relieve symptoms, healthy lifestyle, SSRIs for depression, speech and physical therapy, occupational therapy, benzodiazepienes for chorea, levodopa and dopamine agonists for bradykinesia and rigidity

genetic testing, counselling and education


Symptoms of juvenile Huntington's disease?

parkinsonian features, dystonia, pyramidal tract signs, dementia, epilepsy, mild or absent chorea, behavioural and learning difficulties


What is a transient ischemic attack?

a mini/warning stroke, normally last less than 5 minutes and symptoms resolved in 24 hours, caused by brief ischemia from a block blood vessel to the brain, it is not long enough to cause permanent damage


Risk factors of TIA?

age, FH, male, alcohol, hypertension, smoking, hyperlipidaemia, DM, obesity, high fat diet, stress, emoboli, south asian, african, caribbean


Pathophysiology of TIA?

emboli in carotid artery with existing stneosis and plaques, leads to impaired perfusion of the brain


Symptoms of TIA?

contralateral hemiparesis (weakness on opposite side of body), dysarthria (slurred sppech), vision problems, gait disturbance, carotid bruit from atherosclerosis, increased BP, heart murmur


Causes of TIA?

atherothromboembolism, caridoembolism, mural thrombus post MI or in AF, valve disease, prosthetic valve, hyperviscosity, polycythamia, sickle cell anemia,, leukostasis, myeloma, vasculitis


DD of TIA?

hypoglycaemia, migraine aura, focal epilepsy, hyperventilation


Tests for TIA?

carotid US, CT of head, test for hypertension , diabetes, cholesterol, ECG, eye exam, doppler and angiography


Treatment of TIA?

prevent stroke - antiplatelets, aspirin, warfarin, clopidogrel, statins for cholesterol, carotid endarterectomy to remove plaque and correct stenosis, intraoperative transcranial Doppler to monitor middle cerebral artery flow, healthy lifestyle, avoid driving for 1 month, antihypertensives, stop smoking and drinking


What is amarurosis fugax?

caused by an emboli passing into the retinal artery and causes a temporary loss of vision in one eye due to lack of blood flow to the retina


What is the risk of a stroke after TIA based on?

ABCD2 risk
>60(1), BP>140mmHG or 90mmHg diastole(1), unilater weakness(2), speech distubrance without weakness(1), >60mins(2), 15-59mins(1), diabetes(1)


What is meningitis?

an inflammation of the lepto meninges and underlying subarachnoid CSF


What are the most common pathogens for meningitis for kids, adults, elderly and neonates?

adults - strep. pneumoniae, neisseria meningitis, listeria monocytogenes

kids - listeria monocytogenes, pneumococcus, neisseria meningitis

neonates - group b strep, e.coli

elderly - listeria monocytogenes

mainly viral > bacterial > fungal

aerobic gram negative bacilli associated with trauma/neurosurgery


Risk factors for meningitis?

immunocompromised, C5-C9 deficiency, injections, VP shunts, splenectomy, transplants, in crowds, DM, alcoholic, cirrhosis, infection, surgery, itrathecal durgs, tumours, SLE,


Symptoms of meningitis?

fever, vomiting, severe headache, stiff neck, dislike of bright lights, seizures, rash, very sleepy/difficult to stay awake, confused/delirious, altered mental sate, non blanching petechial and purpric skin rash, cold hands and feet, papiloedema, Kernigs sign (inability to extend knee when hip is flexed), Brudzinski sign (spontaneous flexion of the hip with active flexion of the neck)


DD of stiff neck?

infections in head and neck e.g. pharygitis


What are latent viral infections?

VZV, HSV, HIV, priors


Toxin mediated diseases?

botulism, tietanus


Immune mediated disease?

Guillian Barre, post infection, ADEM, HLE


Investigations for meningitis?

CBC w/diff, Chemo10, 2 blood cultures, CT head no contrast (but dont do if no focal neurological findings), LP (but obtain CT first if needed to identify ICP) and CSF studies, gram stain, HSV PCR, cryptococcal Ag, TB PCR, fungal PCR


What are bacterial CSF results like?

cloudy/yellow, increasing opening pressure, increased protein, decreased glucose, increased WBC (PMN), gram positive


What are viral CSF results like?

cloudy/clear, gram negative, increased lymphocytes


What are fungal/TB CSF results like?

cloudy/straw, increased opening pressure, increased protein, gram negative, lymphocyes


Complications of meningitis?

hydrocephalus, abscess, cerebritis and cranial nerve lesions, thrombosis, infarct, ventriculitis and ventriculopathy, extra axial collections, seizures, DIC, septic shock, SIADH, decreased hearing


DD of meningitis?

malaria, encephalitis, septicaemia, subarachnoid haemorrhage (have blood in csf), dengue, tetanus


Management of viral meningitis?

IMMEDIATE ANTIBIOTICS (55 or immunocompromised = cefotaxime and amoxicillin due to listeria risk) - they must be able to pass the blood brain barrier

aciclovir is encephalitis suspected

IV fluids and inotropes if septicaemia

TB meningitis treated for 9 months

dexamethasone if pneumococcal menigitis

vancomycin added if travelled to area of increased pneuomococcal resistance

ganciclovir for CMV infectiosn

corticosteroids if hearing loss and neurological sequelae

benzylpenicillin if penicillin sensitive organiism


Which organism has the poorest prognosis in meningitis?

pneuomococcal meningitis


What is shingles?

a skin rash caused by infection of the herpes zoster virus (also causes chicken pox)


Symptoms of shingles?

pain that precede the rash and rash in a cluster or band, headache, burning/itching in affected skin, fever


Cause of shingles?

when the herpes zoster virus becomes reactivated, it normally is dormant, but become reactivated during lowered immunity during stress,


How does the shingles rashs progress?

initial red blotches on the skin, just develops into itchy blisters which contain the virus, eventually they become yellow, flatten and dry out, leaving a scab


What is ophthalmic shingles?

when it affects one of the eyes when the reactivated virus is in the trigeminal nerve, causing rash over forehead, nose, eye, conjunctivitis, red eye and vision problems


How to prevent spread of shingles?

not sharing towels, dont swim or play contact sport, avoid work if rash is weeping and cannot be covered, avoid pregnant women who haven't had it yet, avoid immunocompromised


Treatment of shingles?

antiviral medications e.g.acyclovir, preferably become blisters appear

prednisolone, analgesics, cool wet compresses

zinc containing ointment to dry the blisters

loose fitting clothing

anticonvulsants relieve nerve pain


side effects of antivirals?

decreased GFR, vomiting, encepahlopathy, urtcaria, nausea, abdominal pain, dizziness


Complications of shingles?

postherpetic neuralgia (severe nerve pain) and intense itching even after rash is gone, pain is in affected dermatomes, can last years and it is intolerable

opthalamic shingles, ramsay hunt syndrome, infection, scarring,


At what age are you likely to get shingles?



What is encephalitis?

inflammation of the brain normally due to an infection which can lead to brain damage, swelling, pressure and death


Risk factors of encephalitis?

exposure to viruses


In who is encephalitis most common?

young or elderly


What are the most common viruses to cause encephalitis?

herpes simplex virus, varicella zoster virus, CMV, EBV, HIV, measles, mumps, rabies, japanese B encephalitis, west nile tick, tick borne encephalitis


What are non viral causes of encephalitis?

bacterial meningitis, TB, thyphus, malaria, listeeria, Lyme disease, post vaccination, autoimmune ecephalitis, legionella, cryptococcus, vaccination, sphyllis, roundworm


Symptoms of encephalitis?

fever, headache, nausea, vomiting, joint pain, seizures, focal neurological deficits, confusion, change in personality, photophobia, hallucinations


what are the 3 types of chronic encephalitis?

subacute sclerosing panencephalitis, progressive multifocal leukodystrophy, chronic progressive HIV encephalitis


Investigations for encephalitis?

LP - increased CSF protein and lymphocytes, decreased glucose
culture and PCR of HSV within CSF
increased mononuclear cells
EEG - shows diffuse abnormalities
CT contrast - focal bilateral temporal lobe


Treatment of encephalitis?

acyclovir within 30 mins for 14days (21days if immunocompromised), supportive therapy, antibiotics, immunoglobulin therapy, plasmapheresis, corticosteroids if autoimmune, sedatives for irritability, physical/speech therapy, immunosuppressants, symptomatic treatment


Prognosis of encephalitis?

70% mortality in untreated, 20% mortality in treated

normally lasts for 1-2 weeks, but can take several months to revocer

probable reactivation


complications of encephalitis?

permanent brain damage, affecting hearing, memory, msucle control, sensation, speech and vision, personality changes, epilepsy


What is rabies and how is it transmitted?

the rhabdovirus RNA virus, transmitted by saliva of animals, which spreads to the CNS via peripheral nerves and forms Negn bodies, it replicates in the grey matter then trans-located to tissues and salivary glands


Symptoms of rabies at each stage?

prodomal stage - fever, jeadache, malaise, hyperaethesia at bite site
encephalitis - excitation, hallucinations, lucent period
brain stem dysfunction, cranial never involvement, salivation, hydrophobia


Diagnosis of rabies?

biopsy nape of neck or corneal impression smear of fluorescent antibody, salivary viral culture, PCR investigated, non specific serology


Treatment of rabies?

exposure prophylaxis, local wound cleaning, vaccine,


What is polio and how is it transmitted?

viraemia, types 1, 2 and 3, transmitted by faecal oral route, crosses the blood brain barrier causing meningocephalitis with destruction of motor nuceli of anterior horn of brain stem and spinal cord


What complications can polio lead to?

paralytic poliomeylitis, asymeme flaccid paralysis, respiratory muscle involvement


Treatment and prevention of polio?

vaccine with life attenuated types 1, 2 and 3, inactivated polio vaccine, 3 doses, boosters


What is cerebral toxiplasma, its symptoms and treatment?

reactivation of latent infection, seroprevalence rates 15-50%, CD4 usuallu


Treatment of CMV?



What is tetanus?

clostridium tetani, a soil organism infecting via dirty wounds, terminal spore, gram positive anaerobe, ubigulitis, toxin secretor (tetanospasmin) and translocates via the peripheral nerve, binds to GABA receptors of inhibitory interneurone


Symptoms of tetanus?

parasthesia of wound, lock jaw, sustained muscle contractins, involvement of facial muscles, paraoxysmal generalised spasm, autoimmune dysfunction


Diagnosis of tetanus?

debride wound, benzyle penicillin, human tetanus immunoglobulin, control spasm with dantolen, baclofen, control hypertension, ITU, airway mangement, tetanus toxin vaccination, 3 doses, signle booster


What is botulism?

clostridium botulism, spore former, anaerobe, e.g. cans, a neurotoxin seen in IVDU with contaminated heroin, incubation of 18-36 hours


Symptoms of botulism?

descending paralysis with cranial neuorpathy first, diplopia, dysrathria, dysphagia peripheral weakeness


Treatment of botulism?

equine of human antitoxin


Cord compression symptoms?

acute lower back pain precedes then neurological deficits, neuropathic pain, motor and autonomic dysfunction e.g. sensory loss below lesions (one or 2 segments below), tone and reflexes reduced, leg weakness and sensory loss, arm weakness is less severe


Investigations for cord compression?

MRI - show site of compression
CXR - shows degenerative bone disease and destruction of vertebrae by infection of neoplasm
biopsy and surgical exploration
serology, ESR, FBC, syphillis serology, serum electrophoresis


What is cord compression linked to and why?

cancer patients do to the rich venous plexus which increases haematogenous spread to metastases to the spine


Red flag symptoms for cord compression needing urgent referral and why?

bladder dysfunction, sphincter disturbance, saddle anesthesia (numbess to perineal area), bilateral lower extremity weakness

can lead to complete paralysis


Treatment of spinal cord compression?

malignancy - dexamethasone IV to reduce oedema around lesion and improve outcome before radiotherpay and chemo with decompressive laminectomy
epidural abscesses surgically decompression
anti inflammatories


What is the cauda equina?

it is the nerve roots caudal to the spinal cord temination, provides sensory and motor innervation to the hip muscles, lower extremity muscles, urinary and anal sphincters and muscles for sexual function


What is cauda equina syndrome?

compression of the spinal cord or spinal damage from L2 down,


What is the most common cause of cauda equina syndrome?

lumbar disc prolapse from lumbar disc herniation at L4/l5 and L5/S1


Causes of cauda equina syndrome?

disc herniation, lumbar stenosis, trauma, neoplasm, infection, abscess, inflammatory disease, CIDP, pagets, ankylosing spondylitis, post operative haematoma, spondylolisthesis, sarcoidosis, congenital lumbar disease


Symptoms of cauda equina syndrome?

depend on where the lesion is compressing the nerves - weakness, anesthsia/sensory deficiency, pain, paraesthesias, loss of sphincter tone resulting in incontinence, sexual problams, fasciculations, reduced of absent DTRs, saddle numbness, back pain and radicular pain down the legs, sexual dysfunction, reduced cremasteric reflex, reduced sensation over particular dermatomes


Investigations for cauda equina syndrome?

MRI to determine level of compression
CSF analysis if meningismal
Myelography and CT
urodynamic studies to monitor recovery of bladder function post decompression studies


DD of cauda equina syndrome?

conus medullaris syndrome, mechanical lower back pain, prolapsed lumbar discm fracture of lumbar verterbraw due to trauma, spinal tumour, spinal cord compression, peripheral neuropathy, guillain barre syndrome, amyoropjhic lateral sclerosis, MS, diabetic neuropathy, spinal neoplasm


Risk factors for cauda equina syndrome?

age, cancer patient, trauma,


Treatment of cauda equina syndrome?

based on underlying cause, referred to specialist, surgical intervention for trauma, rehabilitation, anti inflammatories, physio, chemo and radiotherapy, lesion debulking for space occupying lesions


Complications of cauda equina syndrome?

paralysis, sensory abnormalities, bladder bowel and sexual dysfunction,


prognosis of cauda equina syndrome?

based on cause, but poor treatment leads to long term bladder, bowel and sexual dysfunction, and late diagnosis leads to neurological deficit, poorer diagnosis if bilateral sciatica or complete perianal anesthesia than if it was just unilateral pain.


What is motor neuron disease?

cluster of degenerative disease with selective loss of neurons in the motor cortex, cranial nerve nuclei and anterior horn cells, upper and low motor neurons are affect but no sesnory loss of sphincter disturbance


Difference between MS and MND?

MS has sensory and motor loss and causing sphincter disturbance and MDN only affects motor and no distubrance


Difference between mysasnthia gravis and MND?

MND never affects eye movements unlike MG


What are the 4 clinical types of MND?

1 - progressive muscular atrophy - LMN lesion, causing weakness, wasting and fasciculation (spontaneous, irregular and brief contractions of part of a muscle) in hands and arms, affects distal first

2 - amyotrophic lateral sclerosis - combination of lateral corticospinal tracts and anterior horn cells producing progressive spastic tetraparesis or paraperesis UMN signs with added LMN signs

3 - progressive bulbar and pseudobulbar palsy - from destruction of UMN and LMN in lower cranial nerves with dysarthria, dysphagia, wasting and fasciculation of the tongue, cranial nerves 9-12

4 - primary lateral sclerosis - rare with progressive tetraparesis, loss of Betz cells in motor cortex so mainly UMN signs with spastic leg weakness and pseudobulbar palsy, no cognitive decline


Cause of MND?

5-10% familial, trauma, electric shocks, infection

events at the ALD-FTD locus explain 87% of familial ALS and is autosomal dominant


How common is MND?

5/100,000 and rare below 30, increasingly common over 50, often fatal within 2-4years


What symptoms are linked to a worse prognosis in ALS MND?

bulbar onset, increased in age, decreased FVC, split hand sign with thumb adrift due to wasting around it


What are UMN signs in MND?

spasticity, brisk reflexes, increased plantars


What are LMN signs in MND?

signs, wasting, fasciculation on tongue, abdomen, back, thigh


Investigations of MND?

diagnosis is clinical, fasciculation is characteritic

EMG shows muscle denervation

MRI excludes structural causes

LP excludes inflammatory causes


DD of MND?

cervical spine lesion, idiopathic multifocal motor neuropathy


Treatment of MND?

Riluzole - a sodium channel blocker that inhibts glutamate release, slow progression slightly

propantheline and amitriptyline for drooling

ventilatory support helps prolong survival and for resp failure

feeding via PEG helps dysphagia

spasticity drugs

neurlogists, dietitian, physio, speech therapist, palliative care


SE of Riluzole?

vomiting, increased pulse, somnolence, headache, vertigo and increased LFTs


What is a spinal muscular atrophy?

rare disorder which detroys anterior horn cells of the spinal cord, slowly progressive with symmetrical wasting weakness of the limbs


What are the 3 types of peripheral neuropathy?

sensory (affects touch, temperatire, pain and other sensations), motor (affects movement) and autonomic (affects involuntary bodily processes

can have a combination of them at one time e.g. sensorimotor polyneuropathy


What is peripheral neuropathy?

group of conditions in which the periopheral nervous system is damaged


Symptoms of sensory neuropathy?

tingling in the affected body part, numbeness, reduced feeling of pain and changes in temperature (feet), burning/sharp pain in feet and legs, allodynia (pain from light touch), loss of balance or coordination


Symptoms of motor neuropathy?

twitching, muscle cramps, muscle weakness and paralysis, muscle wasting, foot drop


Symptoms of autonomic neuropathy?

depends on location, constipation, diarrhea, nausea, bloating, hypotension, tachycardia, excessive or lack of sweating, sexual dysfunction, bowel incontinence, dysphagia


What is a mononeuropathy?

affects a single nerve caused by acute compression, particularly where the nerves are exposed anatomically or entrapment where the nerve passes through a relatively tight anatomical passage (carpal tunnel) or caused by direct damage


what is the most common form of mononeuropathy?

carpal tunnel syndrome, leading to tingling, pain or numbness in the fingers due to irritation of the median nerve


What is mononeuritis multiplex?

a systemic disorder, when 2 or more peripheral nerves are affected and it is a systemic cause


Symptoms of carpal tunnel syndrome?

tingling, numbness and pin i the thumb, index finger, middle finger and half of ring finger

discomfort in hand and arm, paraesthesia in hand, dry skin, swelling, hypoaesthesiia, atrophy of muscles, worse pain on repetitive use or in same position, impaired dexterity, drop things a lot, Tinnel's sign


Risk factors for carpal tunnel syndrome?

25% FH, pregnancy, menopause, injuries to wrist, manual labour, playing an intrument, diabetes, arthritis, hypothyroidism, obesity in the young, breast cancer medications, abnormal wrist structure


Investigations of carpal tunnel syndrome?

assess ability of hand and arm, look for weakness and wasting around thumb, Tinnel's sign, flexing wrist or holding above head will cause pain, nerve conduction study, EMG, x-ray, US of median nerve


Treatment of carpal tunnel syndrome?

avoid triggers, treat underlying cause, wrist splint to prevent bending of wrist which can aggrevate symptoms, no direct pressure on it, corticosteroids for inflammation (tablets of injection into wrist), carpal tunnel decompression surgery


Complications of carpal tunnel syndrome and surgery?

infection, recurring pain, scarring, nerve injury, post op bleeding, failure to separate root of carpel tunnel resulting in persistent symptoms


What is a polyneuropathy?

symmetrical disease process, can be sensory, motor or autonomic, and leads to symptoms of numbness, tingling, pins and needles, pain in extremities, unsteadiness of feet, weakness, postural hypotension, urinary retention, erectile dysfunction, diarrhea, dimished sweating, impaired pupillary reponses, cardiac arrhythmias


Causes of polyneuropathy?

DM, guillain barre sundrome, toxins, vitamin deficiency, leprosy, renal failure


Investigations in polyneuropathy?

FBC, ESR, serum vit b12, glucose, urea, electrolytes, LFTs, nerve conduction tests


Cause of vitamin B1 deficiency neuropathy (thiamin)?

chronic alcohol dependent patients when little food is consumed, starvation, beriberi


Symptoms of vitamin B1 deficiency neuropathy?

Wernicke Korsakoff syndrome, polyneuropathy and cardiac failure


Treatment of vitamin B1 deficiency neuropathy?

thiamin for 3 days, anaphylaxis in parenteral thiamin, given before IV glucose in high risk patients


Causes and treatment of vitamin E6 deficiency neuropathy (pyridoxine)?

isoniazid therapy for TB, give prophylactic pyridoxine with isoniazid, it mainly cuases a sensory neuropathy


Symptoms of vitamin B12 deficiency neuropathy?

polyneuropathy with subacute combined degeneration of the cord, distal sensory loss, absent ankle jerks, evidence of cord disease


Treatment of vitamin B12 deficiency neuropathy?

IM vit B12 to reverse peripheral nerve damage but has little affect on the CNS


What is hereditary sensorimotor neuropathy?

large and complex group with variable mutations, most common being Charcit-Marie-Tooth disease (peroneal muscular atrophy)


Symptoms of hereditary sensorimotor neuropathy?

distal limb wasting and weakness that progresses over many years, mostly in legs with variable loss of sensation and reflexes, legs can resemble inverted champagne bottles due to the wasting


What are the 2 types of diabetic neuropathy?

progressive and reversible


What are the 2 types of progressive diabetic neuropathy?

symmetrical sensory polyneuropathy and autonomic neuropathy


What are the 3 types of reversible diabetic neuropathy?

acute painful neruopathy, mononeuropathy and mononeuritis multiplex with cranial nerve or isolated peripheral never lesions, diabetic amyotrophy


What causes isolated mononeuropathies?

occlusion of the vase nervorum (small arteries that supply the peripheral nerves)


Where do diffuse neuropathies occur from?

accumulation of fructose and sorbitol (metabolized from glucose in the peripheral nerve) which disrupts the structure and function of the nerve


What is the most common form of neuropathy?

symmetrical mainly sensory neuropathy


Symptoms of symmetrical mainly sensory neuropathy?

affects the feet first, loss of vibration sense, pain sensation and temperature sensation in the feet, then 'walking on cotton wool' and lose balance due to impaired proprioception, hand involvement is uncommon.


Complications of symmetrical mainly sensory neuropathy?

trauma, blistering from ill fitting shoes causing ulceration, abnormal mechanical stress, repeated minor trauma, neuropathic arthropathy (Charcot's joints) in ankle and knee, distorted foot with high arch, clawing of toes, insensitive foot ulceration


What is acute painful neuropathy's symptoms and treatment?

burning or crawling pains in lower limbs, worse at night and with bed clothes pressure, treat with good diabetic control, tricyclic antidepressants, gabapentin and carbamazepine


Which are the most common cranial mononeuropathies and their presentation?

3rd and 6th, which supply the extraoccular muscles, presenting with unilateral pain, ptosis and diplopia with sparing of pupillary function


Symptoms of diabetic amyotrophy?

painful wasting, usually asymmetrical, or quadriceps, wasting may be marked and knee reflexes may be diminished or absent


Symptoms of autonomic neuropathy?

affects CV system (tachycardia, loss of sinus arrhythmia, postural hypotension, peripheral vasodilation), GI (diarrhea, gastroparesis and intractable vomiting), bladder (incomplete emptying) and male ED - treatment is symptomatic


Treatment of peripheral neuropathy?

treat symptoms, physio, splints, viagra, male prosthesis, catheter, botulinum toxin, stand up slowly, amitiptyline, duloxteine, pregabalin, tramadol, lidocaine plasters


What is a stroke?

a rapid onset of neurological deficit caused by blood flow to the brain being stopped by a blockage or rupture


What are the types of stroke and what causes them?

ischemia - caused by blockage from emboli of thrombus

haemorrhagic - caused by breakage in blood vessel in the brain of the membrane surrounding the brain

intracranial venous thrombus - caused by space occupying lesion in the brain e.g. tumoru, abscess, MS

carotid or vertebral artery dissection - allowing blood to track the wall of the artery and occlude the lumen - 20% of young strokes


What is the difference between a completed stroke and evolutary stroke?

a completed stroke has reached it's maximum in 6 hours, turns into evolution when the signs and symptoms are getting worse, usually within 24 hours of onset


What is classed as a mini stroke?

patient recovers without a significant neurological deficit and recovers in about 1 week


Symptoms of a stroke?

difficulty walking, loss of balance and coordination, dysarthria and difficulty understanding, unilateral face paralysis and weakness, blurred or darkened vision, sudden headache with nausea, vomiting and dizziness


Common symptoms for a cerebral stroke?

sensory loss, hemiplegia, dysphagia, homonymous hemianopia, visual spatial deficit


Common symptoms in a brainstem stroke?

quadriplegia, disturbances of gaze and vision, locked in syndrome


Common symptoms in Lacunar stroke?

ataxic hemiparesis, pure motor, pur sensory, sensorimotor and dysarthria


Risk factors for stroke?

hypertension, DM, smoking, hyperlipidaemia, obesity, oestrogen containing oral contraceptive, excessive alcohol consumption, polycythaemia, atrial fibrillation, migraine, vasculitis, cocaine, antiphospholipid syndrome, thrombophilia


Investigations in a stroke?

check reflexes, vision, speaking and senses, check for carotid bruit which indicated abnormal blood flow, blood tests, angiogram to find location of black, carotid US, CT, MRI, ECHO, carotid artery stenosis, doppler uS (>70% stenosis is significant), vasculitis shows increased ESR and ANA positive, hyperviscosity with polycythaemia and sickle cell disease


Complications of a stroke?

difficulty speaking, moving, thinking which can improve but others need care, aspiration pneumonia, contractures, depression, constipation, stress in spouse


Immediate treatment of stroke?

protect airway, urgent CT/MRI, thrombolysis, nil by mouth, hydrate, antiplatelets


Primary prevention of stroke?

control risk factors, antihypertensives, treat DM and hyperlipidaemia, exercise, folate supplements, smoking cessation, lifelong anticoagulation if rhuematic or prosthetic heart valves, chronic non rheumatic atrial fibrillation


Secondary prevention of stroke?

antiplatelet drugs e.g. clopidogrel monotherapy, anticoagulation, lower BP, repair ruptured blood vessel, remove blockage with clot dissolving drugs or blood thinners


What is the mortality after 1st stroke?

12% die by day 56


What are primary causes of headache?

migraine, cluster or tension type

hemicrania continua, new daily persistant headache, chronic paraoxysmal hemicrania


Secondary causes of a headache?

meningitis, subarachnoid haemorrhage, GCA, idiopathic intracranial hypertension, medication overuse, low CSF pressure, post traumatic


Red flags in headache history?

>50, HIV, cancer, trauma, change in personality, vomiting, jaw claudication, severe eye pain, change in frequency, postural, sudden onset, exercise, focal neuorlogical symptoms, fever, altered consciousness, neck stiffness etc


What does a thunderclap headache suggest?

subarachnoid haemorrhage


Symptoms of idiopathic intracranial hypertension?

not benign, headache with raised ICP, visual disturbance with acuity/fields, papilloedema, normal constituents of CSF


Risk factors of idiopathic intracranial hypertension?

obesity, drugs e.g. tetracycline


idiopathic intracranial hypertension management?

modify risk factors, monitor visual fields with Goldman fields, acetazolamide/topiramate/diuretics

repeated LPs, CSF shunt, optic nerve fenestration


GCA criteria?

>50, new headache with localised pain, tenderness, decreased pulsation, raised ESR, abnormal biopsy


What is a chronic daily headache?

headache on 15 or more day per month


How common are migraines?

1/4 women and 1/12 men develop it at some point, commonly starts in childhood or as a young adult, many stop in adult life, can be several attacks a week or once every few years


What are the 2 types of migraine?

with aura (classic) and without aura (common)


Symptoms of a migraine (without aura)?

headache on one side of head, typically at front or side, sometimes both, then spreads all over, throbbing pain

nausea, vomiting, photophobia, being off food, blurred vision, poor concetration, stuffy nose, hunger, abdo pain, pale, sweating


When is migraine pain worse?

most often in morning, but can be at any time, then gradually gets worse and peaks 2-12 hours after and then eases off, can last from 4-72 hours


What is a migraine with an aura?

a warning sign before a migraine, which can be visual, numbness, speech, smells etc lasts a few minutes but can last up to an hour, then the migraine develops soon after


Examples of migraine auras and which are most common?

visual aura - temporary loss of vision, flashed of light, objects rotate, shake or boil

numbness, pins and needles, travel up the arm and involve face, lips and tongue

problems with speech

old smells, food craving, feeling odd


What are the phases of a migraine?

irritability, aura, headache, irritable


What are less common types of migraine?

menstrual - only during periods
abdominal - in children, with nausea, and turns to normal migraine in later years
ocular - temporary loss
hemiplegic - severe, on one side of the body, can cause severe dizziness, double vision, hearing problems
basilar type - in the back of the head


What causes an aura?

narrowed blood vessels in the brain, which then soon dilate, causing the headache


Causes/triggers of migraine?

increased signals in the brain, confusing the brain, familial, dieting too fast, irregular meals, smokey rooms, glaring light, flickering TV, loud noises, depression, tiredness, stress, HRT, contraceptibe pill, periods, shift work, bad sleep patterns, menopause


Treatment of migraine?

paracetamol, aspirin, NSAISs, anti emetics, oral triptam medicine e.g. almotriptan which interfere with 5HT (don't take too early on in attack though),

CI in pregnant women, even though migraine can rarely be worse in pregnancy but migraine improves in most cases with pregnacy and breatfeeding


Preventative treatment for a migraine?

topiramate or propanolol, acupuncture, amityriptaline, riboflavin, botulinum toxin type A


How common is a tension type headache?

the most common type of headache, 50% of adults have it at least every month and 1/30 adults have chronic tension type headaches


What is the difference between episodic and chronic type headache?

episodic = occur on less than half of the days in the month

chronic = occur more than half of the days in the month


Symptoms of tension type headache?

band across the forehead, uncomfortable, tiring, worse on activity, sensitive to light and noise, sore neck


How long do tension type headaches last for?

from few hours to several days, usually a few hours, they don't disturb sleep and most people can work through them, you are normally well in between headache


Cause of tension type headache?

tightness of neck muscles or ones over the scalp, poor posture, stress, anything that can make the back of the neck muscles tense, may be more genetically prone to develop them due to being stressed and anxious, painkillers too often due to increasing the number of pain receptors in the head and they become excessively sensitive


When should a tension headache suggest something more serious?

significant head injury in past few months, pain getting worse, accompnaying fever, problems with speech, balance or memory, headache start when coughing/sneezing, worse when sitting or standing, red or painful eyes, worse headache than ever, low immunity, previous cancer


Lifestyle treatment of tension headache?

exercise, change routine, shoulder massage, hot flannel on neck, warm batch, less caffeine, balanced diet, more water, less pillows, avoid excessive noise and reading in low light, diary of frequent headaches, avoid stress triggers


Treatment of tension headache?

paracetamol but not for more than a couple of days at a time, codeine, amitriptyline


What is a cluster headache?

severe unilateral pain, localised in or around the eye with ipsilateral autonomic features, rare


What is an episodic cluster headache?

occurs in periods lasting from several days to one year, usually 2-9 weeks, separated by pain free periods of a month or longer


What is a chronic cluster headache?

CHs occuring for one year without remission or short lived remissions of less than a month (can start as this or develop from episodic)


How common is cluster headache?

rare, 1/1000, more men than female, with a bigger difference between them in chronic then episodic but episodic being more common overall, usually occurs 20-40 years


What signs suggest an episodic cluster headache is turning into chronic?

later onset, sporadic attacks occur, higher frequency of cluster periods, shorter periods of remission


Causes of cluster headache?

1/20 have a 1c family member, associated with HCRTR2 gene


Pathophysiology or cluster headaches?

ipsilateral hypothalamic grey matter has dysfunction causing dilation of cranial vessels mediated by trigeminoparasympathtic reflexes, changes in melatonin in relation to the light and dark cycle affect CH


Clinical features of cluster headaches?

rapid onset of pain over 10 minutes which maintains intensity, excruciating, sharp and penetrating, centered behind the eye/temple/forehead (occasional neck), unilateral pain, lacks 45-90 minutes, 1-2x/day

ipsilateral lacrimation, rhinorrhoea, nasal congestion, eyelid swelling, facial sweating, flushing, conjunctival injection

restless (unlike migraine), band head against wall, nausea


Triggers of cluster headaches?

alcohol, heat, exercise, solvents, disruption of sleep habits


What is the diagnostic criteria of a cluster headache?

for at least 5 attacks:
severe unilateral orbital, supaorbital and/or temporal pain lasting 15-180 minutes if untreated
and at least one:
Ipsilateral conjunctival injection and/or lacrimation.
Ipsilateral nasal congestion and/or rhinorrhoea.
Ipsilateral eyelid oedema.Ipsilateral forehead and facial sweating.
Ipsilateral miosis and/or ptosis
Restlessness or agitation.
One every other day to eight times daily.


DD of CH?

paroxysmal hemicrania
short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)


Red flags of a headache?

change in pattern of headache, new headache aged 50, seizures, headache with systemic illness, personality change, raised ICP symptoms, worst headache ever


Management aim of CH?

suppress attack and reduce frequency and severity of attack


Management of CH?

stop smoking, abstain from alcohol, regular sleep pattern and good hygiene

acute attack - SC sumatriptam and 100% o2, antiinflammatories, lidocaine


Prophylaxis of CH?

verapamil, ECG monitoring, prednisolone, lithium is verapamil not effective, melatonin, sodium valproate in chronic, ergotamine before predicted attack, deep brain stimulation, trigeminal nerve blockage, acupuncture


Side effects of verapamil?

constipation, flushing


What is a subarachnoid hemorrhage?

a spontaneous arterial bleed into the subarachnoid space


How common and in who does subarachnoid hemorrhage occur?

6/100,00 pa, usually around 50years and more common in women


Causes of subarachnoid hemorrhage?

rupture of berry aneurysm (80%) or congenital arteriovenous malformations


Symptoms of unruptured aneurysm?

sight problems, pain on one side of face or around the eye, persistent and severe headaches, painful third nerve palsy, nausea, vomiting, loss of consciousness, meningeal irritation, focal neurological signs, sub hyaloid haemorrhages between the retina and vitreous membrane with or without papilledema, coma, drowsiness


Risk factors for subarachnoid hemorrhage?

smoking, high blood pressure, excessive alcohol consumption, family history, bleeding disorders, mycotic aneurysm, post menopause from decrease in oestrogem, ADPKD, Ehlers Danlos Syndrome, coartation of aorta


Investigations in subarachnoid hemorrhage?

CT shows subarachnoid blood, lumbar punctureshows biliruibin or oxyHb, must be at least 12 hours after symptoms so that Hb can degrade into the bilirubin and turn the CSF xanthochromic (yellow) which can then be detected by spectrophotometry of the supernatant after centrifugation, should therefore be protected from light, MRA find the source of the bleeding


Treatment of subarachnoid hemorrhage?

hydrate to maintain cerebral perfusion, treat hypertension, CCB (nimodipine), analegics, anticonvulsants, antiemtics, intracranial stents, neurosurgical clipping, endovascular coiling, occupational therapist, antidepressants, CBT


What is the difference between neurosurgical clipping and endovascular coiling?

clipping to shut off the aneurysm to prevent it growing or rupturing again
coiling inserts a catheter into an artery in the leg into head and passes coils through the aneurysm so blood can't enter it again so cant rupture, it has increased survival over clipping but increased risk of bleeding


Complications of subarachnoid hemorrhage?

rebleeding (commonest cause of death), delay cerebral ischemia from vasospasm causing vessel to narrow, hydrocephalus, hyponatraemia, epilepsy, cognitive dysfunction, depression, PTSD


Prognosis of subarachnoid hemorrhage?

50% die suddenly or soon after haemorrhage, 10-20% from rebleeding, variable outcome, glasgow coma score on admission has the most prognostic significance which checks level of consciousness and brain injury severity (12 or more has good prognosis)


What 3 things does the Glasgow coma scale base on?

eye opening response, best verbal response, best motor response


What is a subdural haematoma?

where the blood collects in the subdural space following a rupture of a vein running from the hemisphere to the saggital sinus, increasing pressure on the brain (intracranial hypertension) causing brain damage (head injury)


When do symptoms occur in subdural haematoma?

develop soon or can be days or weeks after


Symptoms of subdural haematoma?

progressively bad hangover, feeling and being sick, confusion, personality changes, drowsiness, speech problems, problems with vision, paralysis on one side of the body, problems with walking, frequent falls, seizures, loss of consciousness, localising neurlogical symptoms (unequal pupils)


In who is subdural haematoma most commonly seen?

older, blood thinning medication, drink excessively, accident prone, atrophic brains which makes the connecting veins more susceptible to rupture due to the increased tension


Diagnosis of subdural haematoma?

CT and MRI to rule out other illnesses

shows clot and midline shift and a CRESCENT shape collection of blood over 1 hemisphere


Treatment of subdural haematoma?

surgery to remove haematoma, or if small, monitor to see if they can heal spontaneously


Complications of subdural haematoma surgery?

further bleeding on the brain, infection, deep vein thrombosis, seizures, stroke


What is an extradural hemorrhage?

an injury that fractures the temporal bone and ruptures the underlying middle meningeal artery causing a collection of blood in the potential space between dura and bone


In who is extradural hemorrhage seen?

males, rare in small children due to the plasticity of the skull, less common over 60 due to dura being tightly adherent


What happens shortly after an extradural hemorrhage?

there is a brief period of unconsciousness followed by a lucid interval of recovery which can be a few hours to a few days before a bleed with decrease GCS from rising ICP with rapid deterioration with focal neurological signs and deterioration in consciousness level if surgical drainage is not immediately carried out


What causes a spontaneous extradural hemorrhage?

a coagulation or platelet defect


Symptoms of extradural hemorrhage?

increasingly severe headache, nausea, vomiting, seizures, confusion, bradycardia indicating raised ICP, skull fractures, haematomas, lacerations, CSF otorrhoea or rhinorrhoea, alteration in consciousness and deterioation of the glasgow coma scle, unequal pupils, facial never injury, weakness of limbs, hemiparesis, visual field defects, numbness, coma, death from respiratory arrest


Investigations of extradural hemorrhage?

x ray - skull fracture, fracture lines crossing the middle meningeal vesicles
ct san - haematoma LENS SHAPE (not sickle like subdural) as the tough dural attatchments to the skull keep it more localised
fbc, u and e


Management of extradural hemorrhage?

ABC, O2, full trauma assessment, IV fluids to preserve cerebral perfusion, osmotic diuretics if ICP raised, intubation and ventilation and mannitol if ICP decreased, Burr holes to evacuate haematoma, DVT prophylaxis


Complications of extradural hemorrhage?

neurological deficits (temporary or permanent), death, PTS seizures 1-3 months post injury


What are delayed effects of extradural hemorrhage?

post concussion syndrome which has headaches, dizziness, vertigo, restlessness, emotional liability, inability to concentrate and fatigue


Symptoms of spinal extradural hemorrhage?

spasticity, neuropathic pain, urinary complications


Prognosis of extradural hemorrhage?

excellent in children, low GCS is bad prognosis, prognosis worse if older, intradural lesions, volume of haematoma, temporal location, rapid clincal porogrssion, pupillary abnormalities, increased ICP


What are Charcot Bouchard aneurysm?

0.3-0.9mm, a hypertensive hemorrhage arising from deep perforator arteries that can lead to lacunar infarct, microbleeds and hemorrhage


What are lobar haemorrhages?

large and superficial, common in elderly people


Treatment of an anticoagulant related ICH?

warfarin check urgent INR, consider reversal with Beriplex and vitamin K, if low platelets have platelet transfusion


Treatment of infratentorial bleed with brainstem compression?

surgical decompression


Treatment of hydrocephalus?

external ventricular drain


When should a neurosurgeon be called in a hemorrhage?

deteriorating GCS, hydrocephalus, coning, large cerebellar hemorrhage >3cm, distortion of the 4th ventricle with gcs >8


How are head injuries classified?

on type of injury, distribution of the lesions, time course of damage following the traumatic event


What are the types of distribution of head injuries?

scalp - contusions, lacerations
skull - facture
meninges - haemorrhage, infection
brain - contusion, lacerations, hemorrhage, infection

diffuse axonal injury
diffuse vascular injury


What does a focal head injury suggest?

considerable force, increased risk of haematoma and infection and aerocele

angled or pointed objects cause localised fractures that are open or depressed

flat surfaces cause linear fractures that can extend into skull base and be called contrecoup factures


What is the difference in focal brain damage mechanisms?

contact damage - at or just deep to point of impact

acceleration/deceleration damage - causes differential movement of skull, brain and brain tissue causing traction and damage blood vessels

contusions - superficial bruises of the brain, coup at site of impact/contre coup away from site of impact, first become haemorrhagic then become brown and soft then indented and cavitated after months

lacerations - when the contusion is sufficientlu severe to tear the pia mater


What are the different types of diffuse lesions?

diffuse axonal injury - a clinicopathological syndrome of widespread axonal damage

diffuse traumatic axonal injury

traumatic axonal inury - involving acceleration and decelaration

axonal injury

swelling - increasing ICP, caused by vasodilation, increased cerebral blood volume, extravasation of oedema fluid from damaged vessels, increased water content of neurons and glia

hypoxia-ichemia -

diffuse vasuclar injury - results in near immediate death, multiple petechial hemorrhages throughout the brain


What causes herniation?

bleeding and brain swelling


What is a missile head injury?

can be a depressed injury that does not penetrate the skull, penetrating injuries, or perforating injuries


What is chronic traumatic ecephalopathy?

a neurodegenerative condition seen 8-10 years after repetitive mild traumatic brain injury, 1/3 are progressive

initally irritability, impulsivity, aggression, depression, memory loss

e.g. dementia, gait, speech problems, parkinsonism


How does chronic traumatic encephalopathy appear?

an atrophic neocortex, hippocampus, diencephalon and mamillary bodies

enlarged ventricles with fenustrated cavuum septum

tau positive neurofibrillary and astrocytic tangles

frontal and temporal cortex and limbic regions especially around depths of sulci and limbic regions


What is dystonia?

a state of abnormal muscle tone resulting in muscular spasm and abnormal posture usually caused by a neurological disease, side effect of drug therapy and trauma


Why does vibration induce illusion of movement?

either subnormal muscle spindles responsiveness to stretch improves with fatigue of the muscle or subnormal responsiveness of the CNS to the afferent input improves after fatigue of muscle


What is dopa dysregulation syndrome?

result of presynaptic neuron loss and post synpatic receptor supersensitivity


Symptoms of anterior carotid artery stroke?

leg weakness, sensory disturbance in legs, gait apraxia, truncal ataxia, incontinence, drowsiness, akinetic mutism, decrease in spontaneous speech, stuporus state


Symptoms of middle carotid artery stroke?

contralateral arm and leg weakness, contralateral sensory loss, hemianopia, aphasia, dysphasia, facial droop


Symptoms posterior carotid artery stroke?

contralateral homonymous hemianopia, cortical blindness with bilater involvement of the occipital lobe branches, visual agnosia, prosopagnosia, dyslexia, anomic, aphasia, colour naming, discrimination problems, unilateral heaches


Symptoms of posterior circulation strokes?

motor deficits, dysarthria, speech impairment, vertigo, nausea, vomiting, visual disturbances, altered consciousness


What makes up a muscle?

muscle fibres, which are long multinucleated structures bound by sarcolemma


What are myofibrils?

the contractile element within the myofibre, with thin (I actin) or thick (A myosin) filaments, they are multinucleated and bound by sarcolemma


How do myofibrils work?

excitation force along the whole length simultaneously, and release calcium from SR, it is a T tubule system with invaginations of sarcolemma


What mechanisms can cuase muscular dystrophies?

loss of structural proteins, defective enzymes, disruption of sarcolemma repair, loss of signalling molecules, non coding region mutations, defective post translational modifications


What is a muscular dystrophy?

a group of familial disorders causing replacement of muscle tissue with fibrous connective tissue causing progressive muscle weakness and muscle wasting


What is Duchenne muscular dystrophy?

X linked inheritance (Xp21), muscle degeneration, large deletions and duplications and point mutations disrupting the reading frame causing absence of dystrophin (which connects cytoskeleton to ECM to protect the muscle during force of contraction)


What is the most common muscular dystrophy in children and the prognosis?

DMD 1/3500 males, manifesting at 5 years of age, leading to wheelchair dependence by 13, survival is rare over 30


Symptoms of DMD?

muscle degeneration, thin weak thighs, tip toeing, weak stomach muscles causing stomach to protrude out, shoulders and arms back awkwardly, swayed back, weak butt muscle pushing body out further, thick calves from fat, tight leg muscles, poor balance, awkward walking, cardiac involvement


What is Beckers muscular dystrophy?

DMD but less severe, slower progression, later onset in 20s, and death around 40, worse cardiac involvement


What is myotonic dystrophy?

autosomal dominant, with a CTG trinucelotide repeat expansion in DMPK on c19, most common muscular dystrophy in adults

mutation causes RNA toxicity, downstream, effect causing missplicing of proteins, the CLCN1 is misspliced


Symptoms of myotonic dystrophy?

sustained involuntary contraction (difficult releasing grip after hand shake) as the muscles are still contracting, prominent forehead, narrow face, boldness, cataracts, poorly developed chin, dysphagia and difficulty breathing, diabetes, frontal balding


Treatment of DMD?

supportive, scoliosis corrective surgery in children, manage heart problems, NIV for resp failure, steroids (prednisolone with slows down disease progression given when motor functions plateau), gene therapy (anti sense oligonucleotides weekly sc injections to promote ribosomal read)


Treatment of MD?

supportive, treat cardiac complications, experimental AON knows down expansions, fleicanide of mexilitine for symptom relief of myotonia


What is facialscapulohumeral muscular dystrophy?

autosomal dominant, presenting at 20 years, 20% need a wheel chair, normal life expectancy, contraction in macrosatallite repeat D4Z4 on 4q35, only occurs if polyadenylated, usually 11-100 repeats but only


Symptoms of FSHD?

scapular fixators and ankle dorsiflexor weakness, retinal telangectasia and sensorineural hearing loss


What is acute pain?

sudden onset, in response to injury and infection which resolves as the condition resolves, recurrent acute pain is not chronic pain


What is chronic neuropathic pain?

a neurological condition resulting from damage to or dysfunction of the sensory nervous system, persists in the absence of tissue damage


Clinical features of chronic neuropathic pain?

burning hot and cold sensations, electric shocks, shooting pains, deep severe ache, poorly localised, numbness, paraesthesiae, recognisable dermatomal pattern, skin area may be hypersensitive, exacerbated by contact with hot or cold air, worse at night, distracted by activity associated with depression


What increases risk of chronic pain?

cultural beliefs, ongoing litigation, claims, benefits, work pressures, social circumstances, family support


What causes chronic neuropathic pain?

poorly understood level of damage to sensory pathway, pain fibres small unmyelinated C fibres, increased afferent activity or increased synaptic transmission at level of spinal cord in pain pathway


Neuropathic pain terms?

neuralgia, neuropathy, hyperalgesia, causalgia, hyperpathia, allodynia, lumbagp


Chronic pain management?

try and find cause, neurological examination to identify nerve root compression, X rays, MRI spine, serology, MDT approach, pain management programme, reassurance, encourage activities, exercise

drugs - opioids (pre synaptic inhibition), antidepressants, anti convulsants, N-Methyl D-aspartate receptor antagonists (pre synaptic excitation)

nerve blocks to support physiotherapy and increased activities, TEN stimulators, spinal cord nerve stimulator implantation, dorsal column fiber ablation

capsaicin topical cream and lignocaine gel, acupuncture


Prevention of chronic pain?

regional blocks as an adjuvant to surgery to reduce post amputation phantom limb pain, peri operative gabapentin or pregabelin, holistic approach to surgery


Tolerance to opioid analgesic drugs?

tolerance, increased metabolism, down regulation of opioid receptors, neuroplasticity of CNS target areas


What is involved in the aortic arch?

the brachiocephalic trunk dividing into the right common carotid and subclavian arteries and the aortic arch splits into the left common carotid, subclavian and vertebral


At what level do the common carotid arteries bifuricate?



What are the different point of the internal carotid and their characteristics?

cervical - no narrowings, dilations or branches, lies posterior and lateral to ECA at origin, ascending behind then medial to the ECA

petrous - penetrates temporal bone, running horizontally in the carotid canal, small branch to middle/inner ear, vidian artery to ECA

cavernous - turns superiorly at foramen lacerum entering cavernous sinus, piercing dura, small branches supply dura, cranial nerves 3-6 and posterir pituitary, and connects to ECA via ILT

supraclinoid - opthalmic artery (to optic canal), superior hypophyseal (to pituitary gland, stalk, hypothalamus, optic chiasm), posterior communicating artery and anterior chorodial artery (choroid plexus, optic tract, cerebral peduncle, internal capsule, medial temporal artery)


What does the middle cerebral artery supply?

the lateral lenticulostriate arteries (lentiform nucleus, caudate nucleus, internal capsule), insular cistern, onto brain surface to supply vessel on brain surface


What does the anterior middle cerebral artery supply?

medial lenticulostriate arteries to supply head of caudate nucleus and internal capsule, recurrent artery of Heubner, cortical branches from calllosomarginal and pericallosal arteries


What do the extra cranial VA branches supply?

neck muscles, spinal meninges, spinal cord, anastomoses with other neck vessels


Where do verterbral arteries arise from?

the subclavian arteries (left can arise directly from arch)


What are the intracranial VA branches?

anterior spinal artery, small meduallry perforators and posterior inferior cerebellar artery to supply the medulla and the inferior cerebellum


What does the posterior cerebel artery supply?

cortical territories including the inferior temporal lobe, posterior third of the interhemispheric surface and visual cortex and occipital lobe


How many vertebrae and nerves are in each of the spinal cord?

cervial - 7 - 8
thoracic - 12 - 12
lumbar - 5 - 5
sacral - sacrum - 5
coccygeal - coccyx - 1


What structures are in the grey and white mater?

grey - dorsal horn, intermeddiate zone, ventral horn, lateral horn

white - dorsal funiculus, dorsolateral funiculus, ventrolateral funiculus, ventrolmedial funiculus


How do the internal structure differ in the spinal cord?

white mater decreases down the spine, grey mater increased in the cervical and lumbosacral enlargements, lateral horn is present in the thoracic and upper lumbar segments


What is a stretch reflex?

monosynaptic reflex, important postural reflex, basis of tendon jerk


What is a flexor reflex?

polysynaptic reflex, withdrawal of a limb in response to pain


What is the ventral horn?

receives >20,000 synaptic contacts, has primary efferent tracts from the dorsal root, with interneurons and descending tracts, primary sensory afferent neurones


What are the fasciculus cuneatus and gracilis do?

ascending fibres, ipsilateral

discriminative touch, sensation and proprioception

cuneatus (lateral) - arms
gracilis (medial) - legs


Name nerves?

olfactory -s
optic - s
occulomotor - m
trochlear - m
trigeminal nerve - opthalamic, mandibular, maxially - b
abducens - m
facial - b
vestibulacochlear - s
glossopharengeal - b
vagus - b
accessory - m
hypoglossal - m


Function, damage, cause and symptoms of olfactory nerve?

olfaction (smell)

damage causes anosmia (loss of smell) and hyposmia (reduced smell) and calcosmia (smell a bad smell always)

causes by trauma, meningitis, nasal congestion, tumours on floor of anterior fossa


Function, damage, cause and symptoms of optic nerve?

for visual acuity, colour vision

damage can cause optic atrophy, visual field defects, blurred vision, scotomata (decreased vision in visual field)


What controls papillary responses?

optic and oculomotor nerve

damage caused by ichemia, edema, hemorrhage and trauma


What are the 3 main main types of visual field defect and the causes?

monocular - damage to eye or nerve
bitemporal - lesion at chiasm
homonymous hemianopia - lesions in tract, radiation or lesion in visual cortex


What does a retinal lesion cause?

paracentral scotoma


What does optic nerve lesion cause?

mononuclear field loss (if complete destruction, leads to blindness and loss of pupillary light reflex)


What does a chiasmal lesion cause?

bitemporal hemianopia


What does an optic tract lesion cause?

homoymous hemianopia (macular involvement)


What does a temporal lesion cause?

homoymous quandrantopia (macular involvement)


What does a parietal lesion cause?

homonymous quadrantopia (macular involvement)


What does a occipital cortex or optic radiation cause?

homoymous hemianopia (macular sparing)


What does an occipital pole lesion cause?

homoymous hemianopia (macular sparing)


Causes of an optic nerve lesion?

demyelination, nerve compression, occlusion of retinal artery, trauma, papilloedeme, severe anemia, drugs, toxins


What causes a defect in the optic chiasm?

pituitary adenoma compressing the decussating fibres from the nasal half of each eye, craniopharyngioma, secondary neoplasm


What causes a defect of the optic tract and radiation?

tumour, vascular accident


Why is the macular region spared in occipital cortex defects?

in ischemic lesions due to the dual blood supply of this area from the middle and posterior cerebral arteries


Symptoms of papiloedema?

disc oedema develops, enlargement of a blind sport and blurring of vision, leading to optic neuritis and visual loss


Causes of papilloedema?

raised ICP from tumour, abscess of meningitis, retinal vein obstruction form thrombosis or compression, optic neuritis from demyelination causing inflammation or accelerated hypertension


What causes the pupil to constrict?

bright light, stimulates the Edinger-Westphal nucleus in the midbrain (via the optic tract and the convergence centre in the midbrain) and oculommotor nerve causing contriction


What causes pupil dilation?

sympathetic fibres in the hypothalamus running through the brainstem into the cervical cord and emerge in the spinal cord at T1 to ascend to the neck


Causes of persistent pupillary dilation?

third cranial nerve palsy, antimuscarinic eyedrops, myotonic pupil (Holmes-Adie pupil) which is a delayed/absent reaction to light and convergence seen in young women


Causes of persistent pupillary constriction?

parasympathetic eyedrops for glaucoma, Horner's syndrome, Argyll Robertson pupil, opiate addiction


What is Horner's syndrome?

interruption of the sympathetic fibres to one eye, causing unilateral pupillary constriction, slight ptosis, enophthalmos, loss of sweating on ipsilateral side of the face


What is Argyll Robertson pupil?

small irregular pupil fixed to light and contricts on convergence, caused by neruosyphilis and DM


Function, damage, cause and symptoms of occulomotor nerve?

control ciliary muscles in the eye, change the shape of the eyes for accommodation for viewing objects

damage can cause dipilopia (double vision), unilater complete ptosis and ocular nerve palsy

causes deviation down and out of the eyes

caused by trauma or berry aneurysm in the posterior communicating artery which runs alongside the nerve


Function, damage, cause and symptoms of the trochlear nerve?

Controls extrinsic eye muscles (suoerior oblique muscle)

damage can cause eye problems and vertical diplopia (WHEN LOOKING DOWN OR AWAY FROM AFFECTED SIDE) and nerve palsy (weakness or inability of the nerve)

caused by grave's disease


Function, damage, cause and symptoms of the trigeminal nerve?

for mastication muscles and skin sensation of face and scalp, supplies mucus membranes of sinuses, nose, mouth, tongue and teeth

damage can cause face pain (forehead) and difficulty clenching the jaw, pain when eating, jaw deviates to side of lesion

caused by neurlagia, herpes virus, carcinoma


Function, damage, cause and symptoms of the abducens never?

extrinsic eye movement via the lateral rectus muscle

damage causes one eye to face inwards permanently this produces a squint and diplopia when looking at the side of the lesion

caused by nerve palsy, cerebral vascular accidents, combined damage from cranial nerve 4


Function, damage, cause and symptoms of facial nerve?

for taste, saliva and tear secretion and facial expression

damage causes lack of facial expression on the affected side of the face (bells palsy), impaired taste

severe damage can cause face paralysis

caused by versticular voster virus and trauma


What does 7th nerve palsy, cerebellar signs and 5th, 6th and 8th nerve involvement suggest?

lesion of the cerebellopontine angle, from a meningioma or acoustic neuroma


What do cavernous sinus lesions involve?

oculomotor nerves, opthlamic, maxialy division


What conditions can affect the cranial nerves?

DM, sarcoidosis, vasculitis, syphilis, brain stem tumours, MS, infarction


What is Bell's palsy and when does it occur?

acute isolater unilateral facial nerve palsy caused by a viral infection (herpes simplex) cause swelling of the 7th nerve within the petrous temporal bone, peak incidence between 65-74 and more common in pregnancy


Function, damage, cause and symptoms of vestibulocochlear nerve?

for hearing and vestibular function (balance)

damage can cause sensorineural deafness and tinnitus (noise ringing in ear), veritgo (illusion of movement which can cause vomiting), loss of air conduction in ear, nystagmus and loss of balance

caused by perilymph fistula, cochlear concussion and auditory oscicle disruption and damage to nerve directly


What is nystagmus?

rhythmic oscillation of the eyes can be pendular (pendular movement of the eyes, no rapid phase, occurs with poor visual fixation or congental lesion) or jerk (fast and slow component)


What is the difference between horizontal and veritical nystagmus?

horzontal can be peripheral (transient minutes or hours) or central lesions (weeks, months)


Function, damage, cause and symptoms of the glossopharengeal nerve?

taste, tongue sensation, saliva secretion, o2 and co2 monitoring in blood

damage causes pain in tongue, throat, tonsils and ear from compression of nerve

damage caused by combination to 10 and 11


Function, damage, cause and symptoms of vagus nerve?

taste, pharyngeal activity, epiglottis sensation, innervation of GI tract, control trachea and cardiac muscles

damage causes dysphgaia, difficulty speaking, vocal cord weakness, damage to nuclear amygus, cause aphonia (harsh voice), pain


Function, damage, cause and symptoms of the accessory nerve?

pharynx, larynx and soft palate muscles, sternocleoidmastoid and trapezius muscles

damage causes paralysis of the sternocleoidmastoid muscle leadness to flatness of neck and inability to rotate the neck, paralysis of trapezius muscle causing drooping of muscle and inability to raise shoulders

damage usually involves nerves 9 and 10, cuased by wound to neck


Function, damage, cause and symptoms of the hypoglossal nerve?

for tongue muscles

damage cause lingual paralysis, dysarthria, and dysphagia, muscle tongue atrophy, so half of tongue cannot be controlled and deviates to side of lesion.

caused by tumour, stroke, infection


Function of spinal nerves?

carry information between CNS and periphery


What are the two pathways that the peripheral ascend to the thalamus and the cerebral cortex?

posterior columns - carrying sensory modalities for vibration, joint position sense, two point discrimination and light touch, they ascend uncrossed to the gracile and cuneate nuclei in the medulla, then the second oder neurons cross the midline to form the medial lemniscus and pass to the thalamus

spinothalamic tracts - carry sensations of pain and temperature, fibres synapse in the dorsal horn of the cord, crossing the midline and ascend as the spinothalamic tracts to the thalamus


What are the main symptoms of posterior column and spinothalamic tract lesions?

paraesthesiae, numbness and pain


Symptoms of peripheral nerve lesions?

felt in the distribution of the affected peripheral never e.g. median nervem has bilateral symmetrical, distal sensory loss and burning


Symptoms of spinal root lesions?

symptoms in the dermatome supplied by that root, tinglign discomfort (if it was a lesion of the sensory tract there would be general symptoms no specific dermatomes


Symptoms of spinal cord lesions?

evident below level of lesion

spinothalamic leads to contralateral pain and temperature sensation loss below lesions

proprioception lesion results in loss of this ipsilaterally below lesion


Symptoms of a pontine lesion?

loss of all forms of sensation on opposite side of lesion


Symptoms of thalamaic lesions?

complete contralateral sensory loss and spontaenous pain (caused by thalamic infarct)


Symptoms of cortical lesions?

sensory loss, neglect of one side of the body, subtle disorders of sensation , no pain


Upper motor neuron lesion symptoms?

oppssite side to lesion

fasciculation absent, no muscle wasting, spasticity, clonus, weakness in arm extensors and leg flexors, exaggerated tendon reflexes, extensor plantar response, drift of the outstretched hand downwards


Lower motor neuron lesion symptoms?

signs are on the same side

fasiculation present, wasting, hypotonia, loss of tendon reflexes


What is fasciculation?

visible contraction of single motor units, appearing as a twitch


Symptoms of acute injury to upper motor neurons?

transient flaccid weakness and hyporeflexia


What are the 2 main patterns of clinical features that occur in UMN disorders?

hemiparesis - weakness of the limbs on one side, usually caused by lesion within brain or brainstem e.g. stroke

paraparesis - weakness of legs, bilateral damage to corticospinal tracts caused by lesions below T1, tetraparesis in quadriplegic, weakness of arms and legs indicating high cervical cord damage from trauma - cord lesions result in UMN signs below lesion, LMN signs at lesion and unaffected muscle above lesion


What mainly causes LMN lesions?

anterior horn cell lesions (MND and poliomyelitis)

spinal root lesions (cervical and lumbar disc lesions)

peripheral nerve lesions (trauma, compression, polyneuropathy)


Based on rapidity of onset, what does vision loss suggest the cause?

sudden - vascular (GCA)

acute - inflmmatory/infectious (optic neuritis, TB)

subacute - inflammatory/infectious/neoplastic

chronic - neoplastic/degenerative

episodic - migraine


Based on symptoms, what is the suggesting cause of vision loss?

headache - migraine, tumour, raised ICP

malaise, fever, myalgia, headache, jaw claudication - giant cell arteritis

painful monocular visual loss - optic neuritis

systemic weakness, dysarthria, dysphagia - MG

dysarthria and ataxia - posterior fossa lesion


What social factors could lead to vision loss?

smoking, alcohol - carcnoma and tobacco alcohol amblyopia

diet - nutritional optic neuropathy



What genetic disorders can cause vision loss?

Leber's herditary optic neruopathy and AD/R optic neuropathy, MS


What is the papillomacular bundle?

contains >90% of all retinal nerve fibres in the optic nerve, projecting images from the macula, with a high metabolic activity

lesions causes a central or centro caecal scotoma (lebers hereditary optic neuropathy, toxic and nutritional optic neurpathy and optic neuritis)


What causes a superior homonymous quadtrantanopias?

lesion of Meyers loop

inferior striate cortex below the calcarine fissure lesion


What causes an inferior homonymous quadrantanopias?

parietal lobe lesions and superior striate cortex lesions


What is an incongruous visual field defect?

the defects do not match up between the two eyes


When does macular sparing occur?

with occipital lesions that spare the posterolateral striate cortex due to the contralateral blood supply


What are the different causes of ptosis?

partial - horners syndrome
complete - third nerve palsy
varying/fatiguing - MG