Neuroscience Flashcards

Question

What are the presenting symptoms for a cluster headache?

Answer 1

Repeated attacks of unilateral pain Most patients are agitated and restless (only 7% can lie still during an attack) Nausea and vomiting Photophobia and phonophobia Migraine type aura Lacrimation, rhinorrhoea and partial Horner's syndrome (ptosis and miosis: sympathetic features)

Answer 2

Signs of Horner's syndrome (ptosis and miosis)

Answer 3

``` 1st line: Brain CT scan or MRI Bloods-ESR Pituitary function tests Consider: ECG, polysomnogram (for sleep apnoea) ```

Answer 4

Inflammation of the brain parenchyma

Answer 5

In the majority of cases encephalitis is the result of a viral infection. Most common in the UK is HSV. Other: bacteria (syphilis, staph A), immunocompromised (Cytomegalovirus, toxoplasmosis, Listeria)

Answer 6

Annual UK incidence is 7.4 in 100,000

Answer 7

Usually encephalitis is a mild self-limiting illness. Subacute onset (hours to days) headache Fever Vomiting Neck stiffness, photophobia, i.e. symptoms of meningism (meningoencephalitis) with behavioural changes Drowsiness and confusion There is often a history of seizures Focal neurological symptoms (e.g. dysphasia and hemiplegia) may be present It is important to obtain a detailed travel history

Answer 8

Reduced level of consciousness with deteriorating GCS, seizures, pyrexia. Signs of meningism: Neck stiffness, photophobia, Kernigs test positive. Signs of raised intracranial pressure: hypertension, bradycardia, papilloedema. Focal neurological signs. Minimental examination may reveal cognitive or psychiatric disturbances.

Answer 9

Positive when the thigh is flexed at the hip and knee at 90 degree angles, and subsequent extension in the knee is painful (leading to resistance)- indicating the presence of meningitis or subarachnoid haemorrhage

Answer 10

Blood: FBC (raised lymphocytes), U&E (SIADH may occur), glucose (compare with CSF glucose), viral serology, ABG. MRI/CT: Excludes mass lesion. HSV produces characteristic oedema of the temporal lobe on MRI. Lumbar puncture: raised Lymphocytes,monocyte and protein, glucose usually normal. Viral PCR is first line. EEG: May show epileptiform activity, e.g. spiking activity in temporal lobes

Answer 11

A chronic disorder that causes unprovoked, recurrent seizures (Paroxysmal synchronised cortical electrical discharges)

Answer 12

Focal seizure: -Seizure localised to specific cortical regions, such as temporal lobe seizures, frontal lobe seizures, occipital seizures, complex partial seizures Generalised seizure: Seizures which affect consciousness typically tonic-clonic, absence attacks, myoclonic, atonic (drop attacks) or tonic seizures

Answer 13

*The majority of cases are idiopathic Primary epilepsy syndromes (e.g. idiopathic generalised epilepsy, temporal lobe epilepsy, juvenile myoclonic epilepsy) Secondary seizures (symptomatic epilepsy): -Tumour -Infection (e.g. meningitis, encephalitis, abscess) -Inflammation (vasculitis, rarely multiple sclerosis) -Toxic/metabolic (sodium imbalance, hyper- or hypoglycaemia, hypocalcaemia, hypoxia, porphyria, liver failure) -Drugs (e.g. including withdrawal e.g.alcohol, benzodiazepines) -Vascular (haemorrhage, infarction) -Congenital anomalies (e.g. cortical dysplasia) -Neurodegenerative disease (e.g. Alzheimers disease) -Malignant hypertension or eclampsia -Trauma

Answer 14

Syncope Migraine Non-epileptiform seizure disorder (e.g. dissociative disorder)

Answer 15

Common Prevalence in 1% of general population Peak age of onset is in early childhood or in the elderly

Answer 16

Seizures result from an imbalance in the inhibitory and excitatory currents (e.g. Na+ or K+ ion channels) or neurotransmission (i.e. glutamate or GABA neurotransmitters) in the brain

Answer 17

Frontal lobe focal motor seizues: Motor convulsions May demonstrate Jacksonian march (spasm spreading from mouth or digit) There may be post-ictal flaccid weakness (Todds paralysis)- on one side Temporal lobe seizures: Aura (visceral and psychic symptoms: fear or deja-vu sensation) Hallucinations (olfactory, gustatory) Frontal lobe complex partial seizures: Loss of consciousness with associated automatisms and rapid recovery May be postictal aphasia seen after focal-onset seizures

Answer 18

Tonic-clonic (grand mal): Vague symptoms before an attack (e.g. irritability), followed by tonic phase (generalised muscle spasm), followed by a clonic phase (repetitive synchro- nous jerks), and associated faecal or urinary incontinence, tongue biting After a seizure, there is often impaired consciousness, lethargy, confusion, headache, back pain, stiffness Absence (petit mal): Usual onset in childhood Characterized by loss of consciousness but maintained posture (patient stops talking and stares into space for seconds), blinking or rolling up of eyes with other repetitive motor actions (e.g. chewing) No postictal phase. Non-convulsive status epilepticus: Acute confusional state Often fluctuating Difficult to distinguish from dementia.

Answer 19

Depends on aetiology, usually normal between seizures | Look for focal abnormalities indicative of brain lesions

Answer 20

Bloods: - FBC: if raised WCC, may indicate an infectious cause - U&E: electrolyte imbalance can lead to a provoked generalised tonic-clonic seizure - Glucose: extreme hypoglycaemia or hyperglycaemia can cause provoked generalised tonic-clonic seizures - Toxicology screen: identify cause - Prolactin, helps to distinguish a generalised tonic-clonic seizure (GTCS) from a non-epileptic event (transient increase shortly after a true seizure) - Creatinine Kinase (Elevated in true seizures) EEG: - Indicated after any unprovoked seizure event - Helps confirm or refute the diagnosis; assists in classifying the epileptic syndrome: generalised epileptiform activity or focal, localising abnormality - Usually performed inter-ictally and often normal and does not rule out epilepsy CT/MRI: For structural, space-occupying and vascular lesions ``` Other investigations: Particularly for secondary seizures according to suspected aetiology e.g. lumbar puncture (infections), HIV serology ```

Answer 21

Only start anti-convulsant therapy after >2 unprovoked seizures: - Lamotrigine: newer agent - Topiramate: newer agent - Oxcarbazepine - Carbamazepine: reasonable choice - Levetiracetam - Valproic acid* contraindicated in pregnant women or women of a child bearing age - Other medications: Phenytoin and Gabapentin Patient education: - Avoid triggers (e.g. alcohol) - Encourage seizure diaries - Recommend supervision for swimming or climbing, driving is only permitted if seizure free for 6 months - Women of childbearing age should be counseled regarding possible teratogenic effects of AEDs and should consider taking supplemental folate to limit the risk - Drug interactions: e.g. enzyme-inducing AEDs can limit the effectiveness of oral contraception Surgery: limited for refractory epilepsy: Removal of definable epileptogenic focus

Answer 22

A life-threatening neurological condition defined as 5 or more minutes of either continuous seizure activity or repetitive seizures without regaining consciousness

Answer 23

Treatment is often initiated in 5–10 minutes as early treatment has higher treatment success (definition is >30 mins) 1. Resuscitate and protect airway, breathing and circulation 2. Check glucose and give if hypoglycaemic- consider thiamine 3. IV lorazepam or IV or PR diazepam (repeat once after 15 min if needed) If seizures recur or fail to respond, IV phenytoin (15mg/kg) under ECG monitoring Alternative IV agents include phenobarbitone, levetiracetam or sodium valproate 4. If these measures fail, consider general anaesthesia. This requires intubation and mechanical ventilation 5. Treat the cause: e.g. correct hypoglycaemia or hyponatraemia 6. Check plasma levels of all anticonvulsants

Answer 24

Fractures with tonic-clonic seizures Behavioural problems Sudden death in epilepsy (SUDEP) Complications of AEDs (e.g. neutropenia or osteoporosis with carbamazepine)

Answer 25

50% remission at 1 year | Mortality 2 in 100 000/year, directly related to seizure or secondary to injury

Answer 26

An acute bleed between the dura mater and the inner surface of the skull- also known as haematomas

Answer 27

Most commonly caused by skull trauma in the temporoparietal area, usually following falls, assaults or sporting injuries The pterion is vulnerable to trauma as it is the fusion point between the parietal, frontal, sphenoid and temporal bones: the middle meningeal artery is involved in 75% of EDH as it lies underneath the pterion, which leads to a high risk of arterial rupture

Answer 28

Patients most commonly affected are adult males between 20-30 years old because they are more likely to experience a traumatic injury

Answer 29

Early signs: - Severe headache: occurs after the initial impact and can persist - Initial loss or fluctuance in consciousness: may present vaguely as tiredness or confusion -Period of lucidity after initial loss of consciousness: usually lasts between 6 and 8 hours but may last for days depending on the speed of the haematoma growth Late signs: Rapid deterioration and loss of consciousness:GCS score will begin to drop as intracranial pressure rises and the brainstem begins to herniate

Answer 30

Eyes: - Focal neurological deficits become apparent in the eyes due to compression of the cranial nerves - E.g. CNIII (oculomotor nerve) palsy may result in fixed dilation of the ipsilateral pupil. This is colloquially known as a “blown pupil” Muscle weakness: - Hemiparesis typically begins on the contralateral side to the EDH due to compression of the ipsilateral motor cortex - Hemiparesis may become bilateral as the ICP increases and the brainstem becomes compressed - Ipsilateral hemiparesis can also occur through Kernohan’s phenomenon (extensive midline shift of the brain due to mass effect from the growing extradural haematoma) Upper motor neuron signs: -Positive Babinski’s sign (upgoing toes), hyperreflexia and spasticity (hypertonia) (signs are not specific to EDH) Cushing’s triad: A physiological response to critically high ICP This is characterised by bradycardia, hypertension and deep/irregular breathing Persisting unconsciousness: deep coma and very low GCS Death: often by respiratory arrest due to compression of the respiratory centres in the brainstem so they are unable to function

Answer 31

Non-contrast CT: FIRST LINE - Show characteristic bi-convex mass within the skull if there is an EDH is present (“lemon-shaped” ) - Occurs as because the dura attaches to the skull more tightly across the suture lines and the pressure of the haematoma is not enough to overcome these sutures - Secondary features on CT head can include midline shift and brain stem herniation, both of which are indications for early surgical intervention MRI/X-ray: can also be performed but not as sensitive as CT. Show bone fractures and MRI can help differentiate between extradural and subdural haemorrhages through looking at the displaced dura *Lumbar punctures are absolutely contraindicated for extradural haematomas, as they result in a drop in CSF pressure, which may speed up brain herniation

Answer 32

A type of acute inflammatory demyelinating polyneuropathy

Answer 33

An inflammatory process where antibodies after a recent infection reacts with self-antigen on myelin or neurons. Often no aetiological trigger is identified (idiopathic in about 40%), in other cases: - Post-infection (1–3 weeks): bacterial (e.g. Campylobacter jejuni ), HIV, herpes viruses (e.g. zoster, CMV) - Malignancy (lymphoma, Hodgkins disease) - Post-vaccination

Answer 34

nnual UK incidence is 1–2 in 100,000 Affects all age groups but rarely infants Slight male predominance

Answer 35

Progressive symptoms of 􏰅1 month duration of: - Ascending symmetrical limb weakness (lower > upper) - Ascending paraesthesia Cranial nerve involvement (e.g. dysphagia, dysarthria and facial weakness) In severe cases, the respiratory muscles may be affected

Answer 36

General motor examination: -Hypotonia -Flaccid paralysis -Arreflexia (typically ascending upwards from feet to head) General sensory examination: -Impairment of sensation in multiple modalities (typically ascending upwards from feet to head) Cranial nerve palsies (less frequently): Facial nerve weakness (lower motor neurone pattern) Abnormality of external ocular movements Signs of bulbar palsy Autonomic function: Assess for postural BP change and arrhythmias

Answer 37

Type II respiratory failure: Important to identify early (e.g. CO2 flap, bounding pulse, drowsiness) This can be insidious, and needs regular assessment

Answer 38

Nerve conduction study: Reduced conduction velocity or conduction block, but can be normal in the early phase of the disease Lumbar puncture: elevated CSF protein, cell count and glucose normal Blood: LFTs: elevation in hepatic enzymes -Anti-ganglioside antibodies: present in 85% to 90% of patients with Miller-Fisher syndrome and 25% of Guillain–Barre syndrome cases Spirometry: reduced fixed vital capacity indicates ventilatory weakness, may also show reduced maximal inspiratory pressure, or maximal expiratory pressure ECG: Arrhythmias may develop

Answer 39

``` A disorder characterised by -pupillary miosis (constricted pupil) -ptosis (drooping of the upper eyelid) -facial anhydrosis (absence of sweating) This is due to the interference of the sympathetic trunk supplying the eye ```

Answer 40

Usually due to the disruption of the sympathetic chain because of a lesion or a growth Variety of factors: -Carotid artery dissection -Tumour in neck or chest cavity, particularly a neuroblastoma and a tumour of the upper part of the lung (Pancoast tumor) -Lesion in midbrain, brain stem, upper spinal cord, neck, or eye orbit -Inflammation or growths affecting the lymph nodes of the neck -Surgery or other forms of trauma to the neck or upper spinal cord

Answer 41

Relatively rare disorder The congenital, and more rare, form of Horner syndrome is present at birth Affects everyone equally

Answer 42

``` Unilateral symptoms: Drooping upper eyelid Constriction of the pupil Dryness (lack of sweating) on the same side of the face (ipsilateral) as the affected eye Retraction of the eyeball ```

Answer 43

``` Pupillary miosis (constricted pupil) Ptosis (drooping of the upper eyelid) Facial anhydrosis (absence of sweating) ```

Answer 44

Drops e.g. tropicamide which will cause significant dilation of the healthy eye and little dilation of the affected eye if Horner syndrome is caused by a third-order neuron abnormality — a disruption somewhere in the neck or above Imaging: locate the site of a probable abnormality causing Horner syndrome: Magnetic resonance imaging (MRI): tumour in neck/lesion in brain Carotid ultrasound: dissection Chest X-ray: pancoast tumour at apex CT: brain lesion/space occupying Bloods: CRP/ESR inflammatory markers

Answer 45

No specific treatment, often resolves when an underlying medical condition is treated In some cases surgical removal of the lesion or growth may be appropriate. Radiation and chemotherapy may be beneficial to patients with malignant tumours

Answer 46

Dependent on the underlying cause of Horner's syndrome and it's treatment

Answer 47

An autosomal dominant neurodegenerative disorder characterised by progressive chorea and dementia, typically commencing in middle age

Answer 48

Caused by an expanded CAG (extended trinucleotide repeat expansion) repeat at the N-terminus of the gene that codes for the huntingtin protein which results in toxic gain of function The disease exhibits anticipation (earlier age of onset in each successive generation)

Answer 49

Worldwide prevalence eight in 100 000 Average age onset 30–50 years, duration of disease is approximately 20 years from time of diagnosis to time of death Affects men and women equally Rare in East Asian populations (particularly Japan)

Answer 50

FH of Huntingtons disease Insidious onset in middle-age: -Progressive fidgeting and clumsiness/restlessness -Developing into involuntary, jerky, dyskinetic movements often accompanied by grunting and dysarthria In late disease: the patient may become rigid, akinetic and bed-bound Early cognitive, emotional and behavioural changes are dominated by lability (exaggerated changes in mood), dysphoria, mental inflexibility, anxiety, leading on to dementia

Answer 51

Classically, patient presents with chorea and dysarthria Slow voluntary saccades (quick simultaneous movement of both eyes) and supranuclear gaze restriction Other presentations include: -Parkinsonism and dystonia, muscles contract involuntary (especially in juvenile-onset disease) -Mental state examination reveals cognitive and emotional deficits

Answer 52

Genetic analysis: Diagnostic if >39 CAG repeats in the HD gene Imaging: Brain MRI or CT may show symmetrical atrophy of the striatum (particularly the caudate nuclei) and butterfly dilation of the lateral ventricles -not specific as it is seen in other conditions Bloods: May be necessary to exclude other pathology: anti-nuclear antibodies blood film (acanthocytes), TFT, ESR

Answer 53

The enlargement of the cerebral ventricular system (accompanied by raised CSF pressure) Divided into: Obstructive and non-obstructive (or communicating or non-communicating)

Answer 54

Abnormal accumulation of CSF in the ventricles can be caused by: (1) Impaired outflow of the CSF from the ventricular system (obstructive) - lesions of the third ventricle, fourth ventricle, cerebral aqueduct - posterior fossa lesions (e.g. tumour, blood) compressing the fourth ventricle - cerebral aqueduct stenosis (2) Impaired CSF resorption in the subarahnoid villi (non-obstructive) - tumours; - meningitis (typically tuberculosis) - Normal pressure hydrocephalus (NPH) is the idiopathic chronic ventricular enlargement * The long white matter tracts (corona radiata, anterior commisure) are damaged causing gait and cognitive decline

Answer 55

Bimodal age distribution In the young: congenital malformations and tumours In the elderly: tumours and strokes

Answer 56

``` Obstructive hydrocephalus: -Acute drop in conscious level -Diplopia (double vision) Normal Pressure Hydrocephalus (NPH): Chronic cognitive decline -Falls -Urinary incontinence ```

Answer 57

``` Obstructive hydrocephalus: -Impaired GCS -Papilloedema -VI nerve palsy (false localizing sign of increased ICP) -In neonates, the head circumference may enlarge, and sunset sign (downward conjugate deviation of eyes) NPH: -Cognitive impairment -Gait apraxia (shuffling) -Hyper-reflexia ```

Answer 58

CT head: *-First-line investigation to detect hydrocephalus (ventricular enlargement) -May also detect the cause (e.g. tumour in the brainstem) CSF: -Obtained from ventricular drains or lumbar puncture -May indicate an underlying pathology (e.g. tuberculosis) Check for MC&S, protein, glucose (CSF and plasma) Lumbar puncture: This is contra-indicated in obstructive hydrocephalus as it can cause tonsilar herniation and death Others: -Levodopa challenge: suspected Parkinsons due to gait apraxia

Answer 59

Also known as a spinous tap inserted between two vertebrae to remove a sample of cerebrospinal fluid

Answer 60

Diagnosis of CNS conditions/infections Inject medications – such as painkillers, antibiotics or chemotherapy Inject a spinal anaesthetic (epidural) – to numb the lower part of your body before an operation Remove some fluid to reduce pressure in the skull or spine

Answer 61

A lumbar puncture is generally a safe procedure and serious side effects are uncommon. Headaches Swelling and lower back pain where the needle was inserted Potential infections at site of insertion

Answer 62

Inflammation of the meninges (pia mater and arachnoid) (coverings of the brain) most commonly caused by infection

Answer 63

Infection: Bacterial: -Neonates: Group B streptococci, Escherichia coli, Listeria monocytogenes -Children: Haemophilus influenzae, Neisseria meningitidis, Streptococcus pneumoniae -Adults: Neisseria meningitidis (meningococcus), Streptococcus pneumoniae, TB -Elderly: Streptococcus pneumoniae, Listeria monocytogenes Viral: Enteroviruses, mumps, Herpes simplex V, HIV Fungal: Cryptococcus (associated with HIV infection)

Answer 64

``` Close communities (e.g. dormitories) Basal skull fractures Mastoiditis Sinusitis Inner ear infections Alcoholism Immunodeficiency Splenectomy Sickle cell anaemia CSF shunts Intracranial surgery ```

Answer 65

Variation according to geography, age, social conditions | Viral meningitis is one of the most common infections of the CNS

Answer 66

``` ASK ABOUT TRAVEL HISTORY *Severe headache *Photophobia *Neck stiffness or backache Irritability Drowsiness Vomiting Fever Clouding of consciousness High-pitched crying or fits (common in children) ```

Answer 67

``` Signs of meningism: -Photophobia -Neck stiffness Signs of infection: -Fever -Tachycardia -Hypotension -Skin rash (petechiae with meningococcal septicaemia) -Altered mental state ```

Answer 68

Blood: Two sets of blood cultures (do not delay antibiotics) Imaging: -CT scan to exclude a mass lesion or increased intracranial pressure before LP, (may lead to cerebral herniation during subsequent CSF removal) *A CT scan of the head must be done before LP in patients with: -Immunodeficiency -History of CNS disease -Reduced consciousness -Fit -Focal neurologic deficit -Papilloedema Lumbar puncture: Send CSF for MCS and Gram staining (Streptococcus pneumoniae: Gram-positive diplococcic, Neisseria Meningitidis: gram-negative diplococcic) Bacterial: Cloudy CSF, raised neutrophils, raised protein, reduced glucose (CSF: serum glucose ratio of <0.5) Viral: raised Lymphocytes, raised protein BUT normal glucose *TB: Fibrinous CSF, raised lymphocytes, raised protein, reduced glucose

Answer 69

Immediate IV/IM antibiotics if meningitis suspected (before lumbar puncture or CT) Antibiotics: Benzylpenicillin may be given as initial blind therapy Amoxicillin + gentamicin Add vancomycin and if necessary rifampicin If anaphylaxis to penicillin or cephalosporins- use chloramphenicol Steroids: IV (10 mg for 4 days) given shortly before or with first dose of antibiotics to reduce complications Avoid dexamethasone in patients with HIV Resuscitation: Patient is best managed in ITU Prevention (only applicable to meningococcal meningitis): Notify public health services and consult a consultant in communicable disease control, vaccination for meningococcal serogroups A and C

Answer 70

``` Septicaemia Shock DIC-disseminated intravascular coagulation, which is the inappropriate clotting of blood within the vessels Renal failure Fits Peripheral gangrene Cerebral oedema Cranial nerve lesions Cerebral venous thrombosis Hydrocephalus Water- house–Friderichsen syndrome (bilateral adrenal haemorrhage) ```

Answer 71

Mortality rate from bacterial meningitis is high (10–40% with meningococcal sepsis) In developing countries mortality rate often higher Viral meningitis self-limiting

Answer 72

A severe episodic headache that may have a prodrome of focal neurological symptoms (aura) and is associated with systemic disturbance. Usually presents early to mid life. Can be subclassified as: -Migraine with aura (classical migraine) -Migraine without aura (common migraine) -Migraine variants (familial hemiplegic, opthalmoplegic and basilar)

Answer 73

Precise pathophysiological mechanism poorly understood Early aura of cortical spreading depression associated with intracranial vasoconstriction resulting in localized ischaemia This is followed by meningeal and extracranial vasodilation mediated by 5-HT, bradykinin and the trigeminovascular system Initiating event: neuronal depolarisation, triggered more easily

Answer 74

Usual onset in adolescence or early adulthood, but can occur in middle age Females are more commonly affected with 15–20% and 6% in males (3:1)

Answer 75

``` Headache: -Pulsating -Often unilateral Duration 4–72 h (Most migraine attacks are episodic and chronic daily headache lasting many weeks suggest either analgesia-overuse headache or secondary headaches) ``` Associated symptoms: - Nausea and vomiting - Photophobia - Phonophobia * May be preceded by aura that may include: - visual disturbance: flashing lights, spots, blurring, zigzag lines (fortification spectra), blindspots (scotomas) - Other sensory symptoms such as tingling or numbness in limbs

Answer 76

``` Stress Exercise Lack of sleep Oral contraceptive pill Certain foods (e.g. caffeine, alcohol, cheese, chocolate) ```

Answer 77

*Usually no specific physical findings ``` Examination of mental state, neurological examination, funduscopy, sinuses, cervical spine, general examination to exclude secondary causes Secondary causes include: -Meningoencephalitis (meningitis) -Idiopathic intracranial hypertension -Subarachnoid haemorrhage -Space-occupying lesion -Temporal arteritis ```

Answer 78

*Diagnosis based on history Investigations may be needed to exclude other diagnoses. Blood: FBC, ESR (temporal arteritis) CT/MRI: If suspicion of secondary headache disorders Lumbar puncture: If suspicion of meningitis. Do not perform until space-occupying lesion excluded for risk of herniation

Answer 79

``` Migraine is a chronic condition Acute: -NSAID (e.g. naproxen) -Paracetamol -Codeine -Antiemetics (e.g. metoclopramide) -Triptans (5-HT1 agonists):sumatriptan and zolmitriptan (orally, nasally or subcutaneously) ``` Prophylaxis (if > 2/month, 50% patients benefit): - b-blockers - Amitriptyline (TCA) - Topiramate and sodium valproate (anti-epileptics) - Pizotifen (5-HT2 antagonist) - Calcium-channel blocker *Menstrual migraine can be controlled by the oral contraceptive pill Advice: Encourage regular meals and sleep, caffeine restriction, measures to reduce stress, avoid triggers, symptom diary. Rest in quiet dark room during episode.

Answer 80

Disruption of daily activities Can progress onto analgesia-overuse headache due to chronic use of analgesics Gastric ulcers (overuse of NSAIDs)

Answer 81

Usually chronic, but majority of cases can be managed well by preventative/ early treatment measure

Answer 82

A progressive neurodegenerative disorder of cortical, brainstem and spinal motor neurons (lower and upper motor neuron)

Answer 83

1. Amyotrophic lateral sclerosis (ALS) : combined degeneration of upper and lower motor neurones producing a mix of UMN and LMN neurones 2. Progressive bulbar palsy variant: Dysarthria and dysphagia with wasted fasciculating tongue (LMN) and brisk jaw jerk (UMN) 3. Primary lateral sclerosis variant: UMN pattern of weakness, brisk reflexes, extensor plantar responses, without LMN signs 4. Progressive muscular atrophy variant: Only LMN signs, e.g. flail arm or flail foot syndrome. Better prognosis

Answer 84

Unknown Free radical damage and glutamate excitotoxicity have been implicated as mutations Pathology: Progressive motor neuron degeneration and death with gliosis (proliferation or hypertrophy of several different types of glial cells) replacing lost neurons

Answer 85

Rare, annual incidence is 2 per 100,000 Mean age of onset is 55 years 5–10% have family history with autosomal dominant inheritance

Answer 86

``` Weakness of limbs (focal or asymmetrical) Speech disturbance (slurring or reduction in volume) Swallowing disturbance (e.g. choking on food, nasal regurgitation) There may be behavioural changes (e.g. disinhibition, emotional lability-mood changes) ```

Answer 87

``` Combination of upper motor neuron (UMN) and lower motor neuron (LMN) signs often affecting several regions asymmetrically LMN features: -Muscle wasting -Fasciculations -Flaccid weakness -Depressed or absent reflexes UMN features: -Spastic weakness -Brisk reflexes -Extensor plantars (Babinski reflex) *Sensory examinaiton: Should be normal ```

Answer 88

Investigations aimed to confirm the clinical diagnosis by providing evidence of combined UMN and LMN loss and excluding other causes Blood: - CK (mild elevation), ESR - Consider testing for anti-GM1 ganglioside antibodies (present in multifocal motor neuropathy) Electromyography (EMG): Features of acute and chronic denervation with giant motor unit action potentials in more than 1 limb and/or paraspinals Nerve conduction studies: Most often normal MRI: To exclude cord or root compression, and brainstem lesion in progressive bulbar palsy variant. May show high signal in motor tracts on T2 imaging

Answer 89

An inflammatory demyelinating disease characterised by the presence of episodic neurological dysfunction in at least two areas of the central nervous system (brain, spinal cord, and optic nerves) separated in time and space

Answer 90

1. Relapsing-remitting MS: Commonest form -Characterised by clinical attacks of demyelination with complete recovery in between attacks 2. Clinically isolated syndrome: Single clinical attack of demyelination; (does not qualify as MS), 10–50% progress to developing MS 3. Primary progressive MS: Steadily accumulation of disability with no clear relapsing-remitting pattern

Answer 91

Unknown Inflammatory and degenerative components that may be triggered by an environmental factor or factors in persons who are genetically susceptible Immune-mediated damage to CNS myelin results in impaired conduction along axons There is also associated grey and white matter involvement-atrophy

Answer 92

``` Female sex Genetic factors Smoking (weak) PMH and FH of autoimmune diseases EBV exposure Prenatal and childhood vitamin D deficiency ```

Answer 93

The most common cause of neurological disability among young adults Prevalence in UK is 1 in 1000 (rare in Far East) Female to male ratio of around 3:1 Usually presents at 20–40 years A geographic gradient, with higher incidence at latitudes closer to the poles, has been linked with MS

Answer 94

``` Optic neuritis (commonest): -Unilateral deterioration in visual acuity and colour perception -Pain on eye movement Sensory system: -Pins and needles -Numbness -Burning Motor: -Limb weakness -Spasms -Stiffness -Heaviness Autonomic: -Urinary urgency -Hesitancy -Incontinence -Bowel dysfunction -Impotence Psychological: Depression, psychosis ``` *Uhthoff’s phenomenon: Transient increase or recurrence of symptoms due to conduction block precipitated by a rise in body temperature e.g. in the bath

Answer 95

Optic neuritis: - Fundoscopy, in active disease, there is a swollen optic nerve head, in chronic disease, there may be optic atrophy - Visual field testing: Central scotoma (optic nerve affected) or field defects (optic radiations affected) - Relative afferent pupillary defect: Tested with a swinging torch test. Both pupils contract when light is shone on the unaffected side, both pupils dilate when light is swung to the diseased (eye) - Internuclear opthalmoplegia: Lateral horizontal gaze produces a failure of adduction of the contralateral eye Sensory: -Paraesthesia (vibration and joint position sense loss more common than pain and temperature) Motor: - UMN signs (e.g. spastic weakness, brisk reflexes) - Cerebellar: Limb ataxia (intention tremor, past-pointing and dysmetria on finger-nose test and heel-shin test), dysdiadochokinesis, ataxic wide-based gait (foot dragging), scanning speech Lhermitte’s phenomenon: Electric shock-like sensation in arms and legs precipitated by neck flexion

Answer 96

Diagnosis based on two or more CNS lesions with corresponding symptoms, separated in time and space (McDonald criteria) MRI-brain: inflammation and degeneration, typical lesions seen in MS in the periventricular regions MRI-spinal cord: demyelinating lesions in the spinal cord, particularly the cervical spinal cord Bloods: exclude differentials, FBC, TFTs, Vitamin B12 Evoked potentials: prolongation of conduction, particularly asymmetrical prolongation in the visual evoked potentials (delayed in 􏰄90% of patients with MS) Others: - CSF electrophoresis:unmatched oligoclonal bands - Lumbar puncture: Microscopy to exclude other infective or inflammatory causes

Answer 97

An autoimmune disease affecting the neuromuscular junction producing weakness of skeletal muscles (muscle fatiguability)

Answer 98

Impairment of neuromuscular junction transmission, most commonly (80-90%) due to auto-antibodies against the nicotinic acetylcholine receptor (nAChR) Myasthenia gravis is associated with other autoimmune conditions (e.g. pernicious anaemia) and thymoma development (75%)- FH*

Answer 99

Uncommon disease | More common in females at younger ages (child bearing age), but equal gender distribution in middle age

Answer 100

Muscle weakness that worsens with repetitive use or towards end of day Ocular symptoms: Drooping eyelids, diplopia Bulbar symptoms: -Facial weakness (myasthenic snarl) -Disturbed hypernasal speech -Difficulty in smiling, chewing or swallowing (nasal regurgitation of fluids)

Answer 101

Can be generalized (affecting many muscle groups), bulbar (affecting bulbar muscles) or ocular (affecting only the eyes) Eyes: Bilateral/unilateral ptosis Complex opthamoplegia *Test for ocular fatiguability by ask patient to sustain upward gaze for 1 min and watch for progressive ptosis -Ice on eyes test: Placing ice-packs on closed eyelids for 2 min can improve neuromuscular transmission, reducing ptosis= considered positive when ptosis improves by 􏰃2 mm from baseline Bulbar: Reading aloud may provoke dysarthria or nasal speech after 3 min Limbs: Test the power of a muscle before and after repeated use of the muscle (e.g. 20 repetitions)

Answer 102

Bloods: - *Serum acetylcholine receptor antibody (positive in 80%) - Muscle specific tyrosine kinase (anti-MUSK) antibody (positive in up to 70%) Serial pulmonary function tests: If suspected Myasthenia Gravis crisis, low forced vital capacity (FVC) and negative inspiratory force (NIF) Repetitive nerve stimulation: >10% decline in compound muscle action potential (CMAP) amplitude EMG: Single-fibre EMG may demonstrate jitter (variability in latency from stimulus to muscle potential) indicating fluctuation in neuromuscular conduction CT chest: thymoma (which occurs in about 15% of patients with MG) or thymic hyperplasia (which occurs in 75%), malignancies in the lung

Answer 103

An autosomal dominant genetic disorder affecting cells of neural crest origin, resulting in the development of multiple neurocutaneous tumours

Answer 104

Type 1 NF: -Characterized by peripheral and spinal neurofibromas -Multiple cafe au lait spots- painless, coffee coloured patches -Freckling (axillary/inguinal) -Optic nerve glioma -Lisch nodules (on iris) -Skeletal deformities -Phaeochromocytomas -Renal artery stenosis Type 2 NF: -Characterized by schwannomas, e.g. bilateral vestibular schwannomas (acoustic neuromas), peripheral/spinal schwannomas, meningiomas, gliomas, cataracts *Schwannoma: tumor that grows in the sheaths of nerves in your peripheral nervous system

Answer 105

Multiple mutations have been described in tumour suppressor genes NF1 (encodes neurofibromin resulting in excessive activity of the proto-oncogene p21-ras) and NF2 respectively (encodes merlin (or schwannomin))

Answer 106

Incidence is 1 in 3000 births for type 1 NF and 1 in 40000 for type 2 NF No gender or racial predilection

Answer 107

``` Positive family history (but 50% are caused by new mutations) Type 1 NF: -Skin lesions (cafe au lait spots) -Learning difficulties (in 40%) -Headaches -Disturbed vision (optic gliomas, in 􏰄15%) -Precocious puberty (may indicate lesions of the pituitary from optic glioma involving the chiasm) Type 2 NF: -Hearing loss -Tinnitus -Balance problems -Headache -Facial pain -Numbness ```

Answer 108

Type 1 NF: >5 cafe au lait macules of >5mm (pre-pubertal individuals) or >15mm (post-pubertal individuals) Neurofibromas (appear as cutaneous nodules or complex plexiform neuromas) Freckling in armpit or groin Lisch nodules (hamartomas on iris) Spinal scoliosis Type 2 NF: Few or no skin lesions Sensorineural deafness with facial nerve palsy or cerebellar signs if schwannoma large (Acoustic neuroma)

Answer 109

Ophthalmological assessment and audiometry MRI brain and spinal cord: For vestibular schwannomas, meningiomas and nerve root neurofibromas, other brain tumours, hydrocephalus Skull X-ray: Sphenoid dysplasia in type 1 NF Genetic testing: identifiable mutation involving the NF1 locus and NF2

Answer 110

Neurodegenerative disease of the dopaminergic neurones of the substantia nigra, characterized by: - Bradykinesia - Rigidity - Tremor - Postural instability

Answer 111

Sporadic and idiopathic (most common): Unknown Environmental toxins and oxidative stress have been proposed (e.g. pesticides, wood pulp) Secondary: -Neuroleptic therapy (e.g. in schizophrenia). -Vascular insults (e.g. basal ganglia or midbrain strokes). -MPTP (neurotoxin) from illicit drug contamination -Post-encephalitis (e.g. influenza) -Repeated head injury (e.g. boxing)

Answer 112

Very common: 1–2% of >60-year-olds Mean age of onset is 􏰄57 years Annual incidence is 20 in 100,000

Answer 113

``` Insidious onset Tremor at rest, usually noticed in hands Stiffness and slowness of movements Difficulty initiating movements (e.g. getting out of chair, rolling in bed) Frequent falls Smaller hand writing (micrographia) Insomnia Mental slowness (bradyphenia) ```

Answer 114

Tremor: Classically pill rolling rest tremor, decreased on action or flexed posture, usually asymmetrical Rigidity: Lead pipe rigidity of muscle tone, with superimposed tremor (cogwheel rigidity) Gait: Stooped, simian, shuffling, small-stepped gait with reduced arm swing (difficulty in initiation of walking) Postural instability: Falls easily with little pressure from the back (propulsion) or the front (retropulsion) Psychiatric: Depression is very common. Cognitive problems and dementia may occur in late disease Others: -Bradykinesia -Frontalis overactivation (furrowing of the brow) -Expressionless face (hypomimia) -Soft monotonous voice (hypophonia) -Impaired olfaction on formal testing -Mmild impairment of up-gaze and tendency to drool (sialorrhoea) -Involuntary movements in one part of the face associated with voluntary movement in another part of the face (synkinesis)

Answer 115

Diagnosis is clinical Levodopa trial: -Timed walking and clinical assessment after levodopa may be informative -Antiemetic (domperidone) may be needed. Bloods: Exclude other causes, serum ceruloplasmin (excludes Wilsons disease in young onset) CT or MRI brain: Useful for excluding other causes of gait decline (e.g. hydrocephalus, vascular disease) *(DAT-scan): Reduction in striatum and putamen. May be necessary for distinguishing from essential tremor

Answer 116

The sum of volumes of brain, cerebrospinal fluid (CSF) and intracerebral blood is constant Therefore an increase in one should cause a reciprocal decrease in either one or both of the remaining two

Answer 117

Raised intracranial pressure (ICP) can arise as a consequence of intracranial mass lesions, disorders of cerebrospinal fluid (CSF) circulation and more diffuse intracranial pathological processes Its development may be acute or chronic

Answer 118

- Localised mass lesions: traumatic haematomas (extradural, subdural, intracerebral) - Neoplasms: glioma, meningioma, metastasis - Abscess - Focal oedema secondary to trauma, infarction, tumour - Disturbance of CSF circulation: obstructive hydrocephalus, communicating hydrocephalus - Obstruction to major venous sinuses: depressed fractures overlying major venous sinuses, cerebral venous thrombosis - Diffuse brain oedema or swelling: encephalitis, meningitis, diffuse head injury, subarachnoid haemorrhage, encephalopathy, water intoxication - Idiopathic intracranial hypertension.

Answer 119

Most common cause is head injury

Answer 120

Headache Early changes in mental state include lethargy, irritability, slow decision making and abnormal social behaviour *Untreated, this can deteriorate to stupor, coma and death Vomiting (in early stages without nausea) Pupillary changes, including irregularity or dilatation in one eye

Answer 121

Fundoscopy shows: blurring of the disc margins, loss of venous pulsations, disc hyperaemia and flame-shaped haemorrhages. In later stages, obscured disc margins and retinal haemorrhages may be seen Unilateral ptosis or third and sixth nerve palsies In later stages, ophthalmoplegia and loss of vestibulo-ocular reflexes Late signs include motor changes (hemiparesis), raised blood pressure, widened pulse pressure and slow irregular pulse

Answer 122

- Head injury and obtundation: bleeding can form a rapidly expanding haematoma leading to rapidly rising ICP if not treated promptly - Syncope, headache and meningismus: abrupt onset of headache with these symptoms suggests ruptured cerebral aneurysm or vascular lesion - Focal deficit followed by seizures: focal deficit can be associated with a mass lesion and when there is oedema or haemorrhage. Intracranial compartment shift can cause increased ICP within minutes or hours; status epilepticus can cause decompensation of cerebral volume regulation - 'Talk and deteriorate': patients typically talk recognisably following head injury, then go into coma in the first two days. The usual cause is an intracranial haematoma

Answer 123

Headache Papilloedema Vomiting

Answer 124

CT/MRI scanning to determine any underlying lesion: haematoma, space occupying lesion, thrombosis Check and monitor blood glucose, renal function, electrolytes and osmolality

Answer 125

Results from processes that compress or displace arterial, venous, and cerebrospinal fluid spaces, as well as the cord itself This can occur as a result of extrinsic causes and lesions, or intrinsic aetiologies of the cord substance

Answer 126

Can occur as a result of spine trauma, vertebral fracture, intervertebral disc herniation, primary or metastatic spinal tumour (CNS, multiple myeloma, lung, breast) or infection (TB, abscess) *Trauma is a leading cause of acute SCC Vertebral compression fractures are usually due to low-energy trauma in weakened bone and are seen in older people (with conditions such as osteoporosis, osteomalacia, osteomyelitis)

Answer 127

A devastating condition affecting people across all age groups worldwide Trauma is the main cause of acute SCC Age < 29: most common cause are sport and recreation injury Age > 65: Falls are the leading cause

Answer 128

Back pain with sensory, motor, or autonomic symptoms: -Numbness or paraethesias -Weaknessor paralysis -Bladder or bowel dysfunction Age affected: 16-30: trauma 30-50: disc disease 40-75: malignancy -Acute symptoms are commonly associated with traumatic cord compression or disc herniation -Chronic symptoms are associated with osteoporosis, osteomyelitis, or malignancy

Answer 129

Due to disc compression and stenosis of the spinal canal The syndrome consists of: - Saddle (perineal) anaesthesia (numbness around the anus) - Back pain which radiates down leg - Bladder retention - Leg weakness The reported incidence of cauda equina syndrome resulting from herniated lumbar disc varies from 1% to 15% and commonly affects males aged 40 to 60 years Bladder dysfunction may present as incontinence but often presents earlier as difficulty starting or stopping a stream of urine. Urinary incontinence is on the basis of overflow

Answer 130

Hyper-reflexia: an early sign of spinal cord compression and is more commonly associated with malignancy Sensory loss: loss of pinprick, temperature, position, and vibratory sensation occurs early in cord compression, especially when due to malignancy Muscle weakness or wasting

Answer 131

*MRI spine: (the imaging study of choice for suspected cord compression of any aetiology) -Disc displacement -Epidural enhancement -Mass/lesion Gadolinium enhanced MRI spine: -imaging modality of choice in infection including osteomyelitis and epidural abscess -Infection: epidural space and bone involvement -Malignancy: visualisation of tumour (and metastatic disease) Plain spine X-ray: (of limited value) -Decreased disc space height (disc compression) -Loss of bony detail (tumour, infection) -Misalignment of vertebral elements (trauma) -Loss of end-plate definition (infection) CT spine: (used when MRI is unavailable/planning surgery) -Cord compression from tumour expansion into the canal or bony fragments from pathological fracture CT Myelograhpy: (allows imaging of the degree of cord compression in flexion and extension) -classical hour-glass constriction shape of the dye column Identify cause: -Bloods: FBC(WCC), ESR/CRP -Blood/CSF cultures Tumour biopsy and histopathology -Urodynamic studies: evaluate the degree and cause of sphincter dysfunction -PET scan of spine: identify lesions/areas of hypermetabolism

Answer 132

When intervertebral discs become damaged and cause compression or irritation of a nearby nerve root

Answer 133

Can be caused by a variety of conditions and injuries: - a herniated disc - sciatica - degenerative disc disease - tumors of the spine - osteoarthritis or spinal arthritis - spinal stenosis - compression fractures - spondylolisthesis - scoliosis caused by an abnormal curve - diabetes - cauda equina syndrome

Answer 134

``` Aging Overweight Poor posture Improper lifting techniques Repetitive motions FH of degenerative bone conditions ```

Answer 135

More common in 40-50 year olds | May be more common in men

Answer 136

Different symptoms, depending on the nerve compressed: Cervical: pain in the neck, shoulder, upper back, or arm Thoracic: burning or shooting pain in the rib, side, or abdomen Lumbar: sharp pain starting in the back, extending to the foot (or numbness/tingling/hypersensitivity)

Answer 137

Physical examination: neurological and limbs

Answer 138

Physical examination Radiological imaging: x-ray, MRI, CT EMG: test nerve function

Answer 139

The rapid permanent neurological deficit from cerebrovascular insult Also defined clinically, as focal or global impairment of CNS function developing rapidly and lasting >24 h

Answer 140

A transient episode of neurological dysfunction caused by focal brain, spinal cord, or retinal ischaemia, without acute infarction, fully resolving within 24h

Answer 141

Ischaemic (caused by vascular occlusion or stenosis) | Haemorrhage (caused by vascular rupture, resulting in intraparenchymal and/or subarachnoid haemorrhage)

Answer 142

87% of strokes are Ischaemic Causes include: Thrombosis: -In the elderly, this arises from atherosclerosis within cerebral vessels affecting mainly small vessels (causing lacunar infarcts) and less commonly large vessels (e.g. middle cerebral artery) -Can also arise from prothrombotic states (e.g. dehydration or thrombophilia). Emboli: -From intimal flap of carotid dissection, atheromatous plaques in the carotid arteries or from the heart (e.g. atrial fibrillation) Hypotension: -If below the autoregulatory range maintaining cerebral blood flow, infarction results in the watershed zones between different cerebral artery territories Others: Vasculitis, cocaine

Answer 143

``` Around 10% of cases Hypertension -Charcot–Bouchard microaneurysm rupture Amyloid angiopathy Arteriovenous malformations Less commonly: trauma, tumours, arteriovenous malformations, vasculitis ```

Answer 144

``` Common Annual incidence is 2 in 1000 Third most common cause of death in industrialized countries (US, UK) Most patients are in seventh decade Young strokes (<50 years merit extensive investigation) Ischaemic -87% Haemorrhagic -10% Subarachnoid haemorrhage -3% ```

Answer 145

*Sudden onset (deterioration within seconds) *Weakness, sensory, visual or cognitive impairment, impaired coordination, or consciousness Unilateral Head or neck pain (in carotid or vertebral artery dissection) *Enquire time of onset (critical for emergency management if <4.5 h) Enquire if history of atrial fibrillation, MI, valvular heart disease, carotid artery stenosis, recent neck trauma or pain

Answer 146

Anterior circulation - Anterior cerebral: Lower limb weakness (motor cortex), confusion (frontal lobe) - Middle cerebral: Facial weakness, hemiparesis (motor cortex), hemisensory loss (somatosensory cortex), apraxia, hemineglect (parietal lobe), receptive or expressive dysphasia (language centres), quadrantanopia (superior or inferior optic radiations) Posterior circulation - Posterior cerebral: Hemianopia (blindness over half the visual field) - Anterior inferior cerebellar artery: Vertigo, ipsilateral ataxia, ipsilateral deafness (or tinnitus), ipsilateral facial weakness - Posterior inferior cerebellar artery (lateral medullary syndrome of Wallenberg): Vertigo, ipsilateral ataxia, ipsilateral Horners syndrome, ipsilateral hemifacial sensory loss, dysarthria and contralateral spinothalamic sensory loss - Basilar artery: Combination of cranial nerve pathology and impaired consciousness (emergency)

Answer 147

Disease in the deep perforating arteries: - Internal capsule or pons: Pure sensory or motor deficit (or combination of both) - Thalamus: Loss of consciousness, hemisensory deficit - Basal ganglia: Hemichorea, hemiballismus (movement disorder), Parkinsonism Multiple lacunar infarcts: - Vascular dementia - Urinary incontinence - Gait apraxia (marche a petits pas, shuffling small-stepped gait, with upright posture and often normal or excessive arm-swing)

Answer 148

``` Headache Meningism Focal neurological signs Nausea and vomiting Signs of raised ICP (papilloedema) Seizures ```

Answer 149

Protect the airway: This avoids hypoxia/aspiration Maintain homeostasis: -Blood glucose: keep between 4–11 mmol/L *URGENT CT/MRI within 1h: (1st)CT-head: For rapid detection of haemorrhages- often normal especially in lacunar infarcts or very early in the stroke (<6 h) MRI-brain: Rarely available acutely, but much higher sensitivity for infarction Diffusion-weighted imaging (DWI) can differentiate between recent strokes (<2 weeks) and old strokes -*Essential if: thrombolysis considered, high risk of haemorrhage (reduced GCS, signs of high ICP, severe headache, meningism, progressive symptoms, bleeding tendency or anticoagulated) Others: - ECG: exclude arrhythmia or ischaemia - Carotid Doppler ultrasound: Important to exclude carotid artery disease. - CT-cerebral angiogram: To detect artery dissections or intracranial stenosis - Cardiac enzymes: exclude concomitant MI - Prothrombin time: if no DH of anticoagulant use then thrombolysis does not need to be delayed for test results

Answer 150

F- facial dropping A- arm weakness S- speech difficulties T- time (narrow therapeutic window as stroke is a permanent condition)

Answer 151

*If haemorrhage excluded on CT-head: Intravenous thrombolysis may be considered (Alteplase) *best results are within 90 minutes

Answer 152

1. Aspirin or clopidogrel to prevent further thrombosis once haemorrhage excluded on CT-head. 2. Heparin anti-coagulation may be considered in certain subgroups where there is a high risk of emboli recurrence or stroke progression (e.g. carotid dissection, recurrent cardiac emboli, critical carotid artery stenosis) 3. Formal swallow assessment is essential (nasogastric tube may be required) 4. Close nursing and GCS monitoring 5. Thromboprophylaxis (graded compression stockings) - Hemicraniectomy may be indicated for mass effect from infarcted tissue in the first 48 h *Multidisciplinary rehabilitation: Speech and language therapy, occupational therapy, physiotherapy and neuropsychology.

Answer 153

Control hypertension and seizures IV mannitol and hyperventilation helps lower intracranial pressure Evacuation of haematoma or ventricular drainage may be required *Multidisciplinary rehabilitation: Speech and language therapy, occupational therapy, physiotherapy and neuropsychology.

Answer 154

Aspirin and dipyridamole, warfarin anticoagulation (if atrial fibrillation), stop smoking, control hypertension and hyperlipidaemia, treatment of carotid artery disease

Answer 155

Carotid endartectomy within 2 weeks of stroke or TIA reduces risk of further stroke although carries a significant peri-operative risk - symptomatic stenosis of 70–99% or - symptomatic stenosis of 50–99% or - crescendo TIAs not responding to medical treatment

Answer 156

Cerebral oedema (raised ICP and local compression) Immobility Infections (e.g. pneumonia, UTI, from pressure sores) DVT Cardiovascular events (arrhythmias, MI, cardiac failure) Death

Answer 157

10% mortality in first month Up to 50% of those who survive remain dependent 10%have a recurrence in 1 year Generally, poorer for haemorrhage than for ischameic.

Answer 158

Arterial bleeding into the subarachnoid space and is a medical emergency

Answer 159

Rupture of an intracranial saccular aneurysm is the leading cause of non-traumatic SAH, accounting for approximately 80% of cases (usually at Circle of Willis) The remaining 20% are attributed to non-aneurysmal perimesencephalic SAH, arteriovenous malformations, arterial dissections, use of anticoagulants, drug abuse (e.g. cocaine, amphetamines) and other rare conditions

Answer 160

Peak age of incidence in the fifth decade | 3% of all cases of Stroke

Answer 161

``` Hypertension Smoking Excess alcohol intake Saccular aneurysms are associated with: -polycystic kidney disease Marfans syndrome -pseudoxanthoma elasticum (genetic disease that causes mineralization of elastic fibers in some tissues, can cause premature atherosclerosis) -Ehlers–Danlos syndrome (connective tissue disorder) ```

Answer 162

``` Sudden onset severe headache (classically described as 'THUNDERCLAP HEADACHE') Nausea and vomiting Neck stiffness Photophobia Reduced level of consciousness ```

Answer 163

Meningism: -Neck stiffness -Kernigs sign (resistance or pain on knee extension when hip is flexed) because of irritation of the meninges by blood -Pyrexia Reduced GCS Signs of increased intracranial pressure: -Papilloedema -IV or III cranial nerve palsy, ophthalmoplegia -Hypertension and bradycardia. Fundoscopy: -Rarely subhyaloid haemorrhage (between retina and vitreous membrane) Focal neurological signs: Usually develop on second day and are caused by ischaemia from vasospasm and reduced brain perfusion

Answer 164

*Order an emergency non-contrast CT head in all patients presenting with acute sudden, severe headache (thunderclap headache)- standard diagnostic test: -Hyperdense areas in the basal regions of the skull (caused by blood in the subarachnoid space) Bloods: -FBC: Leukocytosis (cerebral vasospasm) -U&E: Hyponatraemia is the most common electrolyte abnormality in SAH -Troponin I: elevated during first 24h, associated with poorer outcomes -Serum glucose: Hyperglycaemia develops in 1/3 of SAH -clotting: coagulopathy may be present ECG: arrhythmias, ischaemia Angiography (CT or intra-arterial): -To detect the source of bleeding if the patient is a candidate for surgery or endovascular treatment Lumbar puncture: -Raised opening pressure, increased red cells, few white cells, xanthochromia (straw-coloured CSF, bloody CSF) because of breakdown of Hb, confirmed by spectrophotometry of CSF

Answer 165

A collection of blood that develops between the dural and arachnoid coverings of the brain Acute: Within 72 h Subacute: 3–20 days Chronic: After 3 weeks

Answer 166

The primary aetiology of both acute and chronic subdural haematomas is trauma -results in shearing forces which tear veins (bridging veins) that travel from the dura to the cortex Less commonly, subdural haematomas are associated with rupture of a cerebral aneurysm or vascular malformation In children, non-accidental injury should always be considered

Answer 167

Acute: Tend to occur in younger patients/associated with major trauma (5–25% of cases of severe head injury) More common than extradural haemorrhage Chronic: More common in elderly, studies report incidence of 1–5 per 100 000

Answer 168

``` Acute: -History of trauma with head injury -Patient has reduced conscious level Subacute: -Worsening headaches 7–14 days after injury, altered mental status Chronic: -Can present with: headache confusion cognitive impairment psychiatric symptoms gait deterioration focal weakness seizures *There may not be a history of fall or trauma; hence have low index of suspicion especially in the elderly and alcoholics ```

Answer 169

Acute: -Reduced GCS -With large haematomas resulting in midline shift, an ipsilateral fixed dilated pupil may be seen (compression of the ipsilateral third nerve parasympathetic fibres) -Pressure on brainstem: reduced consciousness, bradycardia Chronic: -Neurological examination may be normal -There may be focal neurological signs (III or VI nerve dysfunction, papilloedema, hemiparesis or reflex asymmetry)

Answer 170

*Non-contrast CT scan: 'lemon' shaped (crescent) mass, concave over brain surface -CT appearance changes with time -Acute subdurals are hyperdense, becoming isodense over 1–3 weeks (such that presence may be inferred from signs such as effacement of sulci, midline shift, ventricular compression and obliteration of basal cisterns) -Chronic subdurals are hypodense (approaching that of CSF) MRI-brain: Has higher sensitivity especially for isodense or small SDHs Plain x-ray: skull fracture

Answer 171

Acute: ALS protocol with priorities of cervical spine control and ABC (head injury has a significant risk of cervical spine injury) Disability: GCS, pupillary reactivity Antiplatelet or anticoagulant agent stopped and/or reversed If signs of raised ICP, head elevation and consider osmotic diuresis with mannitol and/or hyperventilation Once stabilised, obtain CT-head Conservative: Especially if small and minimal midline shift (SDH<10mm thickness, and midline shift <5 mm) Surgical: Prompt Burr hole or craniotomy and evacuation for symptomatic subdurals >10 mm, with >5 mm midline shift (better outcome if within 4 h) ICP monitoring devices may be placed Chronic: - If symptomatic or there is mass effect on imaging, surgical treatment with Burr hole or craniotomy and drainage (a drain may be left in for 24–72 h) - Asymptomatic SDH without significant mass effect is best managed conservatively with serial imaging to monitor for spontaneous resorption - Haematomas that have not fully liquefied may require craniotomy with membranectomy Children: Younger children may be treated by percutaneous aspiration via an open fontanelle or if this fails, placement of a subdural to peritoneal shunt

Answer 172

Raised ICP Cerebral oedema pre-disposing to secondary ischaemic brain damage Mass effect (transtentorial or uncal herniation) Post-op: - Seizures are relatively common - Recurrence (Up to 33% for SDH) - Intracerebral haemorrhage - Subdural empyema: a collection of pus, in the subdural space - Brain abscess or meningitis - Tension pneumocephalus: life threatening neurosurgical emergency, subdural air causes a mass-effect over the underlying brain parenchyma

Answer 173

Acute: Underlying brain injury is the most important factor on outcome Chronic: Generally have a better outcome than acute SDHs, reflecting lower incidence of underlying brain injury, with good outcomes in 3/4 of those treated by surgery

Answer 174

Can be either episodic or chronic They are rarely disabling or associated with any significant autonomic phenomena and so patients do not usually seek medical care and usually successfully self-treat

Answer 175

Muscle contraction is often considered the cause of pain in tension headaches Psychological stress is the most common trigger -Extended periods of mental tension or psychological stress may play a role in central sensitisation and the development of chronic tension headache Other associated factors: -Disturbed sleep patterns can result in episodic tension headaches -Insomnia and other sleep disorders can result in chronic tension headaches

Answer 176

Probably the most common type of headache In adults, the mean global prevalence of tension-type headache is 42% Most common between the ages of 20 to 39 years and then decline Patients either do not need treatment or they successfully self-treat and are therefore less likely to mention these headaches than patients with migraine

Answer 177

``` Generalised head pain: BILATERAL -pressure-like and non-throbbing pain Frontal/occipital pain are common areas Non-pulsatile pain Constricting pain: expressed as tight band around head ```

Answer 178

Normal neurological examination | Muscle tenderness: pericardial, trapezius, temporalis, sternocleidomastoid, later ptyergoid, masseter

Answer 179

Clinical diagnosis: typical headache without associated features (nausea, vomiting), normal neurological examination For progressing headaches: - CT sinus: exclude sphenoid sinusitis - MRI brain: exclude tumour - Lumbar puncture: exclude infective causes (e.g. brain abscess, meningitis, encephalitis), sinus venous thrombosis

Answer 180

Episodic headaches: simple analgesics, such as paracetamol, ibuprofen, or aspirin [risk of transformation into chronic headache 'medicine-overuse syndrome' (also termed ‘analgesic rebound’)] Chronic headaches-Preventive: - Used when patients experience greater than 7 to 9 headache days per month - tricyclic antidepressants (amitriptyline) may reduce the frequency and intensity of attacks ``` Non-drug treatment: Relaxation training Electromyographic biofeedback Cognitive behavioural therapy Myofascial trigger point-focused massage ```

Answer 181

Peptic ulcer: occurs secondary to NSAID use

Answer 182

Self-treatment with simple analgesic medicine is usually effective

Answer 183

A transient episode of neurological dysfunction caused by focal brain, spinal cord, or retinal ischaemia, without acute infarction (fully resolve)

Answer 184

1. 29%: Cardioembolic events - Intracardiac thrombus may form in response to some secondary risk factor such as stasis from impaired ejection fraction or atrial fibrillation - The precipitating factor may be thrombogenic within the heart such as an infectious vegetation or artificial valve 2. 16%: in situ thrombosis of an intracranial artery or artery-to-artery embolism of thrombus as a result of stenosis or unstable atherosclerotic plaque 3. 16%: Small-vessel occlusion - Microatheromas, fibrinoid necrosis, and lipohyalinosis of small penetrating vessels are seen - Hypertension and diabetes predispose to small ischaemic lesions - Because these may occur in the brainstem and internal capsule, a small lesion can result in significant symptoms 4. 3%: Occlusion due to hypercoagulability, dissection, vasculitis, vasospasm, or sickle cell occlusive disease

Answer 185

Stroke is a major health concern worldwide-accounts for 11% of all deaths in England and Wales TIAs are more common in men, a large number of TIAs go unrecognised

Answer 186

``` Sudden onset and brief duration of symptoms (majority will resolve within the first hour) Unilateral symptoms: -Weakness -Sensory, visual or cognitive impairment -Impaired coordination, or consciousness PMH: cardiac disease/atherosclerosis ```

Answer 187

``` Unilateral weakness or sensory loss Amaurosis fugax (cannot see out of one or both eyes due to a lack of blood flow to the eye(s)) or hemianopia Aphasia- impairment of language Cranial nerve defects Vertigo Incoordination, ataxia, or syncope ```

Answer 188

Bloods: -Glucose: blood glucose <3.3 mmol/L (<60 mg/dL) suggests hypoglycaemia as mimic of TIA -FBC: exclude infection -U&Es: usually normal -Lipid profile: should be started on statin therapy ECG: AF may be present MRI brain (with diffusion): localise infarct, suggest aetiology, and distinguish TIA from stroke, if unavailable Head CT CT head: poor ability to rule in ischaemia in TIA or early stroke but has nearly 100% sensitivity in ruling out acute haemorrhage ESR: rule out vasculitis such as temporal arteritis Carotid doppler ultrasound: screening test for stenosis Angiography(CT/MR): visualise stenosis

Answer 189

Therapies to prevent second ischaemic events - Antiplatelet agents are primary therapy for atherosclerotic disease (arteries) - Anticoagulation is preferred for cardioembolic events (veins) Aggressive risk-factor modification: - Statin therapy - Antihypertensive therapy - Lifestyle modification - Carotid endarterectomy or stenting

Answer 190

Stroke: The risk of stroke following TIA is considerably high due to unstable plaque, thrombus, or exposed thrombogenic surfaces MI: less than that of a stroke, but still significant. This reflects common risk factors and also increased cardiovascular stress after intracranial events

Answer 191

The most significant risk to the patient is a second ischaemic event causing permanent disability (stroke) TIA is also frequently indicative of underlying cardiac or atherosclerotic disease

Answer 192

A facial pain syndrome in the distribution of ≥1 divisions of the trigeminal nerve

Answer 193

Majority of patients (80-90%): trigeminal nerve compression Other causes include: -Demyelinating disease such as MS, demyelinating plaques in the pons that encompass the root entry zone of the trigeminal nerve -Brainstem lesions: brainstem infarcts and amyloid or calcium deposition along the trigeminal sensory pathway

Answer 194

Incidence is 4 to 13 per 100,000 in the UK | Slight female predominance and rates appear to increase with age

Answer 195

Facial pain: restriction to trigeminal distributions, bilateral is more common - Ophthalmic - Maxillary - Mandibular

Answer 196

Dermatomal pattern of pain of the trigeminal nerve | Sensory/motor changes: suggestive of pathological cause

Answer 197

Usually clinical diagnosis If examination is suggestive of pathology: - Intra oral x-ray: exclude dental cause of pain - MRI: may demonstrate presence of abnormal vessel loop in association with the trigeminal nerve, presence of other pathologies (e.g., tumour, infarct, multiple sclerosis plaque) - Trigeminal reflex testing: highly suggestive of symptomatic trigeminal neuralgia

Answer 198

A neurological emergency resulting from thiamine deficiency with varied neurocognitive manifestations, typically involving mental status changes and gait and oculomotor dysfunction

Answer 199

An acute or sub-acute deficiency of thiamine in a susceptible person is the usual precipitant cause Thiamine deficiency could be a result of: - decreased intake (either oral or parenteral) - relative deficiency due to increased demand - malabsorption from the GI tract

Answer 200

The prevalence according to clinical studies is 0.04% to 0.13% -possible underdiagnosis of this condition The prevalence is higher in patients with: -Alcohol dependence (12.5%) -AIDS (10%) -Bone marrow transplantation (6%) The prevalence is higher in males, with a male:female ratio of 1.7:1

Answer 201

``` Cognitive dysfunction: -Mental slowing -Impaired concentration -Apathy -Frank confusion -Mild irritability -Delirium Acute psychosis: may be the only presenting manifestation in some patients and is often confused with alcohol intoxication Alcohol dependance ```

Answer 202

Ocular motor findings: These include gaze palsies, sixth nerve palsies, and impaired vestibulo-ocular reflexes, and affect one third of patients Confusion/delirium/psychosis

Answer 203

Bloods: - FBC: an infection may trigger acute decompensation of thiamine deficiency - Glucose: usually normal unless there are co-existent conditions e.g. alcoholic liver disease, acute alcohol intoxication, sepsis - U&Es: normal at presentation, abnormal if not treated or in late-presenting disease due to hypotension and lactic acidosis may cause renal dysfunction - LFTs: elevated if patient has a history of chronic alcohol use - Ammonia: metabolic encephalopathy due to hyperammonaemia can easily mimic or be confused with Wernicke's encephalopathy - Blood alcohol level: an elevated blood alcohol level may coexist with Wernicke's encephalopathy - Blood thiamine (and its metabolites): low *confirm the diagnosis* *Therapeutic trial of parenteral thiamine: clinical response seen in a few hours or days MRI/CT/Lumbar puncture: exclude other pathologies: excluding meningitis, encephalitis, and subarachnoid haemorrhage. Thalamic lesions, involvement of the mammillary bodies

Neuroscience Flashcards

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