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neuro Flashcards

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1
Q

Lambert-Eaton myasthenic syndrome

A
  • associated with small cell lung cancer (also breast and ovarian)
  • antibody directed against pre-synaptic voltage gated calcium channel in the peripheral nervous system
  • can also occur independently as autoimmune disorder

improves with reinforcement unlike MG

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2
Q

Anti-Hu

A
  • associated with small cell lung carcinoma and neuroblastomas
  • sensory neuropathy - may be painful
  • cerebellar syndrome
  • encephalomyelitis
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3
Q

Anti-Yo

A
  • associated with ovarian and breast cancer

* cerebellar syndrome

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4
Q

Anti-GAD antibody

A
  • associated with breast, colorectal and small cell lung carcinoma
  • stiff person’s syndrome or diffuse hypertonia
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5
Q

Anti-Ri

A
  • associated with breast and small cell lung carcinoma

* ocular opsoclonus-myoclonus

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6
Q

Purkinje cell antibody

A
  • peripheral neuropathy in breast cancer
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7
Q

restless leg syndrome causes and associations

+ Mx

A 
Causes and associations
•there is a positive family history in 50% of patients with idiopathic RLS
•iron deficiency anaemia
•uraemia
•diabetes mellitus
•pregnancy

The diagnosis is clinical although bloods such as ferritin to exclude iron deficiency anaemia may be appropriate

Management
•simple measures: walking, stretching, massaging affected limbs
•treat any iron deficiency
•dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole)
•benzodiazepines
•gabapentin

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8
Q

Trigeminal neuralgia

A

first line treatment carbamazepine

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9
Q

CADASIL

A

cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy
rare cause of multi-infarct dementia
patients often present with migraine

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10
Q

Syringomyelia

A

cape like loss of pain and temperature sensation (spinothalamic tract decussating at the anterior white commisure of the spine

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11
Q

increase in protein in CSF

A

Guillain-Barre syndrome
tuberculous, fungal and bacterial meningitis
Froin’s syndrome*
viral encephalitis

*describes an increase in CSF protein below a spinal canal blockage (e.g. tumour, disc, infection)

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12
Q

rapid onset dementia and myoclonbus

A

creutzfeld jakob disease

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13
Q

drug induced parkinsons treatment for tremor

A

Benzhexol (anti muscarinic) is now more commonly referred to as trihexyphenidyl. It is now mainly used for drug-induced parkinsonism rather than idiopathic Parkinson’s disease

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14
Q

down or upbeat nystagmus

A

Upbeat nystagmus
cerebellar vermis lesions

Downbeat nystagmus - foramen magnum lesions
Arnold-Chiari malformation

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15
Q

Palatal myoclonus

A

olivary nucleus degeneration
Palatal myoclonus is a specific feature of hypertrophic olivary degeneration. This is caused by a lesion in the triangle of Guillain and Mollaret (triangle linking the inferior olivary nucleus, red nucleus and the contralateral dentate nucleus). An MRI brain is the gold standard imaging for this lesion.

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16
Q

Miller Fisher syndrome

A

variant of Guillain-Barre syndrome
associated with ophthalmoplegia, areflexia and ataxia. The eye muscles are typically affected first
usually presents as a descending paralysis rather than ascending as seen in other forms of Guillain-Barre syndrome
anti-GQ1b antibodies are present in 90% of cases

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17
Q

Migraine

A

A At least 5 attacks fulfilling criteria B-D
B Headache attacks lasting 4-72 hours* (untreated or unsuccessfully treated)
C Headache has at least two of the following characteristics:
1. unilateral location*
2. pulsating quality (i.e., varying with the heartbeat)
3. moderate or severe pain intensity
4. aggravation by or causing avoidance of routine physical activity (e.g., walking or climbing stairs)
D During headache at least one of the following:
1. nausea and/or vomiting*
2. photophobia and phonophobia
E Not attributed to another disorder (history and examination do not suggest a secondary headache disorder or, if they do, it is ruled out by appropriate investigations or headache attacks do not occur for the first time in close temporal relation to the other disorder)

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18
Q

SAH when to LP

A

after 12 hours

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19
Q

tuberous sclerosis features

A

Cutaneous features
depigmented ‘ash-leaf’ spots which fluoresce under UV light
roughened patches of skin over lumbar spine (Shagreen patches)
adenoma sebaceum (angiofibromas): butterfly distribution over nose
fibromata beneath nails (subungual fibromata)
café-au-lait spots* may be seen

Neurological features
developmental delay
epilepsy (infantile spasms or partial)
intellectual impairment

Also
retinal hamartomas: dense white areas on retina (phakomata)
rhabdomyomas of the heart
gliomatous changes can occur in the brain lesions
polycystic kidneys, renal angiomyolipomata
lymphangioleiomyomatosis: multiple lung cysts

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20
Q

hyperacusis

A

sensitivity to noise - stapedius muscle after facial nerve injury

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21
Q

after a stroke

A

thrombolyse within 4.5 hours IF haemorrhage has been excluded

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22
Q

Mx of lambert eaton

A

Management
treatment of underlying cancer
immunosuppression, for example with prednisolone and/or azathioprine
3,4-diaminopyridine is currently being trialled**
intravenous immunoglobulin therapy and plasma exchange may be beneficial

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23
Q

Multiple sclerosis acute episode treatment

A

IV methylprednisolone

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24
Q

Disease modifying MS medications

A

Beta-interferon has been shown to reduce the relapse rate by up to 30%. Certain criteria have to be met before it is used:
relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided
secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided)
reduces number of relapses and MRI changes, however doesn’t reduce overall disability

Other drugs used in the management of multiple sclerosis include:
glatiramer acetate: immunomodulating drug - acts as an ‘immune decoy’
natalizumab: a recombinant monoclonal antibody that antagonises Alpha-4 Beta-1-integrin found on the surface of leucocytes, thus inhibiting migration of leucocytes across the endothelium across the blood-brain barrier
fingolimod: sphingosine 1-phosphate receptor modulator, prevents lymphocytes from leaving lymph nodes. An oral formulation is available

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25
MS Symptomatic treatmentes
Fatigue - CBT and amantidine Spasticity - baclofen and gabapentin Bladder dysfunction may take the form of urgency, incontinence, overflow etc guidelines stress the importance of getting an ultrasound first to assess bladder emptying - anticholinergics may worsen symptoms in some patients if significant residual volume → intermittent self-catheterisation if no significant residual volume → anticholinergics may improve urinary frequency Oscillopsia (visual fields apper to oscillate) gabapentin is first-line
26
Temporal lobe lesion
Wernicke's aphasia: this area 'forms' the speech before 'sending it' to Brocas area. Lesions result in word substituion, neologisms but speech remains fluent superior homonymous quadrantanopia auditory agnosia prosopagnosia (difficulty recognising faces)
27
Occipital lobe lesions
homonymous hemianopia (with macula sparing) cortical blindness visual agnosia
28
Parietal lobe lesions
``` sensory inattention apraxias astereognosis (tactile agnosia) inferior homonymous quadrantanopia Gerstmann's syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation ```
29
Frontal lobe lesion
``` expressive (Broca's) aphasia: located on the posterior aspect of the frontal lobe, in the inferior frontal gyrus. Speech is non-fluent, laboured, and halting disinhibition perseveration anosmia inability to generate a list ```
30
features of MS
Visual optic neuritis: common presenting feature optic atrophy Uhthoff's phenomenon: worsening of vision following rise in body temperature internuclear ophthalmoplegia ``` Sensory pins/needles numbness trigeminal neuralgia Lhermitte's syndrome: paraesthesiae in limbs on neck flexion ``` Motor spastic weakness: most commonly seen in the legs Cerebellar ataxia: more often seen during an acute relapse than as a presenting symptom tremor Others urinary incontinence sexual dysfunction intellectual deterioration
31
Guillain barre syndrome monitoring and Rx
monitor resp function with FVC Management plasma exchange IV immunoglobulins (IVIG): as effective as plasma exchange. No benefit in combining both treatments. IVIG may be easier to administer and tends to have fewer side-effects steroids and immunosuppressants have not been shown to be beneficial FVC regularly to monitor respiratory function
32
Chorea causes and common in
Chorea is caused by damage to the basal ganglia, especially the caudate nucleus. ``` Causes of chorea Huntington's disease, Wilson's disease, ataxic telangiectasia SLE, anti-phospholipid syndrome rheumatic fever: Sydenham's chorea drugs: oral contraceptive pill, L-dopa, antipsychotics neuroacanthocytosis pregnancy: chorea gravidarum thyrotoxicosis polycythaemia rubra vera carbon monoxide poisoning cerebrovascular disease ```
33
Brachial neuritis
Brachial neuritis is characterized by acute onset of unilateral (occasionally bilateral) severe pain, followed by shoulder and scapular weakness several days later. Sensory changes are usually minimal. There may be subsequent rapid wasting of the arm muscles in accordance to which nerve is involved. Precipitating factors include recent trauma, infection, surgery, or even vaccination. Rarely it may be hereditary. The prognosis is usually good except when the phrenic nerve is involved since this can result in significant breathlessness.
34
Brachial neuritis
Brachial neuritis is characterized by acute onset of unilateral (occasionally bilateral) severe pain, followed by shoulder and scapular weakness several days later. Sensory changes are usually minimal. There may be subsequent rapid wasting of the arm muscles in accordance to which nerve is involved. Precipitating factors include recent trauma, infection, surgery, or even vaccination. Rarely it may be hereditary. The prognosis is usually good except when the phrenic nerve is involved since this can result in significant breathlessness.
35
after 14 days ischaemic stroke
clopidogrel and statin clopidogrel can be swapped for aspirin/dipyridamole
36
myasthenic crisis can be ppt by which drugs
The following drugs may exacerbate myasthenia: penicillamine quinidine, procainamide beta-blockers lithium phenytoin antibiotics: gentamicin, macrolides, quinolones, tetracyclines
37
motor neuron disease drug
Riluzole prevents stimulation of glutamate receptors used mainly in amyotrophic lateral sclerosis prolongs life by about 3 months
38
subthalamic region lesion
hemiballism involuntary, sudden, jerking movements which occur contralateral to the side of the lesion. The ballisic movements primarily affect the proximal limb musculature whilst the distal muscles may display more choreiform-like movements Symptoms may decrease whilst the patient is asleep. Antidopaminergic agents (e.g. Haloperidol) are the mainstay of treatment
39
loss of muscle tone after laughing
cataplexy
40
otogenic diseases like mastoiditis and sinusitis infections can cause
cerebellar abscesses
41
neurolepti malignant syndrome treatment
dantrolene
42
Cavernous sinus thrombosis
other causes of cavernous sinus syndrome: local infection (e.g. sinusitis), neoplasia, trauma periorbital oedema ophthalmoplegia: 6th nerve damage typically occurs before 3rd & 4th trigeminal nerve involvement may lead to hyperaesthesia of upper face and eye pain central retinal vein thrombosis
43
Natalizumab risk
reactivate JC virus causing progressive multifocal leucoencephalopathy JC virus can cause demyelination
44
malignancy + psychiatric features including agitation, hallucinations, delusions and disordered thinking; seizures, insomnia, dyskinesias and autonomic instability
anti NMDA receptor encephalitis usually ovarian teratoma treat with immunosuppresion with intravenous steroids, immunoglobulins, rituximab, cyclophosphamide or plasma exchange, alone or in combination. Resection of teratoma is also therapeutic.
45
Transverse myelitis
inflammation across spinal cord causes - HIV viral infections: varicella-zoster, herpes simplex, cytomegalovirus, Epstein-Barr, influenza, echovirus, human immunodeficiency virus bacterial infections: syphilis, Lyme disease post-infectious (immune mediated) first symptom of multiple sclerosis (MS) or neuromyelitis optica (NMO)
46
is Guillain barre upper or lower
it's lower motor neuron disease (polyneuropathy)
47
Uhthoff's phenomenon
hot temperature, blurred vision (MS)
48
Lhermitte's sign
parasethesia of limbs on neck flexion
49
cerebellar syndrome
DANISH: D - Dysdiadochokinesia, Dysmetria (past-pointing), patients may appear 'Drunk' A - Ataxia (limb, truncal) N - Nystamus (horizontal = ipsilateral hemisphere) I - Intention tremour S - Slurred staccato speech, Scanning dysarthria H - Hypotonia
50
sodium valproate adverse effects
``` P450 inhibitor gastrointestinal: nausea increased appetite and weight gain alopecia: regrowth may be curly ataxia tremor hepatotoxicity pancreatitis thrombocytopaenia teratogenic hyponatraemia hyperammonemic encephalopathy: L-carnitine may be used as treatment if this develops ```
51
guillain barre pathogenesis
Pathogenesis cross reaction of antibodies with gangliosides in the peripheral nervous system correlation between anti-ganglioside antibody (e.g. anti-GM1) and clinical features has been demonstrated anti-GM1 antibodies in 25% of patients classically campylobacter jejuni
52
urinary incontinence dementia and bradyphrenia gait abnormality (may be similar to Parkinson's disease)
normal pressure hydrocephalus Management ventriculoperitoneal shunting around 10% of patients who have shunts experience significant complications such as seizures, infection and intracerebral haemorrhages
53
hypodense (dark), crescentic collection around the convexity of the brain
chronic subdural acute would be light (hyperdense)
54
pergolide
dopamine receptor agonist | causes pulmonary fibrosis
55
wernicke's encephalopathy
``` nystagmus (the most common ocular sign) ophthalmoplegia ataxia confusion, altered GCS peripheral sensory neuropathy ``` treat with thiamine
56
korsakoff's
wenicke's + amnesia + confabulation
57
thrombectomy target time
6 hours
58
brown sequard
Overview caused by lateral hemisection of the spinal cord Features ipsilateral weakness below lesion ipsilateral loss of proprioception and vibration sensation contralateral loss of pain and temperature sensation
59
absent ankle jerk and extensor plantars | upper and lower MN signs
``` Causes subacute combined degeneration of the cord motor neuron disease Friedreich's ataxia syringomyelia taboparesis (syphilis) conus medullaris lesion ```
60
Pituitary apoplexy
Pituitary apoplexy Sudden enlargement of pituitary tumour secondary to haemorrhage or infarction Features sudden onset headache similar to that seen in subarachnoid haemorrhage vomiting neck stiffness visual field defects: classically bitemporal superior quadrantic defect extraocular nerve palsies features of pituitary insufficiency e.g. Hypotension secondary to hypoadrenalism
61
head trauma with decreased GCS and then lucid interval
extradural haematoma (typically blunt force)
62
Generalised tonic-clonic seizures Rx
sodium valproate | 2nd line - lamotrigine, carbamazepine
63
Absence seizures* (Petit mal) Rx
sodium valproate or ethosuximide sodium valproate particularly effective if co-existent tonic-clonic seizures in primary generalised epilepsy not carbamazepine
64
Myoclonic seizures** | Rx
sodium valproate second line: clonazepam, lamotrigine not carbamazepione
65
focal seizure Rx
carbamazepine or lamotrigine | second line: levetiracetam, oxcarbazepine or sodium valproate
66
Arnold-Chiari malformation
Arnold-Chiari malformation describes the downward displacement, or herniation, of the cerebellar tonsils through the foramen magnum. Malformations may be congenital or acquired through trauma. Features non-communicating hydrocephalus may develop as a result of obstruction of cerebrospinal fluid (CSF) outflow headache syringomyelia
67
homonymous quadrantinopias
PITS parietal inferior temporal superior
68
PCKD and renal angiomyolipomata assw/
tuberous sclerosis
69
extradural haemorrhage which artery?
middle meningeal artery most likely
70
peripheral neuropathy (demyelinating) examples
Guillain-Barre syndrome chronic inflammatory demyelinating polyneuropathy (CIDP) amiodarone hereditary sensorimotor neuropathies (HSMN) type I paraprotein neuropathy
71
peripheral neuropathy axonal
``` alcohol diabetes mellitus* (can be both) vasculitis vitamin B12 deficiency* hereditary sensorimotor neuropathies (HSMN) type II ```
72
4th nerve palsy
Overview supplies superior oblique (depresses eye, moves inward) Features vertical diplopia subjective tilting of objects (torsional diplopia) classically noticed when reading a book or going downstairs the patient may develop a head tilt, which they may or may not be aware of when looking straight ahead, the affected eye appears to deviate upwards and is rotated outwards
73
Bitemporal hemianopia
lesion of optic chiasm upper quadrant defect > lower quadrant defect = inferior chiasmal compression, commonly a pituitary tumour lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a craniopharyngioma
74
Spastic paraparesis Spastic paraparesis describes a upper motor neuron pattern of weakness in the lower limbs
``` Causes demyelination e.g. multiple sclerosis cord compression: trauma, tumour parasagittal meningioma tropical spastic paraparesis transverse myelitis e.g. HIV syringomyelia hereditary spastic paraplegia osteoarthritis of the cervical spine ```
75
anti-GQ1b antibodies are present in 90% of cases
of Miller Fisher syndrome | GBC with ataxia, opthalmoplegia and areflexia
76
Von Hippel-Lindau syndrome
Von Hippel-Lindau (VHL) syndrome is an autosomal dominant condition predisposing to neoplasia. It is due to an abnormality in the VHL gene located on short arm of chromosome 3 Features cerebellar haemangiomas: these can cause subarachnoid haemorrhages retinal haemangiomas: vitreous haemorrhage renal cysts (premalignant) phaeochromocytoma extra-renal cysts: epididymal, pancreatic, hepatic endolymphatic sac tumours clear-cell renal cell carcinoma
77
migraine + COCP
significantly increased stroke risk
78
phenytoin side effect
peripheral neuropathy
79
acute onset vertigo what are you worried about
posterior circulation stroke
80
postural hypotension + parkinsons
Multi system atrophy
81
Jacksonion march
Focal highlights how it is focal epilepsy that involves abnormal electrical activity in just one part of the brain. It characteristically starts by affecting a peripheral body part such as a toe, finger or section of the lip and then spreads quickly 'marches' over the respective foot, hand or face. In some with Jacksonian march seizures (as in this case), the electrical disorder spreads over larger areas of the brain, causing the seizure to develop into a tonic-clonic seizure.
82
idiopathic raised ICP
``` Risk factors obesity female sex pregnancy drugs*: oral contraceptive pill, steroids, tetracycline, vitamin A, lithium ``` Management weight loss diuretics e.g. acetazolamide topiramate is also used, and has the added benefit of causing weight loss in most patients repeated lumbar puncture surgery: optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve. A lumboperitoneal or ventriculoperitoneal shunt may also be performed to reduce intracranial pressure
83
herpes symplex encephalitis Ix
CSF: lymphocytosis, elevated protein PCR for HSV CT: medial temporal and inferior frontal changes (e.g. petechial haemorrhages) - normal in one-third of patients MRI is better EEG pattern: lateralised periodic discharges at 2 Hz
84
motor neuron disease with worst prognosis
progressive bulbar palsy palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei carries worst prognosis
85
MND with upper motor neuron symptoms only
primary lateral sclerosis
86
Progressive muscular atrophy
MND LMN signs only affects distal muscles before proximal carries best prognosis
87
Amyotrophic lateral sclerosis (50% of patients)
typically LMN signs in arms and UMN signs in legs | in familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutase
88
myotonic dystrophy
``` General features myotonic facies (long, 'haggard' appearance) frontal balding bilateral ptosis cataracts dysarthria ``` ``` Other features myotonia (tonic spasm of muscle) weakness of arms and legs (distal initially) mild mental impairment diabetes mellitus testicular atrophy cardiac involvement: heart block, cardiomyopathy dysphagia ```
89
Neuromyelitis optica
Neuromyelitis optica (NMO) is monophasic or relapsing-remitting demyelinating CNS disorder Although previously thought to be a variant of multiple sclerosis, it is now recognised to be a distinct disease, particularly prevalent in Asian populations1. It typically involves the optic nerves and cervical spine, with imaging of the brain frequently normal. Vomiting is also a common presenting complaint. Diagnosis is requires bilateral optic neuritis, myelitis and 2 of the follow 3 criteria2: 1. Spinal cord lesion involving 3 or more spinal levels 2. Initially normal MRI brain 3. Aquaporin 4 positive serum antibody
90
alexia, acalculia, finger agnosia and right-left disorientation
Gerstmann's syndrome Parietal lobe lesion
91
``` Neurological features absent ankle jerks/extensor plantars cerebellar ataxia optic atrophy spinocerebellar tract degeneration ``` Other features hypertrophic obstructive cardiomyopathy (90%, most common cause of death) diabetes mellitus (10-20%) high-arched palate
Friedreich's ataxia
92
Lateral medullary syndrome
Posterior inferior cerebellar artery (PICA) Ipsilateral: facial pain and temperature loss Contralateral: limb/torso pain and temperature loss Ataxia, nystagmus
93
vertigo following viral infection no hearing loss no tinnitus
vestibular neuronitis need to rule out PICA Management vestibular rehabilitation exercises are the preferred treatment for patients who experience chronic symptoms buccal or intramuscular prochlorperazine is often used to provide rapid relief for severe cases a short oral course of prochlorperazine, or an antihistamine (cinnarizine, cyclizine, or promethazine) may be used to alleviate less severe cases
94
carotid artery endarterectomy:recommend if
patient has suffered stroke or TIA in the carotid territory and are not severely disabled should only be considered if carotid stenosis > 70% according ECST* criteria or > 50% according to NASCET** criteria
95
Meniere's disease Management ENT to confirm stop driving until control achieved acute attacks: buccal or intramuscular prochlorperazine. prevention: betahistine and vestibular rehabilitation exercises
recurrent episodes of vertigo, tinnitus and hearing loss (sensorineural). Vertigo is usually the prominent symptom a sensation of aural fullness or pressure is now recognised as being common other features include nystagmus and a positive Romberg test episodes last minutes to hours typically symptoms are unilateral but bilateral symptoms may develop after a number of years
96
alexia (inability to read), without agraphia (inability to write).
infarction of the left posterior cerebral artery which perfuses the splenium of the corpus callosum and left visual (occipital) cortex
97
Sydenhams chorea from which part of brain
caudate nucleus
98
Anterior spinal artery occlusion
1. Lateral corticospinal tracts 2. Lateral spinothalamic tracts 1. Bilateral spastic paresis 2. Bilateral loss of pain and temperature sensation

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