neurology Flashcards
which AED is associated with steven -johnson syndrome?
Lamotrigine
what are the features of wallenberg syndrome?
- posterior inferior cerebellar artery.
Cerebellar features
- ataxia
- nystagmus
Brainstem features
- ipsilateral: dysphagia, facial numbness, cranial nerve palsy e.g. Horner’s
- contralateral: limb sensory loss
whats the difference in features seen in Anterior inferior cerebellar artery occlusion vs PICA?
The main difference between AICA and PICA is that AICA involves facial paralysis andPICA involve Nucleus Ambiguous ( Hoarseness, loss of gag, dysphagia). Other involvement(vestibular,spinothalamic,Horner,trigeminal and inferior cerebellr peduncle) are common among both.
what are the features of ataxisa telengiectasia?
- cerebellar ataxia
- telangiectasia (spider angiomas)
- IgA deficiency resulting in recurrent chest infections
- 10% risk of developing malignancy, lymphoma or leukaemia, but also non-lymphoid tumours
what is the most common cardiac complication of myotonic dystrophy?
heart block - PR interval prolonged
cardiomyopathy
what is conduction aphasia?
The arcuate fasciculus is a bundle of axons that generally connects Broca’s area and the Wernicke’s area. Lesions in this area result in conduction aphasia.
Characterised by fluent speech, relatively intact comprehension and poor repetition. The main deficit in this scenario is impaired repetition.
Good comprehension and good speech but poor repetition
what are the features of CJD?
myoclonus + dementia
what are the investigations that help confirm CJD
MRI: hyperintense signals in the basal ganglia and thalamus
EEG: biphasic, high amplitude sharp waves (only in sporadic CJD)
what are the features of variant CJD?
psychological symptoms such as anxiety, withdrawal and dysphonia are the most common presenting features
younger patients
The typical presentation is that of a younger patient with progressive dementia (less rapid the sporadic CJD) with myoclonus and, in the later stages, mutism and vertical upgaze palsy (found in 50%)
MRI brain reveals a characteristic ‘hockey stick sign’ where the pulvinar region and dorsomedial thalamus are hyperintense on T2-weighted imaging (or pulvinar sign where the pulvinar region is hyperintense only)
what condition causes hypertension in patients with neurofibromatosis?
Pheochromocytoma is associated with 1, 2, 3
- NF-1
- MEN-2
- VHL (chromosome 3)
tx for generalised tonic clonic seiures?
male: sodium valproate
female : lamotrigine or levetiracetam
tx for focal seizures?
1st - lamotrigine or levetiracetam
2nd - carbamazepine, oxcarbazepine or zonisamide
tx for absence seizures?
1st - ethosuxamide
2nd - male - sodium valproate
female - levetiracetam
tx for myoclonic seizures?
males: sodium valproate
females: levetiracetam
tx for atonic seziures?
males: sodium valproate
females: lamotrigine
what tracts are affected by subacute degeneration of the spinal cord?
dorsal columns, lateral corticospinal tracts and spinocerebellar tracts..
which drugs may exacerbate myaesthenia gravis?
- penicillamine
- quinidine, procainamide
beta-blockers - lithium
- phenytoin
- antibiotics: gentamicin, macrolides, quinolones, tetracyclines
what are features of ramsay hunt syndrome?
- facial nerve palsy
- auricular pain
- vesicular rash around the ear
- vertigo and tinnitus
common regional lesion symptoms of strokes?
Astereognosis = parietal.
Visual agnosia = occipital.
Disinhibition = frontal.
Broca’s = frontal.
what is the chromosomal abnormallity seen in von hippel lindau syndrome?
VHL tumour suppressor gene situated on chromosome 3 and predisposes patients to visceral cysts and tumours
associated with renal cell carcinoma
what is used in the prophylaxis of cluser headaches?
verapamil
what are the side effects of sodium valproate?
VALPROATE
Vomiting
Alopecia
Liver toxicity
Pancreatitis/ Pancytopenia
Retention of fats (weight gain)
Oedema (peripheral oedema)
Anorexia
Tremor
Enzyme inhibition
how do you differentiate transient global amnesia from transient psychogenic amnesia?
Transient global amnesia is a clinical syndrome of uncertain aetiology, characterised by a discrete episode lasting for a few hours (always less than 24 hours) of anterograde amnesia, retrograde amnesia, repetitive questioning with an absence of other cognitive or neurological impairments.
person with psychogenic amnesia is profoundly unable to remember personal information about themselves; there is a lack of conscious self-knowledge which affects even simple self-knowledge
what is the most common cause of vascular cognitive impairment in the young?
cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADSIL)
an autosomal dominant condition caused by a NOTCH3 mutation on chromosome 19
features: migraine with aura, and a family history of such, which can ultimately lead to stroke-like features and is a recognised important cause of stroke in the young
It usually presents with the onset of migraines in middle age, followed by recurrent TIAs and strokes, and resulting in neuro-cognitive decline, psychiatric problems and dementia.
MRI often shows multiple widespread hyper-intense lesions in the white matter, basal ganglia, thalamus and pons.
what is the tx for restless leg syndrome?
dopamine agonists - pramipexole, ropinorle
benzodiazepine
gabapentin
what is the other cause of restless leg syndrome that needs to be rules out?
iron deficiency anaemia - check ferritin
what are the antibodies seen in lambert -eaton syndrome?
Lambert-Eaton syndrome - antibodies against the voltage-gated Ca2+ channels (anti VGCC)
what are the paraneoplastic syndromes of the nervous system and how do they present
Lambert-Eaton myasthenic syndrome - small cell lung cancer
Anti-Hu - small cell lung carcinoma and neuroblastoma
sx: sensory neuropathy - painful
anti H : imagine H sticks as 2 lungs or 2 brain hemispheres
anti Yo
associated with ovarian and breast cancer
-cerebellar syndrome
anti Yo : imagine Y as ladys private organ
anti-GAD
associated with breast, colorectal and small cell lung
stiff person syndrome or diffuse hypertonia
anti GAD in Arabic means a serious person:: so a serious person = stiffed
anti RI
associated iwth breast and small cell lung carcinoma
ocular opsoclonus- myclonus
what medications are used in motor sx of parkinsons that do not affect the pateint’s life?
dopamine recptor agonists - ropinirole
what is foster-kennedy syndrome?
frontal lobe tumour - usually a meningioma in this age group - leading to ipsilateral optic atrophy and papilloedema of the contralateral optic nerve. The reason for the optic atrophy is as a result of direct damage from the space occupying lesion. Other causes are AVMs and juvenile nasopharyngeal angiofibroma.
what is gerstmann syndrome?
Gerstmann syndrome is a constellation of acalculia, right-left disorientation, finger agnosia and agraphia
This occurs as a result of a deficit in the angular and supramarginal gyri between the dominant parietal and temporal lobes
what is the drug used to treat drug induced parkinsonism?
trihexyphenidyl / Benzhexol
antimuscuranic - used to treat tremor and rigidity - simialr to procyclidine
quick way to think of qundrantonopia lesions?
PITS (Parietal-Inferior, Temporal-Superior)
what are the causes of cerebellar syndrome?
Friedreich’s ataxia, ataxic telangiectasia
neoplastic: cerebellar haemangioma
stroke
alcohol
multiple sclerosis
hypothyroidism
drugs: phenytoin, lead poisoning
paraneoplastic e.g. secondary to lung cancer
what are the features of myotonic dystrophy?
distal weakness initially
autosomal dominant
diabetes
dysarthria
what causes predominantly sensory peripheral neuropathy?
diabetes
uraemia
leprosy
alcoholism
vitamin B12 deficiency
amyloidosis
what is wallenberg syndrome?
Ipsilateral: facial pain and temperature loss
Contralateral: limb/torso pain and temperature loss
Ataxia, nystagmus
Posterior inferior cerebellar artery
what is weber syndrome?
Ipsilateral CN III palsy
Contralateral weakness of upper and lower extremity
what cancer is associated with anti-NMDA encephalitis?
ovarian teratoma
what are the typical repeat expansions seen in neurological diseases?
CAG - huntington’s disease
GAA - friedrich ataxia
CTG - myotonic dystropy
CGG - fragile x syndrome
how do you differentiate neuromyelitis optica and MS?
both are relapsing remitting demyelinating CNS disorders
Typically affects the optic nerves and the cervical spine
Diagnosis requires bilateral optic neuritis, myelitis and 2 of the following 3 criteria:
1. Spinal cord lesion involving 3 or more spinal levels
2. Initially normal MRI brain
3. Aquaporin 4 positive serum antibody
what is the treatment for neuromyelitis optica?
immunosuppresants - with anti CD20 agent - rituximab
how does riluzole function?
prevents stimulation of glutamate receptors
how does baclofen work?
Agonist o GABA recpetors
thereby inhibiting the release of excitatory neurotransmitters. This results in the blocking of mono- and polysynaptic reflex transmission
what is the cause of ramsay hunt syndrome?
varicella zoster virus
what segments of the spinal cord are affected in subacute degeneration of the cord?
posterior and lateral columns of the spinal cord
Damage to the posterior columns - loss of proprioception, light touch and vibration sense (sensory ataxia and a positive Romberg’s test).
Damage to lateral columns - spastic weakness and upgoing plantars (UMN signs).
Damage to peripheral nerves - absent ankle and knee jerks (LMN signs).
what are the drugs that exacerbate myasthenia gravis?
M PPP QQ LGBT
macrolide
procainamide, penicilliamine, phenytoin
quinolone, quinidine
Lithium, Gentamycin, beta blockers, tetracycline
what is affected in common peroneal nerve lesion?
PED
- weakness of foot eversion
- weakness of foot dorsiflexion
- weakness of extensor hallucis longus
what drugs can cause tinnitus?
Aspirin/NSAIDs
Aminoglycosides
Loop diuretics
Quinine
what drugs cause miosis?
opiates
parasympathomimetics: pilocarpine
organophosphate toxicity
features of Anterior spinal artery occlusion?
- Bilateral spastic paresis
- Bilateral loss of pain and temperature sensation
GBC CSF results?
increase in protein without increase in white blood cells
