Neuro Exam Cram Flashcards by Olivia Wells

3 most common causes of ataxia?

Acute cerebellar ataxia
Drug intoxication
Guillain-Barre Syndrome

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2yo child presents with sudden inability to sit/walk with wide based gait. Coordination impaired. Viral illness 2/53 ago. Cause of ataxia?

Acute cerebellar ataxia

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Treatment of acute cerebellar ataxia?

Supportive, usually improved within 2-3 weeks however can last up to 3 months
Can sometimes give steroids/IVIG

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Most common degenerative ataxia?

Ataxia Telangiectasia

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18mo child now walking with narrow based gait, nystagmus and apraxia of horizontal gaze. Recurrent sinopulmonary infections. Telangiectasias in conjunctivae and on bridge of nose. High AFP. What dx?

Ataxia Telangiectasia

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Immune deficiency seen in Ataxia Telangiectasia?

Decreased secretory IgA
Diminished IgG2, Ig4 and IgE

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What malignancy seen in ataxia telangiectasia and what RF to avoid?

Lymphoreticular tumours, brain tumours
Highly radiation sensitive (avoid unnecessary imaging)

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Most common hereditary ataxia?

Friedrich’s Ataxia

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Gene mutation and inheritance of Friederich’s Ataxia?

AR
Trinucleotide repeat expansion GAA causing mutation in mitochondrial frataxin protein

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11yo child presents with ataxia, loss of lower limb reflexes and diminished sense of vibration in lower limbs. Dysarthric speech. Might have skeletal abnormalities or hypertrophic cardiomyopathy. What dx?

Friederich’s Ataxia

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Management of Frederich’s Ataxia?

Antioxidant therapy - coenzyme Q10
Vitamin E

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7yo boy presenting with fever, encephalopathy, ataxia, and behaviour/language disturbance in last 24hrs. Recent ED presentation with enterovirus.
MRI shows hyper-intense white matter abnormalities - mostly subcortical
CSF - increased protein
What dx?

Acute Disseminated Encephalomyelitis (ADEM)

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Management of ADEM?

?no management
Steroids - IV methylpred then a PO pred slow wean
IVIG if not responsive to steroids
Plasmapheresis if not responsive to either
Can consider rituximab, cyclophosphamide

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Risk of MS after ADEM?

2-10%
60-90% of children make full recovery without sequelae

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Child presents with bilateral leg weakness, hyperreflexia and clonus, urinary retention.
MRI shows demyelinating lesions of spinal cord with cord swelling and enhancement. Dx?

Transverse myelitis

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Treatment of transverse myelitis?

IV steroids with a slow PO steroid wean
IDC if urinary retention
Plasma exchange if not responsive to steroids

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Child presents with vision worsening over the last day or so, on testing has central scotoma and failed Ishihara tests with red numbers. Eye pain with eye movement. Had a viral illness 1/52 ago. Dx?

Optic neuritis

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Optic neuritis treatment?

Steroids (MRI B should be completed prior to commencing)

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Two arterial circulations in the brain and the point at which they meet?

Anterior internal carotid
Vertebrobasilar
Meet at Circle of Willis

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Risk factors for arterial ischaemic stroke?

Arteriopathy (50%) - moyamoya
Cardiac (25%) - inc PFO, post cath/surg, arryhtmia/endocarditis
Haematological - sickle, prothrombotic diseases

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Child presents with headache, diplopia, lethargy, then has a seizure in ED. Reading through their history you see they have a promthrombotic condition/maybe they have leukaemia and chemo regimen includes L-aparaginase. What dx?

Cerebral Venous SInus Thrombosis (CVST)

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Risk factors for CVST?

Pro-thrombotic conditions - V Leiden, protein C/S deficiency, anti phospholipid syndrome
Dehydration
Iron def anaemia
Drugs - L-asparaginse, COCP
Sepsis/DIC
Local infection - otitis media, mastoiditis
Trauma
Venous malformation

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Ix for CVST?

CT or MR venography

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Mx for CVST?

Heparin or enoxaparin, usually for 3 months with re-imaging for assessment.
30% have thrombus extension in first week
Regular ophthal exam for optic neuropathy secondary to raised ICP

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Epidural haematoma - what vessel injured?

Middle meningeal

Subdural hameatoma - what vessel injured?

Bridging vein

Subdural haematomas of variable age?

NAI

Child presents with focal neurological deficit and psych sx, angiogram demonstrates features of vasculitis. Dx?

CNS vasculitis - Childhood primary angitis of the CNS (cPACNS) Secondary CNS vasculitis

Bloods for Ix of CNS vasculitis?

Inflam markers - CRP ESR FBE Endothelial markers - vWF proposed biomarker of vasculitis (correlates w disease activity) Bacterial cultures/viral serology Complement/autoantibodies Pro-coagulant profile LP - CSF and opening pressures, neuronal antibodies

Treatment of CPACNS?

Corticosteroids (IV pulse therapy) Antithrombotic therapy - esp if large/medium vessel involvement

Child presents with headache, seizures. MRI demonstrates increased signal intensity of occipital lobe on T2 images. No hypertension. What dx?

Posterior reversible leukoencephalopathy syndrome (PRES)

Child severely encephalopathic. Presented to ED 1 week ago and resp swab confirmed influenza. Bloods largely unremarkable however liver enzymes raised with normal ammonia level. MRIB shows symmetrical lesions in the thalami. What dx?

Acute necrotising encephalopathy Often triggered by viral illness (HHV6, influenza) in genetically susceptible people RANBP2 gene

Treatment of acute necrotising encephalopathy?

Steroids & IVIG

NMDA encephalitis mechanism?

IgG antibodies against NR1 subunit of NMDA receptor causing disruption of function to GABAergic neurons.

NMDA encephalitis associated with what underlying disease?

Ovarian teratoma

LP findings in autoimmune encephalitis?

Increased protein, lymphocytic pleocytosis

Opsoclonus-myoclonus associated with what underlying disease?

Neuroblastoma

CSF is produced where?

Choroid plexus

CSF flow pattern?

Lateral ventricles - \> through foramen of munro to 3rd ventricle - \> via Silvian fissure to 4th ventricle - \> via lateral foramina to basal cistern

CSF resorbed by?

Mostly arachnid villi Some lymphatic channels & choroid plexus

Acute migraine management?

``` Paracetamol, environmental change Aspirin 1g (if \>12yrs) Sumitriptan IN (if \>12yrs) Chlorpromazine in 1L NS ```

Migraine prophylaxis indications?

Frequent or long lasting migraines, causing sig disability for diminished QoL Contraindication to acute therapies Risk of medication overuse Menstrual migraine

Prophylaxis for migraine?

Flunarizine (Ca ch blocker) Beta blockers (contraindicated in asthma/depression) Cryohepatidine (antihistamine/serotonin antagonist, anticholinergic and blocks Ca channels) Amitriptyline

Child presents with off balance, reaching for furnitures. Might be sweaty and vomit during episode. Episode lasts about 1min then resolves, usually will happen intermittently for days/week then go a longer period with out any episodes. Fhx migraine. What dx?

BPPV

Features of Bell's palsy and red flags to suggest alternative aetiology?

Facial nerve palsy - unilateral facial weakness WITHOUT forehead sparing - loss of taste anterior 2/3 of tongue - impaired lacrimation Red flags: - ipsilateral abducens nerve palsy (brainstem lesion) - forehead sparing (UMN lesion)

Common causes of bacterial meningitis in \<2mth and \>2mth olds?

\<2 mths: GBS, E. Coli, Listeria \>2 mths: Strep Pneumo, Neisseria, HiB

Most common neurological sequelae of bacterial meningitis?

Sensorineural hearing loss. Most commonly with pneumococcal meningitis. Needs audiol reveiew 6-8/52 post

RFs for CNS abscess?

Direct spread CNS abcess vs haematological spread?

Most common pathogens causing CNS abscess and Mx?

Streptococci - 60-70% Staph aureus - 10-15% Abx if \<2cm and \<\<2 weeks w normal ICP and no neuro sx. Cefotax & metro

Delayed motor milestones, difficulty climbing stairs, frequent falls. Calf hypertrophy. Learning difficulty/autism traits. CK very very high (\>20x ULN)

Complications of DMD?

Genetics of DMD?

Management of DMD?

Glucocorticoids - usually ofered at time of declines/frequent falling (4-6yrs) Nutritional optimisation Pulm optimisation - PFTs & prompt treatment of infections Cardiac - annual screening from 10yrs Ortho - scoliosis/contracture management

Differences between DMD and BMD?

DMD - out of frame genetic mutation BMD - in frame genetic mutation CK levels higher in DMD Symptom onset and wheelchair use is earlier in DMD than BMD Neck flexor muscles are presevered in BMD

EEG pattern in autoimmune encephalitis?

Tuberous Sclerosis genetics?

Autosomal dominant High rate of spont mutation TSC1 - harmatin (20%) TSC2 - tuberin (80%)

NF1 genetics?

Autosomal dominant however 50% de novo Complete penetrance with highly variable expression Germline NF1 mutation 17q11.2 - neurofibromin protein RAS/mapK pathway causing increased cell growth

Genetics of SMA?

Autosomal recessive SMN1 gene - homozygous deletion

3mo baby w feeding difficulties and respiratory insufficiency Frog leg posture Paucity of antigravity movements Tongue fasciculations Bright, alert face

SMA 1

Mechanism of neusinersin?

Modulation of SMN2 pre-RNA splicing "Antisense olinucleotide therapy"

Benefit of steroids in DMD?

Slows decline in muscle strength and function (walks for longer) Decreased risk of scoliosis Stabilisation of lung function and delays need for NIV Might reduce decline in cardiac function

Child with distal weakness and wasting Pes cavus Reduced/absent reflexes

Charcot Marie Tooth PMP22 on microarray diagnostic

Agenesis of corpus callosum Seizures horioretinal lacunae

Cobblestone lissencephaly Coloboma/cataract/microphthalmia

Walker-Warberg Syndrome