Neuro longs Flashcards

1
Q

MS
-dx
-clinical features
-mx
-pregnancy
-ddx

A

DX:
Dissemination in time and space of episodes and lesions.
RRMS, PPMS, SPMS, benign MS, NMO (Aquaporin 4 ab), MOG (myelin oligodendrocyte glucoprotein ab)
MRI: T2 hyperintense.
Evoked potentials: visual, somatosensory, brainstem.
LP: oligoclonal bands, IgG

CLINICAL FEATURES:
-optic neuritis
-INO
-myelopathy
-lhermitte’s and uhthoff’s
-exercise induced
-trigeminal neuralgia
-painful spasms
-cerebellar
-UMN

MX:
Bladder
- Spasticity (baclofen)
- Urgency (amitriptyline)
Tic douleroux (carbamazepine)
Facial spasm (physio)
Intention tremor (propranolol, clonazepam)
DMTs: beta interferon, copolymer 1, natalizumab (progressive multifocal leucoencephalopathy, JC virus), IVIG, alemtuzumab (T/B cell depletion, ITP, thyroid dx, rare infections) / rituximab / ocrelizumab, mitoxantrone.
Oral: fingolimod, teriflunomide (TB, HTN, alopecia, diarrhoea), dimethyl fumarate
Autologous SCT.
Acute: steroids.

Pregnancy: copaxone / natalizumab, discontinue disease modifying therapy, teriflunomide X grade, caution GnRH protocol for IVF (increased MS).

DDX:
-NMOSD and MOG ab
-Inflammatory dx (SLE, sjogren, PAN)
-infectious (HIV, HTLV1, PML, syphilis, lyme)
-granulomatous (sarcoid, GPA)
-myelin (adrenoleukodystrophy, metachromatic leucodystrophy)
-other (SCA, vit B12 def, vascular dx, CADASIL).

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2
Q

Spinal cord injury complications

A

Respiratory

Cardiovascular
- Orthostatic hypotension
- Autonomic dysreflexia

Bladder

Bowel

Spasticity

Pain syndromes
- nociceptive pain
- neuropathic pain
- pain treatment

Pressure ulcers

Osteoporosis and bone fractures

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3
Q

Myasthenia Gravis.

A

HISTORY.
-eye: diplopia, ptosis
-bulbar: dysphagia, dysarthria, fatigue
-neck: dropped head
-limb girdle: proximal muscle weakness, fatigueability
-anaesthesia
-thymectomy
-drugs interfering with NMJ
-autoimmune dx (SLE, RA)

EXAMINATION.
-muscle fatigue (ptosis, extraocular muscles, bulbar, facial, dysarthria, prox muscles)
-thymectomy scar
-reflexes preserved

INVESTIGATIONS.
-ice pack test (ptosis)
-AChR-ab, anti-MUSK (muscle-specific kinase), LRP4 (low density lipoprotein receptor-related protein 4)
-EMG: decrementation
-thymoma Ix: CXR, thoracic CT or MRI.
-autoimmune screen (ANA, RF, Anti-dsDNA, TFT)
-PFTs

DDx.
-ocular muscle dystrophy
-lambert eaton syndrome

TREATMENT.
-pyridostigmine (acetylcholinesterase inhibitor)
-increase potassium (supplement, pot sparing diuretics)
-avoid meds interfering with NMJ transmission (streptomycin, gentamicin, quinidine, procainamide, beta blockers).
-vaccination (avoid live)
-myasthenic crisis: triggers, treatment, ICU for NIV and PLEX.
-excessive anticholinesterase tx (cholinergic crisis). Cease meds and monitor muscle strength.

Disease suppressing.
-steroids (worse 7-10/7)
-immunosuppression: azathioprine, ciclosporin, mycophenolate.
-thymectomy
-IVIG, rituximab
-PLEX (myasthenic crisis, periop or peripartum).

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4
Q

Lambert Eaton Syndrome
-associations
-ab
-EMG
-weakness pattern

A

ASSOCIATIONS.
Paraneoplastic or autoimmune (SCLC).

AB: VGCC ab increase ACh.

EMG: increment with increased frequency of stimulation.

WEAKNESS PATTERN:
Proximal muscle weakness, improves with use.
Ocular/ bulbar last.

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5
Q

Parkinsons

DDx: MSA, PSP, CBS, lewy body dementia, drug-induced, vascular, huntingtons, spinocerebellar ataxia, wilsons,

A

Motor:
Rest tremor
Bradykinesia with decrement
Rigidity “lead pipe” velocity independent
Gait: freezing, failure to initiate

Non-motor:
-autonomic dysfunction
-sleep disorder
-sensory symptoms
-neuropsychiatric symptoms

Determinants of QoL: depression, dressing, apathy, pain, fatigue.

Mx: levodopa, dopamine agonists (apomorphine, pramipexole, ropinirole, rotigotine patch), monoamine oxidase inhibitors (MOA, selegile, rasagiline), amantadine, anticholinergics

C/o impulse control disorders = gambling, hypersexual, binge eating, shopping
C/o punding = repetitive behaviours.

Red flags:
-symmetric
-early falls
-dysphagia or dysarthria
-urinary incontinence
-early dementia
-no tremor

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6
Q

MSA-C
MSA-P

A

MSA-P: erectile dysfunction, urinary incontinence, postural hypotension, early instability, pyramidal/cerebellar signs, poor levodopa response, rapid progression, minimal tremor.

Hot cross bun sign (pons)
-MSA
-spinocerebellar atrophy
-parkinsonism with vasculitis
-creutzfeldt-Jakob disease

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7
Q

PSP

A

early falls
supranuclear down gaze palsy
postural instability
frontal dementia

PSP-P
asymmetric
tremor
levodopa response
better prognosis

MRI: hummingbird

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8
Q

corticobasal syndrome = corticobasal degeneration

A

asymmetric rigid jerking arm (alien limb)
apraxia
frontal lobe dementia
limb pain / cortical sensory loss
supranuclear gaze palsy
myoclonus
oculomotor apraxia (saccade delay)

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8
Q

lewy body dementia

A

cognitive decline fluctuating
visual hallucinations
parkinsonism
RBD sleep

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9
Q

drug induced parkinsonism

A

neuroleptics

antiemetics

lithium

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10
Q

CADASIL

A

cerebral
autosomal
dominant
arteriopathy with subcortical
infarcts and leukoencephalopathy

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11
Q

Epilepsy

A

Pregnancy: lamotrigine better.
Bad = multiple, phenobarbitone, valproate.

Driving: seizure.
-private: GP / specialist ax -> factors that reduce crash risk NO 1 year.
-commercial: specialist ax -> factors that reduce crash risk NO 10 years.
-conditional license

Driving: epilepsy treated for the first time.
-6/12 post seizure.

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12
Q

Peripheral nerve disease
-acute
-subacute
-chronic

A

Acute: GBS, vasculitis, prophyria, infectious (lyme), toxic/drug
Subacute: toxic, nutritional, malignancy, paraneoplastic, metabolic
Chronic: inherited

Course:
-monophasic
-relapsing/remitting:
. CIDP
. Hereditary neuropathy with liability to pressure palsy, porphyria

Mononeuritis multiplex:
-diabetes
-vasculitis
-leprosy
-sarcoidosis
-demyelinating neuropathy
-hereditary liability to pressure palsies
-neurofibromatosis
-entrapment/compressive neuropathy

motor neurons
-MND
-poliomyelitis
-lead
-MMNCB
-botulism
-drugs (vincristine, dapsone, amiodarone)

sensory neurones
-rare
-diabetes, b12, b6 excess
-hereditary
-HIV
-amyloid, sarcoid
-sjogren
-paraneoplastic

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13
Q

Hereditary neuropathy with liability to pressure palsies (HNLPP)

A

focal pressure palsy after minor trauma
entrapment neuropathy
demyelinating peripheral neuropathy
autosomal dominant

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14
Q

Stiff person syndrome

A

GAD antibodies
Assoc. w T1DM, pernicious anaemia, thyroid, coeliac, vitiligo.
Stiff
Spasms

C/o autonomic failure (temp, bowel, bladder).

Tx: benzo, gabapentin, baclofen, pregabalin.

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15
Q

Neurofibromatosis
-NF1 and NF2
-autosomal dominant inheritance
-NF1: skin changes + optic glioma
-NF2: vestibular schwannoma, meningioma

A

NF1:
-cafe au laite spots, neurofibromas, Lisch nodules
-optic glioma
-tx: monitor, surgical removal, opthalmology, cognitive/behavioural disturbance.

NF2:
-vestibular schwannoma (may impact facial nerve), meningioma
-tx: audiometry, opthalmology, MRI. Surgery, radiation, bevacizumab (VEGF inhibitor).

16
Q

Posterior reversible encephalopathy syndrome (PRES)
-cerebral dysregulation
-impacting posterior (parrieto-occipital) lobes predominantly

A

Acutely altered mental state
-decreased consciousness
-seizure
-visual disturbance
-headache

Triggers
-hypertension
-pre-eclampsia
-kidney disease
-sepsis

Mx: BP lowering in monitored environment

17
Q

MRI interpretation

A

T1 and T2 weighted images
- T1: ONE bright fat tissue
- T2: TWO bright fat and water tissues (WW2 = water is white in T2)
DWI: acute ischemia (sensitive for water)
FLAIR: periventricular areas, sulci and gyri (MS plaques, oedema post-stroke).