Chronic Neuro Flashcards by Ben Moore

What is MS?

Chronic inflammatory multifocal, demyelinating disease of the central nervous system of unknown cause, resulting in loss of myelin and oligodendorglial and axonal pathology

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Symptoms of MS?

Optic neuritis, motor weakness, sensory disturbances e.g. parasthesiae and fatigue

Diplopia, vertigo and nystagus, dysarthria, hemiparesis and hemisensory loss

Seizures and psychiatric disturbances

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Examinations for MS?

Fundoscopy = retro-bulbar neuritis in 2/3 and optic neuritis in 1/3

Lhermittes sign

Look for cerebellar dysfunction

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Where is MS common?

Nordic areas and high latititudes

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Risk Factors for MS?

HLA BLRB1*15
Women 
Nordic
Lack of vitain D
Other Autoimmune conditions

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What will MS show on MRI?

Demyleinated plaques in the white matter of the CNS show evidence of relapses

Gadolinium contrast allows differentiation in time

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How is MS diagnosed?

Absense of alternative diagnosis
Dissemination in time
Dissemination in Space

Clinic history/exam, MRI, CSF and eletrophysiology = VEPS

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What will MS show in CSF? Also electrophysiology?

Oligoclonal bands +ve only in the CSF.

Visual evoked potentials (VEPs)

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What is Myasthenia Gravis symptoms?

Muscles fatigue with use
= ptosis, diplopia, dysarthria, dysphagia +- SOB

Signs = fatigable muscles but normal reflexes

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Associations and Ix for MG?

Thymic hyperplasia = 70% and thymoma 10%

Bloods = anti-AChR or MuSK
EMG
CT/MRI

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What is Lambert Eaton Myasthenic Syndrome?

Immune system attacks the calcium channels on nerve endings for Ach release.

Weakness improves with repeased use of muscle

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Other names for MND?

ALS (amyotrophic lateral sclerosis), Lou Gehrigs and Charcots disease

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What is MND?

Chronic neurodegenerative condition causing muscle wastng, paralysis and death usually withing 3-5 years due to respiratory failure.

Ubiquinated proteins in the cystoplasm of Motor neurons

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Symptoms of MND?

Secondary muscle weakness of limbs, trunks and tongue and respiratory muscles
Bulbar signs = ipaired swallowing and speech
Spastic weakness and paralysis of skeletal muscle
Resp failure
Oculomotor, sensory and autonomic function spaired

BOTH UMN AND LMN

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What are UMN signs?

Increased weakness, no atrophy, increased reflexes, no fasciculatations, increased tones and extensor response present

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What are LMN signs?

Weakness, atrophy, decreased reflexes, fasciculations, decreased tone and absent etensor response

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Key signs in MND?

Wasting of the thenar hand msucles and wasting of tongue muscles

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Ix for MND?

EMG = fibrillation and fasciculations
CT?MRI for exclusion
Blood Test for exclusion e.g. B12/folate, lyme serology, CK assay

Muscle biopsy?

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Components of MND?

Progressive muscular atrophy, progressive bulbar palsy, pseudobulbar palsy and primary lateral scelrosis

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Features of progressive muscular atrophy?

LMN only

e.g. flair arm/flail foot syndrome

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Features of progressive bulbar palsy?

Cranial 11-12
Flacid tongue
Absent jaw-jerk
Nasal-donal duck-voice

Features of pseudobulbar palsy?

UMN for CN9-11
SLow tongue movements
Increased jaw jerk
Hot potato speech

Features of primary lateral sclerosis?

Loss of betz cells in otor cortex = mainly UMN

UMN weakness, brisk reflexes, extensory planter response, no LMN

What is the classic parkinsonism triad?

Bradykinesia, rigidity, resting tremor

Features of Parkinsons?

``` 6Ms Monotonous, hypotonic speech Micrographia HypoMimesis March a petit pas (gait with little steps = shuffling) Misery = depression Memory loss = dementia ``` Cogwheel rigidity

Causes of parkinsonism?

Parkinsons disease = lack of dopaminergic neurons in the substantia nigra) Drug induced e.g. antipsychotics/antiemetics Atypical parkinsonisms = Parkinsons plus e.g. vascular/multi-infarct parkinsons

Pathophysiology of Parkinsons?

alpha synuclein deposition and accumulation and deposition as Lewy Bodies and Lewy neurites

RFs for parkinsons?

Male Age FHx Double in countryside ?pesticides

What forms atypical parkinsonisms?

Mutliple system atrophy, Progressive supranuclear palsy, corticobasal degeneration (CBD), vascular parkinsons, Lewy body dementia

Features of Multiple system atrophy (MSA)

early autonomic and cerebellar fetures | Papp-lantos bodies

features if PSP?

Early postural instability and vertical gaze palsy

Features of CBD?

Alien limb phenomenom

Features of vascular parkinsons?

Legs worse affected and gait worse than tremor Stepwise deterioration ?hallucinations

Features of Lewy Body dementia?

Early dementia and visual hallucinations

Features of parkinsons disease Dementia?

Amnestic language deficits, Visuospatial dysnfunction (CLOCK), hallucinations, fluctuations, aggression and anxiety Similar to Lewy body but later onset with motor first

Other Gaits and causes?

``` Ataxic = wernickes Scissor = cerebral palsy Hemiplegic = stroke Choreiform = huntingdons ```

Most common types of dementia?

Alzheimers type Lewy body Dementia and PDD Vascular dementia Frontotemporal dementia Wernickes-korsakoff, haematomas, HIV, syphillis, hypoglycaemic and depression and Ddx

Features of Alzheimers?

Amnesia, anomia, apraxia, agnosia, aphasia +- depression Also dyscalclia, attention Atrophy of brain

pathophysiology of Alzheimers?

Amyloid precursor protein accumulates to form amyloid plaques Tau tangles in the neuron cause apoptosis nd decreased Ach in cortex. Thee are neurofibrilliary tangles

Rfs for Alzheimers?

Increasing age, vascular = DM, BP, dyslipidaemia genetics with APOE and Presenilin 1 and 2 Down syndrome

Ix for Alzheimers?

Clinical diagnosis CSF = Tau and beta amylodd Imaging with CT/MRI/PET and SPECT Brain biopsy is definitive MMSE and MOCA

`Features of vascular dementia?

Like alzheimers but location specific deficits, emotional and personality chances and focal neurology Increased AGe and vasculopaths SUDDEN ONSET AND STEPWISE DETERIORATION

RFs for vascular dementia?

M>F Elderly CVD Rfs MRI shows hemosiderin deposition from previous infarcts

Fetures of FTD/Picks?

``` personality change Disinhibition Overeating emotional blunting relative preservation of memory ``` Taupathy 40-60YO

WHat are Pick bodies?

Hyperphosphorylaed tau protein

What are the symptoms of Huntingtons disease?

Motor = chorea, athetosis (writhing hands), ataxia and dysphagia Cognitive = lack of concentration, depression, dementia and personality changes/aggression

Huntingdons pathology?

Atrophy in caudate and putamen (striatum) Mutation in huntingtin gene (HTT) so CAG expansion and Autosomnal dominant <28 no Huntingtons 29-34 = next gen 35-39 = some but not all ma develop >40 will get huntingtons

Features of Wernickes?

``` COAT Confusion Opthalmoplegia (eye = nystagmus, diplopia, ptosis) Ataxia Thiamine deficiency so Alcoholism ```

What can cause wernickes?

Alcohol mainly Malnutrition gastric bypass Dialysis

Ix for Wernickes?

Bloods = serum albumin and B1 (thiamine) ECG CT neuropsychology

Wernickes vs Korsakoffs?

Wernickes = acute, confusion, cerebellar and eye signs and reversible Korsakoffs = chronic, alert, retrograde/antergrade amnesia an confabulation ?irreversible