Chronic Neuro Flashcards

1
Q

What is MS?

A

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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2
Q

Symptoms of MS?

A

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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3
Q

Examinations for MS?

A

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

Lhermittes sign

Look for cerebellar dysfunction

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4
Q

Where is MS common?

A

Nordic areas and high latititudes

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5
Q

Risk Factors for MS?

A
HLA BLRB1*15
Women 
Nordic
Lack of vitain D
Other Autoimmune conditions
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6
Q

What will MS show on MRI?

A

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

Gadolinium contrast allows differentiation in time

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7
Q

How is MS diagnosed?

A

Absense of alternative diagnosis
Dissemination in time
Dissemination in Space

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

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8
Q

What will MS show in CSF? Also electrophysiology?

A

Oligoclonal bands +ve only in the CSF.

Visual evoked potentials (VEPs)

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9
Q

What is Myasthenia Gravis symptoms?

A

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

Signs = fatigable muscles but normal reflexes

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10
Q

Associations and Ix for MG?

A

Thymic hyperplasia = 70% and thymoma 10%

Bloods = anti-AChR or MuSK
EMG
CT/MRI

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11
Q

What is Lambert Eaton Myasthenic Syndrome?

A

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

Weakness improves with repeased use of muscle

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12
Q

Other names for MND?

A

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

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13
Q

What is MND?

A

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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14
Q

Symptoms of MND?

A

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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15
Q

What are UMN signs?

A

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

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16
Q

What are LMN signs?

A

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

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17
Q

Key signs in MND?

A

Wasting of the thenar hand msucles and wasting of tongue muscles

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18
Q

Ix for MND?

A

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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19
Q

Components of MND?

A

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

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20
Q

Features of progressive muscular atrophy?

A

LMN only

e.g. flair arm/flail foot syndrome

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21
Q

Features of progressive bulbar palsy?

A

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

22
Q

Features of pseudobulbar palsy?

A

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

23
Q

Features of primary lateral sclerosis?

A

Loss of betz cells in otor cortex = mainly UMN

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

24
Q

What is the classic parkinsonism triad?

A

Bradykinesia, rigidity, resting tremor

25
Q

Features of Parkinsons?

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

Cogwheel rigidity

26
Q

Causes of parkinsonism?

A

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

27
Q

Pathophysiology of Parkinsons?

A

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

28
Q

RFs for parkinsons?

A

Male
Age
FHx
Double in countryside ?pesticides

29
Q

What forms atypical parkinsonisms?

A

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

30
Q

Features of Multiple system atrophy (MSA)

A

early autonomic and cerebellar fetures

Papp-lantos bodies

31
Q

features if PSP?

A

Early postural instability and vertical gaze palsy

32
Q

Features of CBD?

A

Alien limb phenomenom

33
Q

Features of vascular parkinsons?

A

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

34
Q

Features of Lewy Body dementia?

A

Early dementia and visual hallucinations

35
Q

Features of parkinsons disease Dementia?

A

Amnestic language deficits, Visuospatial dysnfunction (CLOCK), hallucinations, fluctuations, aggression and anxiety

Similar to Lewy body but later onset with motor first

36
Q

Other Gaits and causes?

A
Ataxic = wernickes
Scissor = cerebral palsy
Hemiplegic = stroke
Choreiform = huntingdons
37
Q

Most common types of dementia?

A

Alzheimers type
Lewy body Dementia and PDD
Vascular dementia
Frontotemporal dementia

Wernickes-korsakoff, haematomas, HIV, syphillis, hypoglycaemic and depression and Ddx

38
Q

Features of Alzheimers?

A

Amnesia, anomia, apraxia, agnosia, aphasia
+- depression

Also dyscalclia, attention
Atrophy of brain

39
Q

pathophysiology of Alzheimers?

A

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

40
Q

Rfs for Alzheimers?

A

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

41
Q

Ix for Alzheimers?

A

Clinical diagnosis
CSF = Tau and beta amylodd
Imaging with CT/MRI/PET and SPECT

Brain biopsy is definitive

MMSE and MOCA

42
Q

`Features of vascular dementia?

A

Like alzheimers but location specific deficits, emotional and personality chances and focal neurology

Increased AGe and vasculopaths

SUDDEN ONSET AND STEPWISE DETERIORATION

43
Q

RFs for vascular dementia?

A

M>F
Elderly
CVD Rfs

MRI shows hemosiderin deposition from previous infarcts

44
Q

Fetures of FTD/Picks?

A
personality change 
Disinhibition
Overeating
emotional blunting
relative preservation of memory

Taupathy
40-60YO

45
Q

WHat are Pick bodies?

A

Hyperphosphorylaed tau protein

46
Q

What are the symptoms of Huntingtons disease?

A

Motor = chorea, athetosis (writhing hands), ataxia and dysphagia

Cognitive = lack of concentration, depression, dementia and personality changes/aggression

47
Q

Huntingdons pathology?

A

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

48
Q

Features of Wernickes?

A
COAT
Confusion
Opthalmoplegia (eye = nystagmus, diplopia, ptosis)
Ataxia
Thiamine deficiency so Alcoholism
49
Q

What can cause wernickes?

A

Alcohol mainly
Malnutrition
gastric bypass
Dialysis

50
Q

Ix for Wernickes?

A

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

51
Q

Wernickes vs Korsakoffs?

A

Wernickes = acute, confusion, cerebellar and eye signs and reversible

Korsakoffs = chronic, alert, retrograde/antergrade amnesia an confabulation
?irreversible