Chronic Neuro Flashcards
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
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
Examinations for MS?
Fundoscopy = retro-bulbar neuritis in 2/3 and optic neuritis in 1/3
Lhermittes sign
Look for cerebellar dysfunction
Where is MS common?
Nordic areas and high latititudes
Risk Factors for MS?
HLA BLRB1*15 Women Nordic Lack of vitain D Other Autoimmune conditions
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
How is MS diagnosed?
Absense of alternative diagnosis
Dissemination in time
Dissemination in Space
Clinic history/exam, MRI, CSF and eletrophysiology = VEPS
What will MS show in CSF? Also electrophysiology?
Oligoclonal bands +ve only in the CSF.
Visual evoked potentials (VEPs)
What is Myasthenia Gravis symptoms?
Muscles fatigue with use
= ptosis, diplopia, dysarthria, dysphagia +- SOB
Signs = fatigable muscles but normal reflexes
Associations and Ix for MG?
Thymic hyperplasia = 70% and thymoma 10%
Bloods = anti-AChR or MuSK
EMG
CT/MRI
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
Other names for MND?
ALS (amyotrophic lateral sclerosis), Lou Gehrigs and Charcots disease
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
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
What are UMN signs?
Increased weakness, no atrophy, increased reflexes, no fasciculatations, increased tones and extensor response present
What are LMN signs?
Weakness, atrophy, decreased reflexes, fasciculations, decreased tone and absent etensor response
Key signs in MND?
Wasting of the thenar hand msucles and wasting of tongue muscles
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?
Components of MND?
Progressive muscular atrophy, progressive bulbar palsy, pseudobulbar palsy and primary lateral scelrosis
Features of progressive muscular atrophy?
LMN only
e.g. flair arm/flail foot syndrome
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
