neuroscience Flashcards
Neurofibromatosis type 1 (NF1)
- neurocutaneous syndrome characterized by cafe-au-lait macules and tumors of the skin and central and peripheral nervous systems
- an MRI of the brain and orbits is the best modality for a detailed evaluation of soft-tissue anatomy in any NF1 patient w/ concerning neurologic symptoms (eg, chronic headache, vision changes, early-morning vomiting)
Bilateral acoustic neuromas (vestibular schwannomas) can cause sensorineural hearing loss and are diagnostic of neurofibromatosis type 2. AUDIOMETRY is the best initial screening lab test for the diagnosis of acoustic neuromas, but its not indicated for NF1.
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main clinical features of Neurofibromatosis type 1
- cafe-au-lait spots
- multiple neurofibromas
- lisch nodules
main clinical features of neurofibromatosis type 2
bilateral acoustic neuromas
Tuberous sclerosis
- neurocutaneous genetic (TSC1 and TSC2 genes) syndrome associated w/ intracranial tumors (eg, cortical tubers or hamartomas, subependymal giant cell astrocytomas, subependymal nodules).
- other characteristics: hypopigmented macules (ash leaf spots), facial angiofibromas, cardiac rhabdomyomas, renal angioleiomyomas, mental retardation, and seizures
suspect multiple sclerosis in a patient with neurological deficits that cannot be explained by a single lesion. Exacerabation of these neurological deficits by hot weather or exercise are a useful clue.
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Internuclear ophthalmoplegia is a characteristic finding of what?
Multiple sclerosis
-due to demyelination of the medial longitudinal fasciculus in the dorsal pontine tegument
lesion of the optic nerve
blindness in the same eye
lesion of the optic chiasm
-bitemporal hemianopia
lesion of the optic radiation
contralateral hemianopia
lesion of the oculomotor nerve
-ptosis and the eye looking down and out, due to unopposed actions of the lateral rectus and superior oblique muscles
lesion of the trochlear nerve
vertical diplopia and extorsion of eye
lesion of the abducens nerve
convergent strabismus and horizontal diplopia
lesion of the medial lemniscus
affects touch and vibration sensations bilterally
destruction of the frontal eye field in frontal lobe
ipsilateral deviation of the eyes
Huntington’s disease
- suspect in a patient w/ mood disturbances, dementia, chorea and a family hx of similar symptoms
- autosomal dominant and affects both sexes equally
- age at presentation is typically between 30-50 years
- facial grimacing, ataxia, dystonia, tongue protrusion, writhing movements of extremities
pseudodementia
-severe depression in the elderly which presents with a dementia similar to alzheimer’s disorder
Pick’s disease
- similar in presentation to Alzheimer’s disease, except that it presents at an earlier age
- seen more frequently in females, and frequently causes personality changes due to involvement of the frontal lobes
Carpal tunnel syndrome
- most common mononeuropathy of the upper extremity; paresthesias of the first three-and-a-half digits and occasionally the thenar eminence atrophy are typical
- carpal tunnel is a fibro-osseous structure formed by the carpal bones and covered by the transverse carpal ligaments; median nerve passes through this tunnel along w/ the tendons of FLEXOR DIGITORUM SUPERFICIALIS, FLEXOR DIGITORUM PROFUNDUS, and FLEXOR POLLICIS LONGUS
- it develops in 7% of pts w/ hypothyroidism
location where entrapment of the median nerve can occur
- carpal tunnel is most common
- also in the forearm during pronator teres syndrome
- also at the elbow; deep flexors of the digits are involved, but cutaneous sensation is usually spared
alzheimer disease
- early, insidious short-term memory loss
- language deficits and spatial disorientation
- later personality changes
vascular dementia
- STEPWISE decline
- early executive dysfunction
- CEREBRAL INFARCTION and/or deep white matter changes on neuroimaging
Frontotemporal dementia
- EARLY PERSONALITY CHANGES
- apathy, disinhibition and compulsive behavior
- FRONTOTEMPORAL ATROPHY on neuroimaging
Lewy body dementia
- VISUAL HALLUCINATIONS
- spontaneous PARKINSONISM (rigidity, bradykinesia, gait disturbance)
- fluctuating cognition