Week 2 - Neuro Flashcards
(40 cards)
Types of seizure
Partial: simple and complex
Generalized: absence, tonic-clonic, tonic, clonic, atonic, myotonic
Partial seizures
Simple: consciousness not impaired, sx vary, brief, NO post-ictal sx
Complex: consciousness is impaired, either temporal or psychomotor, staring and automatisms, pt does not recall, 30-60sec, post-ictal impairment of lethargy and confusion
Temporal origin: preceded by aura, often have autonomic sx
Frontal origin: arrest of activity, motor manifestations, blank stare
Generalized tonic-clonic seizures
Tonic: loss of consciousness with stiffening of limbs
Clonic: generalized jerking of muscles
Post-ictal: deep sleep
Absence seizures
generalized
abrupt sessation of activity, blank stare
less than 30sec, no aura
can have clonic eye movement, or peseveration
Clonic and tonic seizures
Clonic= focal or multifocal Tonic= generalized, brief, increased extensor tone
Atonic and Myoclonic seizures
Atonic: "drop attack" sudden loss of tone brief loss of consciousness rare Lennox-Gastaut syndrome Myoclonic: sudden, brief, shock-like, sign of diffuse brain injury
Lysosomal Storage Diseases (LSD)
usually do not affect CNS, some do. autosomal recessive usually Lysosomes= acid hydrolases Gangliosides= neuronal lysosomes -Tay-Sachs= Hexoaminidase A -Danshoff= Hexoaminidase B
Tay-Sachs Disease
lysosomal storage disease (Hexoaminidase A)
Ashkenazi Jews
normal at birth, psychomotor retardation at 6mo, blindness, flaccidity, decerebrate, cherry red spot
death by 2-3yr
enlarged balloon neurons filled with PAS
Krabbe’s Disease (Globoid cell Leukodystrophy)
Lysosomal storage disease
autosomal recessive
def of galactocerebroside-B-galactosidase
accumulation of toxic phychosine, injures oligodendrocytes
accumulates in myelin- Globoid cells
both CNS and PNS
onset 3-6mo, deterioration of motor, tonic spasms, blindness, CSF protein elevated
atrophic white matter, preserved U-fibers
death by 2yo
Tx: marrow transplant early
Metachromatic Leukodystrophy
Lysosomal storage disease, auto recessive
def of aryl sulfatase A
metachromatic lipids accumulate in brain, leads to breakdown of myelin
loss of myelin, preservation of U-fibers, brown staining of white matter
can have late infantile, juvenile, or adult onset
gait and motor sx, adults have psychosis and cognitive impairment
Tx: bone marrow transplant early
Adrenoleukodystrophy
Peroxisomal disorder
decreased activity of very long chain fatty acyl-CoA synthetase
X-linkedclassic= onset less than 20yo, dementia, seizures, adrenal insuff follows
Adrenomyeloneuropathy= adult, progressive leg stiffness, spastic paraplegia, adrenal insuff precedes
gray discolorization of white matter, demyelination, perivascular inflammation
Hepatic encephalopathy
complication of liver disease, hyperammonemia
sx: inattentiveness, confusion, asterixis, coma, foul breath
can be rapidly fatal
increased T1 signal in globus pallidus, Alzheimer type II astrocytes
Tx: liver transplant
Mitochondrial syndromes
usually maternally inherited, can involve many organs
MELAS, MERRF, KSS
Leigh’s disease
Leigh’s disease (Subacute necrotizing Encephalopathy)
mitochondrial syndrome (mutation in nuclear DNA)
autosomal recessive
sx: lactic acidemia, hypotonia, seizures, extraocular palsies
death by 2yo
looks like multifocal infarcts, periventricular gray matter destroyed, spongiform appearance and vascular prolif
Thiamine (B1) deficiency
chronic alcoholics, malnourishment
Wernicke encephalopathy= nystagmus, ataxia, confusion,, lesions in mammillary bodies, dorsomedial thalamus, acute gray-brown discolorization, chronic atrophy,, pallow and myelin loss
Korsakoff syndrome= loss of anterograde episodic memory, confabulation, result of repeated Wernicke’s,, damage to medial dorsal thalamus
Vitamin B12 (cobalamin) deficiency (Subacute combined degeneration of spinal cord)
usually from pernicious anemia
ataxia, romberg, spastic, decreased reflexes
anterior and lateral corticospinal tracts and posterior columns are demyelinated
Carbon monoxide poisoning
binds to globus pallidus and SN, causes necrosis
motor, cognitive, psychiatri, and parkinsonian
Chronic alcoholism
truncal ataxia, nystagmus
cerebellar degeneration of anterior superior vermis
Fetal alcohol syndrome
growth retardation, facial deformities (small eye openings, smooth philtrum, thin upper lip)
also cardiac probs and delayed devo
microcephaly, cerebellar dysgenesis
Drug toxicities
Methotrexate= coagulative necrosis and mineralization, leukoencephalopathy Vincristine= axonal swelling Phenytoin= atrophy of cerebellar vermis Cocaine= infarcts and hemorrhages Amphetamine= infarcts and hemorrhages
Neurogenic vs Myopathic muscle problem
Neurogenic= bimodal size of fibers, angulated fibers, no necrosis Myopathic= random size, round fibers, central nuclei, necrosis
ALS (Amyotrophic Lateral Sclerosis)
UMN and LMN degeneration, normal sensation
onset 50-60yo
weakness, fasciculations, hyperreflex, pos babinski, spasticity, pseudobulbar palsy
anterior horn cell dreath and lateral corticospinal tracts
Spinal Muscular Atrophy (SMA)
LMN degeneration of childhood
autosomal recessive, deletion of SMN1 gene
prosimal muscle weakness, dysphagia, respiratory weakness
normal intellect
areflexic
Muscular dystrophies
X-linked= Duchenne and Becker muscular dystrophy Autosomal= Myotonic MD
