Neuro-02-Path Flashcards
1
Q
Dementia
A
- A decrease in cognitive ability, memory, or function with intact consciousness
- Causes:
- Alzheimer’s
- Pick’s disease
- Lewy body dementia
- Creutzfeldt-Jakob disease
- Multi-infarct: second most common cause of dementia in elderly
- Syphilis
- HIV
- Vitamins B1, B3, or B12 deficiency
- Wilson’s disease
- Normal pressure hydrocephalus (NPH)
2
Q
Alzheimer’s disease
A
- Most common cause of dementia in the elderly
- Down syndrome patient’s have an increased risk of developing alzheimer’s
- Familial form (10%) is associated with the following altered proteins:
- Early onset: APP (chromosome 21), presenilin-1 (chromosome 14), presenelin-2 (chromosome 1)
- Late onset: ApoE4 (Chromosome 19)
- ApoE2 (chromosome 19) is protective
- Findings:
- Widespread cortical atrophy
- Decreased ACh levels
- A-beta (amyloid-beta) synthesized by cleaving amyloid precursor protein (APP)
- Senile plaques: extracellular beta-amyloid core with entangled neuritic processes
- Senile plaques may cause amyloid angiopathy, leading to intracranial hemorrhage
- Neurofibrillary tangles: intracellular abnormally phosphorylated tau protein, forming insoluble cytoskeletal elements
- Tangles correlate with degree of dementia
3
Q
Pick’s disease (frontotemporal dementia)
A
- Presentation: Dementia, aphasia, parkinsonia aspects, change in personality
- Findings:
- Pick bodies: spherical tau protein aggregates
- Frontotemporal atrophy
- Spares parietal lobe and posterior 2/3 of superior temporal gyrus
4
Q
Lewy body dementia
A
- Parkinsonism with dementia and hallucinations
- Findings:
- alpha-synuclein defect
- Lewy bodies are in cortex (instead of in basal ganglia, as in Parkinson’s)
5
Q
Creutzfeldt-Jakob disease (CJD)
A
- Rapidly progressive (weeks to months) dementia with myoclonus (startle myoclonus)
- Findings:
- Spongiform cortex
- Prions: PrPc -> PrPsc sheet (beta-pleated sheets resistant to proteases)
6
Q
Multiple sclerosis
A
- Autoimmune inflammation and demyelination of CNS (brain and psinal cor)
- Can present with: Optic neuritis (sudden loss of vision), internuclear opthalmoplegia (MLF syndrome), hemiparesis, hemisensory symptoms, bladder/bower incontinense
- {Charcot’s classic triad of MS is a sin : Scanning speech, Intention tremor (also incontinence and INO), Nystagmus}
- Relapsing and remitting course
- Most often affects women in their 20s and 30s; more common in whites
- Findings:
- Increased protein (IgG) in CSF: oligoclonal bands are diagnostic
- MRI is gold standard
- Periventricular plaques with destruction of axons: areas of oligodendrocyte loss and reactive gliosis
- Treatment:
- beta-interferon
- immunosuppression
- natalizumab
- symptomatic treatment for bladder (catheterization, muscarinic antagonists), spasticity (baclofen, GABA agonist), and pain (opioids)
7
Q
Acute inflammatory demyelinating polyradiculopathy
A
- Most common variant of Guillain-Barre syndrome
- Autoimmune condition that destorys schwann cells, leading to inflammation and demyelination of peirpheral nerves and motor fibers
- Results in symmetric ascending muscle weakness/paralysis beginning in lower extremitis
- Facial paralysis in 50% of cases
- Autonomic function may be severely affected (e.g., cardia irregularities, hypertenstion, or hypotnesion)
- Amost all patients survive, and the majority recover completely after weeks to months
- Associations (no definitive link to pathogens):
- infections (Campylobacter jejuni and CMV), which lead to autoimmune attack of peripheral myelin due to molecular mimicry
- Inoculations
- Stress
- Findings:
- Albuminocytologic dissociation (increased CSF protein with normal cell count)
- Increased protein leads to papilledema
- Treatment:
- Respiratory support is criticall
- Plasmapheresis
- IV immune globulins
8
Q
Progressive multifocal leukocencephalopathy (PML)
A
- Demyelination of CNS due to destruction of oligodendrocytes
- Associated with JC virus
- Seen in 2-4% of AIDS patients (reactivation of latent viral infection)
- Rapidly progressive, usually fatal
9
Q
Acute disseminated (postinnfectious) encephalomyelitis
A
Multifocal perivenular inflammation and demyelination after infection (commonly measles or VZV) or certain vaccinations (e.g., rabies, smallpox)
10
Q
Metachromatic leukodystrophy
A
- Autosomal recessive lysosomal storage disease
- Most commonly due to arylsulfatase A deficiency
- Leads to buildup of sulfatides with impaired production of myelin sheath
11
Q
Charcot-Marie-Tooth disease
A
- AKA, hereditary motor and sensory neuropathy (HMSN)
- Group of progressive hereditary nerve disorders related to defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath
12
Q
Krabbe’s disease
A
- Autosomal recessive lysosomal storage disease
- Due to deficiency of galactocerebrosidase
- Leads to buildup of galactocerebroside, which destroys myelin sheath
13
Q
Seizures
A
- Synchronized, high-frequency neuronal firing
- Causes of seizures by age:
- Children: genetic, infection (febrile), trauma, congenital, metabolic
- Adults: tumors, trauma, stroke, infection
- Elderly: stroke, tumor, trauma, metabolic, infection
- Partial (focal) seizures: simple partial, complex partial
- Generalized seizures: absence, myoclonic, tonic-clonic, tonic, atonic
14
Q
Epilepsy
A
A disorder of recurrent seizures (febrile seiures are not epilepsy)
15
Q
Status epilepticus
A
- Continuous seizure for > 30 min or recurrent seizurse without regaining consciousness between seizures for > 30 min
- Medical emergency
16
Q
Partial (focal) seizures
A
- Affect 1 are of the brain
- Most commonly originate in medial temporal lobe
- Often preceded by seizure aura
- Can secondarily generalize
- Types:
- Simple partial: consciousness intact; motor, snesory, autonomic, psychic symptoms
- Complex partial: Impaired consciousness; display automatisms
