MS, neurotransmitter, hypothalamus, amygdala Flashcards
acute inflammatory demyelinating polyradiculopathy - result in (symptoms)
- symmetric/paralysis (beginning in lower extremities)
- facial paralysis (in 50%)
- autonomic regulation (eg. cardiac irregulation, hypertension, hypertension)
- sensory abnormalities
acute inflammatory demyelinating polyradiculopathy - lab
increased CSF protein with normal cell count (albuminocytologic dissociation)
acute inflammatory demyelinating polyradiculopathy - increased protein in CSF can cause
papilledema
acute inflammatory demyelinating polyradiculopathy - treatment
- Respiratory support (critical until recovery)
- plasmapheresis
- IV immunoglobins
NO ROLE FOR STEROIDS
Multiple sclerosis epidemiology - MC in
women, 20-30 years old, whites living further from equator
Multiple sclerosis - patients can present with
- optic neuritis (sudden onset of vision resulting in Marcus Gunn pupils)
- intrenuclear ophthalmoplegia
- hemiparesis
- hemisensory symptoms
- bladder/bowel incontinence
- intention tremor
Multiple sclerosis - Charcot classic triad
MNEMONIC –> SIN
- Scanning speech (dysarthria)
- Intention tremor (also Internuclear opthalmoplegia and Incontinence)
- Nystagmus
Multiple sclerosis - gold standard
MRI
Multiple sclerosis - findings in CSF
increased IgG + myelin basic protein
Multiple sclerosis - … are diagnostic
oligoclonal bands
Multiple sclerosis - treatment
- slow progression treatment –> disease modifying therapies (eg. β-interferon, natalizumab)
- treat acute flares –> IV steroids
- symptomatic treatment –> cathetirazation, muscarinic antagonists, baclofen, GABA b receptor agonists, opioids
Adrenoleukodystrophy - pathophysiology / mode of inheritance
X-linked genetic disorder –> disrupts metabolism of very-long-chain fatty acids –> excessive buildup (accumulation) in nervous system, adrenal glands and testes
Adrenoleukodystrophy - coarse of disease
Progressive disease that can lead to
1. long-term coma/death and 2. adrenal crisis
demyelinating and dysmelinating diseases - types
- Multiple sclerosis
- Acute inflammatory demyelinating polyradiculopathy
- Acute disseminated (postinfectious) encephalomyelitis
- Charcot-Marie-Tooth disease
- Krabbe disease
- Metachromatic leukodystrophy
- Progressive multifocal leukoencephalopathy
Acute disseminated (postinfectious) encephalomyelitis? / course
multifocal periventricular inflammation and demyelination after infection or certain vaccination
- rapidly progressive multifocal neurologic symptoms
Acute disseminated (postinfectious) encephalomyelitis is usually caused by (examples)
- infection –> measles, VZV
2. certain vaccinations –> rabies, smallpox
Charcot-Marie-Tooth disease - AKA … / mode of inheritance
hereditary motor and sensory neuropathy (HMSN)
AD
Charcot-Marie-Tooth disease - definition and mechanism
Group of progressive hereditary nerve disorder related to the defective production of proteins involved in the structure and function of peripheral nerves or the meylin sheath
Charcot-Marie-Tooth disease - symptoms and signs
- foot deformities (pes cavus)
- lower extremities weakness
3 .sensory deficits
Krabbe disease - pathophysiology
deficiency of galactocerebrosidase –> buildup (accumulation) of galactocerebroside and psychosine destroys myelin sheath
Krabbe disease - findings (symptoms, signs, histology etc)
- peripheral neuropathy
- Developmental delay
- Optic atrophy
- Globoid cells
Metachromatic leukodystrophy - pathophysiology
deficiency of arysulfatase A –> buildup (accumulation) of sulfatides –> impaired production and destruction of myelin sheath
Metachromatic leukodystrophy - findings (symptoms, signs, histology etc)
- central and peripheral demyelination with ataxia
2. dementia
Progressive multifocal leukoencephalopathy - mechanism/definition / course
demyelination of CNS due to destruction of oligodendrocytes
- rapidly progressive, usually fatal
