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Autoimmune - Neuro
Dr. Ziegler Neurological Disease - Autoimmune
| Term | Definition |
|---|---|
| de-myelinating diseases | optic neuritis, multiple sclerosis, neuromyelitis optica, progressive multifocal leukoencephalopathy |
| optic neuritis | inflammatory de-myelination of the ON; ~50% of patients develop MS; vision loss, typically monocular, eye pain during eye movements |
| etiology of optic neuritis | - inflammatory process triggers during or after viral resolution - pro-inflammatory chemical exposure - vitamin B12 deficiency |
| visual loss due to optic neuritis | central scotoma, reduced acuity around scotoma, impaired color detection - can be complete vision loss (typically monocular) |
| diagnostic testing for optic neuritis | - optic disc can be swollen, inflamed, pallor - afferent pupillary defect - primary visual cortex responding to alternating checkerboard stimulus - long latency of cortical response with normal amplitude (consistent with de-myelination) |
| temporal profile of optic neuritis | - onset: hours to days (max 1 wk) - duration: <2 weeks - recovery: 6 weeks to months |
| reasons to suspect a DD than optic neuritis | age of 45+, absence of eye pain, bilateral, visual loss lasting more than 2 weeks, no recovery stage |
| multiple sclerosis "MS" | range of findings: - cognitive defects, psychiatric symptoms - cranial nerve deficits (such as oculomotor) - cerebellum: poor coordination, tremor - motor exam: weakness, abnormal reflexes - sensory exam: Lhermitte's sign, sensory loss |
| diagnostic criteria for MS | MRI: 2+ white matter lesions "plaques" (can be supratentorial, infratentorial, spinal cord); finger extensions from periventricular zone Reduced conduction velocity (increased latency) on evoked potentional CSF: oligoclonal bands |
| oligoclonal bands in CSF | in MS: abnormal presence of bands at specific molecular weights; reflects large amount of IgG being produced; elevated lymphocyte count in CSF |
| bilateral MLF lesion due to MS | bilateral; pure CN3 functions remain intact but MR won't follow LR during lateral conjugate gaze |
| temporal profile of multiple sclerosis | 50% have a prior episode of optic neuritis; relapse-remitting or cyclic can become chronic and progressive |
| neuromyelitis optica "NMO" | - MS mimic disorder - de-myelination of optic nerves and spinal cord - bilateral vision loss, level down sensory & motor deficits - multifocal but acute & stable - autoimmune: aquaporin 4 antibodies |
| acute disseminated encephalomyelitis "ADEM" | inflammatory damage to white and gray matter, mimics MS or stroke (more often gray matter damage); typically after infection or vaccination |
| ADEM profile | - multi-focal/diffuse - acute onset - non-localizing signs (initial presentation may be focal mimicking stroke) except localization does not conform to a single vascular territory or lesion site |
| progressive multifocal leukoencephalopathy | - viral infection of oligodendrocytes (papovavirus of JC virus); multi foci of white matter de-myelination and damage in the CNS |
| progressive multifocal leukoencephalopathy profile | - subacute and progressive - can be fatal - can progress to dementia - typically no pain |
| leukodystrophy | - dys-myelinating disorders involve myelin structure and function defects - myelination of CNS begins in utero/early childhood - myelination is impaired or defective - symptoms present during 1st 2 years of life (can be delayed though) |
| autoimmune encephalitis | antibody-mediated diffuse inflammatory diseases of brain *subacute onset, CSF show pleocytosis (elevated WBC) |
| NMDA-R autoimmune encephalitis | antibodies against NMDA type glutamate; diffuse inflammation that mimicked psychotic psychiatric conditions; initially misdiagnosed, nearly fatal |
| triggering events for auto-antibodies | paraneoplastic syndrome, immune check point inhibitor Rx, post-infection (most common HSV) |
| autoimmune encephalitis generic aspects | - inflammation: increased intracranial pressure and meningeal irritation signs - paraneoplastic cases - seizures - diagnosis of exclusion: signs of increased intracranial pressure doesn't fit with psychiatric disorder |
| anti-NMDA glutamate receptor | most common type of autoimmune encephalitis, diffuse due to ubiquitous distribution of NMDA receptors, mimics psychotic disorders, major cases have ovarian/testicular teratoma |
| anti-GABA or anti-GAD | enzyme that synthesize GABA: psychiatric, seizures |
| anti-myelin oligodendrocyte glycoprotien | diffuse white matter, motor |
| anti-yo | purkinje neurons of cerebellum affects all cerebellar systems |
| myasthenia gravis "MG" | antibody autoimmune attack on nAchR; induces receptor internalization and impairs NMJ function; presents as fatigueable |
| tensilon test | use of edrophonium (AchEI) to test for MG |
| MG presentation | - diffuse weakness, weakness of EOMs, weakness of levator palpebral (ptosis) - progressive, relapse-remitting pattern |
| Lambert-eaton syndrome | antibody-mediated autoimmune attack against calcium channels coupled to Act release in skeletal muscle and ANS; limb muscles affected but EOMs spared (presynaptically) >> AChEI not effective |
| demyelinating | autoimmune attack against myelin sheath or Schwann cell; detected as slowing of nerve conduction velocity |
| axonopathy | neurodegenerative - detected as reduction in amplitude of nerve conduction due to loss of some axons, many still intact |
| neuronopathy | sensory: degeneration of neuronal cell bodies of sensory in DRG motor: motoneurons of spinal cord |
| guillaine-barre syndrome (acute inflammatory demyelinating polyneuropathy) | emerges after an infection resolves, weakness & sensory loss legs, arms, face, motor weakness, symmetrical and diffuse |
| oculomotor myopathies | weakness most often with eye and lid elevation, often diplopia occurs major etiologies: hyperthyroidism, mitochondrial DNA mutation |
| duchenne muscular dystrophy "DMD" | mutation in dystrophin, pediatric disorder with progressive diffuse weakness (wheel chair bound), common early signs: toe-walking, waddling gait, gower's sign |
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