385.2 🩺 國考版

385.2.1 高頻考點

385.2.1.1 Prion Disease Categories

  • Sporadic CJD (85%)
  • Familial CJD, GSS, FFI (15%)
  • Acquired: vCJD (BSE), iatrogenic, kuru

385.2.1.2 sCJD Features

  • Mean onset 65
  • Rapid progression (4-7 months)
  • Dementia + myoclonus + ataxia + visual + behavioral
  • Periodic sharp wave complexes (EEG late)

385.2.1.3 CJD Imaging

  • DWI bright cortex (cortical ribboning)
  • Basal ganglia DWI bright

385.2.1.4 CJD CSF

  • RT-QuIC (most accurate, > 95% sens/spec)
  • 14-3-3 (less specific)
  • Total tau ↑

385.2.1.5 vCJD

  • BSE-related
  • Younger
  • Psychiatric early
  • Pulvinar sign (bilateral thalamic on MRI)

385.2.1.6 CJD No Treatment

385.2.1.7 HAND

  • HIV-associated neurocognitive disorder
  • Subcortical pattern
  • On cART can still occur
  • Treatment: cART

385.2.1.8 Neurosyphilis

  • Tabes dorsalis, general paresis, meningovascular
  • VDRL CSF + treponemal serum
  • IV penicillin G 18-24 million units/d × 10-14 days

385.2.1.9 Whipple CNS

  • Tropheryma whipplei
  • Oculomasticatory myorhythmia (pathognomonic)
  • Ceftriaxone → TMP-SMX or doxycycline

385.2.1.10 Autoimmune Encephalitis Antibodies

  • Anti-NMDA-R (psychiatric + seizure + movement; ovarian teratoma)
  • Anti-LGI1 (FBDS + amnesia + hyponatremia)
  • Anti-CASPR2
  • Anti-GAD65
  • Onconeural (Hu, Ma2, CV2 — paraneoplastic)

385.2.1.11 Treatable Reversible Dementia

  • B12 deficiency
  • Hypothyroidism
  • Hashimoto encephalopathy (steroid)
  • Neurosyphilis
  • HIV
  • NPH
  • Autoimmune
  • Toxic
  • Medication

385.2.1.12 Hashimoto Encephalopathy

  • Steroid-responsive
  • Anti-TPO high
  • Subacute
  • Often mistaken for CJD

385.2.1.13 Wilson Disease

  • Young < 40 with movement + cognitive + KF rings
  • Low ceruloplasmin, high urine copper
  • Penicillamine, trientine, zinc

385.2.2 Rapidly Progressive Dementia DDx

Mnemonic VITAMINS: - Vascular (multi-infarct, CADASIL, CAA) - Infectious (HIV, syphilis, Whipple, viral encephalitis, fungal) - Toxic (heavy metals, drugs, withdrawal) - Autoimmune (encephalitis, vasculitis, Hashimoto) - Metabolic (B12, thyroid, hepatic, uremic, electrolytes) - Iatrogenic / drug-induced - Neoplastic (paraneoplastic, primary CNS lymphoma, gliomatosis) - Seizure / structural / sporadic CJD

385.2.3 Specific Topics

385.2.3.1 Iatrogenic CJD Sources (Historical)

  • Dural grafts
  • Growth hormone (pituitary-derived)
  • Neurosurgery instruments
  • Corneal transplants
  • Standard sterilization doesn’t inactivate prions

385.2.3.2 Aβ Transmission Historical

  • Aβ deposition reported in patients with iatrogenic CJD from dura/growth hormone
  • Controversial transmission of AD-like pathology
  • Modern sterilization avoids

385.2.3.3 Kuru

  • Papua New Guinea
  • Ritualistic cannibalism
  • Eradicated