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函館稜北病院総合診療科抄読会 #hakodoku

毎週木曜日5:30PMから30分間、Clinical Problem-Solvingを素材にクリニカル・パールを拾い集めます。

第33回:"Diagnosing One Letter at a Time"

抄読会
 病名は、Hakase Taroに似たあの病気です。It's on the tip on the tongue. 別名でPOEMS症候群ともいい、症状の頭字語が症候群名になっていることが、タイトルの由来となっているのでしょう。

Word Count:2896語 (Time:  19'17'', 150 wpm)

参加人数:5名+書面参加1名

クリニカル・パール:

  • The rate of symptom development and progression is often the most valuable historical feature used to differentiate among possible causes. This patient's symptoms developed gradually, making most vascular and bacterial causes unlikely. Since the symptoms progressed over a 5-month period, neurodegenerative and inherited causes are also unlikely. Disease processes that typically develop and progress over a period of months include inflammatory, neoplastic, nutritional, toxic, and atypical infectious diseases.
  • Hyporeflexia in the upper arms with preserved arm strength indicates that the reflex is reduced because of involvement of the afferent (sensory) portion of the reflex arc. Therefore, even without the sensory examination, one can be confident that this disease process is affecting sensory-nerve fibers, localizing the disorder to the peripheral nerve.
  • Patients with the POEMS syndrome usually have uniform demyelination, as suggested by the findings in this patient, whereas patients with CIDP tend to have multifocal demyelination that is more severe in the proximal and distal nerve segments.
  • Sclerotic bone lesions or Castleman's disease (a rare lymphoproliferative disorder) are present in nearly all patients with the POEMS syndrome.
  • Edema and splenomegaly may occur in the POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) and raise suspicion for this diagnosis, especially if demyelination is confirmed on electrodiagnostic testing.
  • A terminal latency index of 0.38 or more, indicating comparatively less distal demyelination, has good sensitivity and specificity for distinguishing the POEMS syndrome from the more common CIDP.
  • The absence of conduction block, temporal dispersion, and sural-nerve sparing and the presence of denervation on needle examination, distal-predominant weakness, and unrecordable sural- and tibial-nerve responses all point to the POEMS syndrome over CIDP.
  • Monoclonal gammopathies of undetermined significance are not uncommon in persons older than 50 years of age and may be associated with a CIDP-like phenotype.
  • VEGF-induced neovascularity and microvascular permeability are postulated to cause most of the hallmark manifestations of this syndrome.
  • Interleukin-12 is also overproduced in the POEMS syndrome and may play a role in the pathogenesis of the disorder.

感想:

  • 神経学の議論は難しい。
  • polyneuropathyの患者さんを診たときに頭の片隅にでも浮かんでくるようにしておいたほうがいいかも。「手袋靴下履かせたろう」とか、「手袋のポエム」とかね。