A Charcot-Marie-Tooth disease resembling to chronic inflammatory demyelinating polyradiculoneuropathy:a report of two cases
- VernacularTitle:慢性炎性脱髓鞘性周围神经病样表现的腓骨肌萎缩症二例
- Author:
Yi WANG
;
Kai QIAO
;
Chuan-Zhen LV
;
- Publication Type:Journal Article
- Keywords:
Charcot-Marie-Tooth disease;
Polyradiculoneuropathy,chronic inflammatory demyelinating;
Electrophysiology
- From:
Chinese Journal of Neurology
2005;0(07):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To observe the clinical, neuropathogical, neurophysiological characteristics of Charcot-Marie-Tooth disease, type 1A (CMT1A) which was similar to chronic inflammatory demyelinating polyradiculoneuropathy(CIDP).Methods The clinical data, neuroelectrophysiological changes and pathological features of sural nerve biopsy were taken from 2 CMT1A patients who were proven to be 17p12 duplication with CIDP features, and analyzed comprehensively. Results Two CMT1A cases with classical chronic course showed both subacute course and clinical manifestations similar to CIDP, however, the changes of neuroelectrophysiology and pathological characteristics of the nerve biopsy in 2 cases were different from CIDP in some way. We confirmed the chronic inflammatory demyelinating type of the CMT1A patients whom the immunotherapy was effective on.Conclusion The classical CMT1A 17p12 duplication patients who obtained immunotherapy effects might resemble CIDP both in clinical course and manifestation.
