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HMSN 1A (CMT 1A) and Sjogren's syndrome

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Abstract from Rev Neurol (Paris). 2004 Jul;160(6-7):717-20.

Demyelinating neuropathy and Sjogren's syndrome: a diagnostic pitfall

Guennoc AM, Corcia P, Maisonobe T, Lefrancq T, De Toffol B, Autret A.

Service de Neurologie et de Neurophysiologie Clinique, CHU Bretonneau,

Tours.

Introduction. Neuropathies induced by Sjogren's syndrome (SS) are

usually axonal. Nevertheless some demyelinating neuropathies have been

described in patients with SS. To date, the relationship between

demyelinating neuropathies and SS remains imprecise. Case report. A 75

Year-old man presented with a chronic history of sensory disturbances

linked to demyelinating neuropathy. Electroneuromyography revealed a

demyelinating neuropathy and complementary tests revealed both Sjogren's

syndrome (SS) and HMSN IA.

Conclusion. We suggested that an inherited affection might be researched

before considering that demyelinating neuropathy might be a form of

peripheral nervous system involvement in SS.

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