Early morphological features in dominantly inherited demyelinating motor and sensory neuropathy (HMSN type I)

Seventeen cases of dominantly inherited demyelinating motor and sensory neuropathy (HMSN type I) with infantile onset were studied. Not only clinical and electrophysiological data, but also the g ratio (axon diameter to fibre diameter), considered to be a distinguishing feature between HMSN type I a...

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Veröffentlicht in:Journal of the neurological sciences 1992-02, Vol.107 (2), p.145-154
Hauptverfasser: Gabreëls-Festen, A.A.W.M., Joosten, E.M.G., Gabreëls, F.J.M., Jennekens, F.G.I., Kempen, T.W.Janssen-van
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Sprache:eng
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Zusammenfassung:Seventeen cases of dominantly inherited demyelinating motor and sensory neuropathy (HMSN type I) with infantile onset were studied. Not only clinical and electrophysiological data, but also the g ratio (axon diameter to fibre diameter), considered to be a distinguishing feature between HMSN type I and HMSN type III, showed overlap. Morphological and morphometrical investigations already revealed a lack of small and large diameter myelinated axons at an early stage, and a demyelinating process most active in early childhood followed later by axonal loss. It was concluded that the histopathology of HMSN type I cannot be sufficiently explained by axonal atrophy with secondary demyelination.
ISSN:0022-510X
1878-5883
DOI:10.1016/0022-510X(92)90282-P