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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.
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T W Janssen-van Kempen
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Rinsho shinkeigaku = Clinical neurology,
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A A Gabreëls-Festen, and
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T W Janssen-van Kempen
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Brain pathology (Zurich, Switzerland),
A A Gabreëls-Festen, and
E M Joosten, and
F J Gabreëls, and
F G Jennekens, and
T W Janssen-van Kempen
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European archives of psychiatry and clinical neuroscience,
A A Gabreëls-Festen, and
E M Joosten, and
F J Gabreëls, and
F G Jennekens, and
T W Janssen-van Kempen
January 1993,
Acta neuropathologica,
A A Gabreëls-Festen, and
E M Joosten, and
F J Gabreëls, and
F G Jennekens, and
T W Janssen-van Kempen
January 1996,
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A A Gabreëls-Festen, and
E M Joosten, and
F J Gabreëls, and
F G Jennekens, and
T W Janssen-van Kempen
November 2009,
Rinsho shinkeigaku = Clinical neurology,
A A Gabreëls-Festen, and
E M Joosten, and
F J Gabreëls, and
F G Jennekens, and
T W Janssen-van Kempen
December 1997,
The International journal of neuroscience,
