Congenital hypomyelination neuropathy: Glial bundles in cranial and spinal nerve roots

Towfighi, Javad

doi:10.1002/ana.410100614

Cited by 18 publications

(2 citation statements)

References 17 publications

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“…The new form was named "Congenital Hypomyelination Neuropathy" (CHN) and it has been considered to be a separate nosologie entity. So far 29 cases have been reported and congenital impairment in myelin formation has been postulated, in contrast with the classical forms of HMSN which are supposed to be degenerative in origin (1,3,6,8,9,10,11,12,13,14,15,16,17,18,20,21,22,23,24). Different clinical, neurophysiological and morphological features have been indicated as specific for CHN.…”

Section: Resumementioning

confidence: 99%

Infantile Hereditary Neuropathy with Hypomyelination: Report of Two Siblings with Different Expressivity

Mr¹,

Cavaletti²,

D’Angelo³

et al. 1991

Neuropediatrics

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We report the cases of two siblings both affected by inherited sensory-motor neuropathy of a demyelinative nature but with markedly different severity and pathological findings. The clinical, neurophysiological and morphological features in these two cases were consistent with the diagnosis of Hereditary Motor Sensory Neuropathy type 3 (HMSN 3), according to the classification of Dyck, with different expressivity. These results raise the still unsettled question of the phenotypic variants in inherited neuropathies. In fact the most severely affected of our cases had clinical and neurophysiological findings identical to those reported in cases of Congenital Hypomyelination Neuropathy (CHN), but the morphological picture in the sural nerve was inconsistent with this diagnosis. The criteria for the diagnosis and the reported cases of CHN have been reviewed.

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Section: Resumementioning

confidence: 99%

Infantile Hereditary Neuropathy with Hypomyelination: Report of Two Siblings with Different Expressivity

Mr¹,

Cavaletti²,

D’Angelo³

et al. 1991

Neuropediatrics

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“…An alternative explanation is that this is an astrocytic reaction secondary to the degeneration of lower motor neurons (Ghatak, 1978). More recently, the occurrence of glial bundles has been demonstrated in a variety of other pathologic conditions such as healed poliomyelitis (Iwata & Hirano, 1979), Charcot-Marie-Tooth disease (Smith et al, 1980), demyelinating radiculopathy in Kearns-Sayre syndrome (Groothuis et al, 1980), congenital hypomyelination (Towfighi, 1981), and amyotrophic lateral sclerosis (Ghatak & Nochlin, 1982). Astrocytic outgrowths resembling glial bundles have also been observed under certain experimental conditions such as fi, p-iminodipropionitrile (IDPN) toxicity in rats (Griffin & Price, 1980) and transection and anastomosis of spinal nerve roots in pigs (Meier & Sollmann, 1978).…”

Section: Introductionmentioning

confidence: 99%

Glial Bundles in Spinal Nerve Roots: A Form of Isomorphic Gliosis at the Junction of the Central and Peripheral Nervous System

Ghatak

1983

Neuropathology Appl Neurobio

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A morphologic study of the spinal nerve roots was undertaken in three cases of Werdnig-Hoffmann disease to investigate the phenomenon of glial bundle formation. The glial elements extended along the ventral roots as discrete cylindrical bundles comprising a large number of parallel astrocytic processes and sparsely scattered cell bodies all enclosed by a basal lamina. The bundles tapered off at a variable distance from the root exit zones. The early stage of glial bundle formation was characterized by the protrusion of astrocytes into the neurilemmal tubes containing degenerated myelinated axons. It was concluded that axonal degeneration, evoking a glial reaction, was the initial event in this process. Subsequently, the reactive astrocytes from the vicinity of the root exit zones enter the neurilemmal tubes previously occupied by myelinated axons and migrated into the domain of the peripheral nervous system in an orderly fashion. Thus glial bundle formation might be considered a special form of isomorphic gliosis occurring in Werdnig-Hoffmann disease and also in several other conditions all sharing a common feature, namely, degeneration of axons within the spinal nerve roots.

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Glial bundle formation in spinal roots following experimental neuronopathy

Yamamoto

Iwasaki

Konno

et al. 1986

Annals of Neurology

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Spinal motor neurons and dorsal root ganglion cells were caused to degenerate by axoplasmic transport of toxic lectins via the sciatic nerve. Within a few months, loss of axons and glial bundle formation were seen in the proximal portions of the L4-6 ventral and dorsal roots. The glial bundles observed were structurally the same as those described in humans, but they were occasionally also invested with Schwann cell cytoplasmic processes, the cell membrane of which was directly apposed to that of astrocytes. Basal lamina surrounding the outer surface of the bundles was shared by these two as a continuous sheet. This unique complex of astroglial and Schwann cell processes, completely covered by basal lamina, may be a newly formed interface between the central and peripheral nervous systems and thus may severe as a guide and potential pathway for regenerating neurites.

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Congenital hypomyelination neuropathy: Glial bundles in cranial and spinal nerve roots

Cited by 18 publications

References 17 publications

Infantile Hereditary Neuropathy with Hypomyelination: Report of Two Siblings with Different Expressivity

Infantile Hereditary Neuropathy with Hypomyelination: Report of Two Siblings with Different Expressivity

Glial Bundles in Spinal Nerve Roots: A Form of Isomorphic Gliosis at the Junction of the Central and Peripheral Nervous System

Glial bundle formation in spinal roots following experimental neuronopathy

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