Severe Sensory Changes, and Trophic Disorder, in Peroneal Muscular Atrophy (Charcot-Marie-Tooth Type)

Motor nerve conduction velocity in peroneal muscular atrophy: evidence for genetic heterogeneity

“…Sensation may be diminished in a distal distribution, but is usually not severely impaired. Some authors have accepted cases with severe distal sensory loss and perforating ulcers as instances of peroneal muscular atrophy (Halliday and Whiting, 1909;England and Denny-Brown, 1952). A mild associated ataxia has also been included as an occasional component of the syndrome, but more severe degrees of ataxia have generally led to such cases being classified as the Roussy-Levy syndrome (Roussy and Levy, 1926;1934).…”

“…Definite conclusions have been hampered by a paucity of autopsy studies. England and Denny-Brown (1952) considered that the conflicting opinions could be divided into those that upheld a primarily spinal pathology as opposed to those that favoured a peripheral neuropathy. They themselves concluded that the disorder was likely to be a 'radicular neuropathy'.…”

Globular neuropathy. A disorder of axons and Schwann cells.

“…Clinically it appears myelin sheath, suggests two possible ways at least y to resemble the variety of peroneal in which they could arise. Firstly, as the globules trophy with sensory loss as described, appear to be breakdown products of myelin there , by England and Denny-Brown (1952), might be a defect in the early stages of its degradaith more prominent hypoaesthesia. The tion.…”

Genetic heterogeneity in hereditary sensory and autonomic neuropathies: The need for improved ascertainment

“…Kindreds described by Denny-Brown and England, 11 Dyck et al, 5 and now by Dubourg et al 3 do not fit neatly into the classification based on class of neurons affected. They appear to fall somewhere between HMSN and HSAN.…”