Hostname: page-component-8448b6f56d-dnltx Total loading time: 0 Render date: 2024-04-23T07:15:11.566Z Has data issue: false hasContentIssue false
  • English
  • Français

Hereditary Motor Sensory Neuropathy Type I Presenting as Scapuloperoneal Atrophy (Davidenkow Syndrome) Electrophysiological and Pathological Studies

Published online by Cambridge University Press:  18 September 2015

Gabriel M. Ronen ,
Noel Lowry ,
John H. Wedge ,
Harvey B. Sarnat  and
Alan Hill
Gabriel M. Ronen
Affiliation:
Departments of Paediatrics and Clinical Neurological Sciences, University of Saskatchewan
Noel Lowry
Affiliation:
Departments of Paediatrics and Clinical Neurological Sciences, University of Saskatchewan
John H. Wedge
Affiliation:
Department of Orthopedics, University of Saskatchewan
Harvey B. Sarnat
Affiliation:
Departments of Paediatrics, Pathology and Clinical Neurosciences, University of Calgary
Alan Hill*
Affiliation:
Departments of Paediatrics and Clinical Neurological Sciences, University of Saskatchewan
*
Division of Neurology, Department of Paediatrics, University of British Columbia, British Columbia's Children's Hospital, 4480 Oak Street, Vancouver, British Columbia, Canada V6H 3V4
Rights & Permissions [Opens in a new window]

Abstract:

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

A 14 year old boy with scapuloperoneal muscular atrophy, pes cavus, areflexia and distal sensory loss (Davidenkow syndrome) is described. Nerve conduction velocities were diminished. Sural nerve biopsy demonstrated a reduction in the number of myelinated fibers and early “onion-bulb” formation. These observations support the hypothesis that the scapuloperoneal amyotrophy associated with distal sensory loss may represent a variant of type I hereditary motor sensory neuropathy.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 13 , Issue 3 , August 1986 , pp. 264 - 266
Copyright
Copyright © Canadian Neurological Sciences Federation 1986

References

1.Oranski, W.Ueber einen hereditaeren Typus progressiver Muskel- dystrophie. Deutsch Z Nervenheilk 1927; 99: 147–155.CrossRefGoogle Scholar
2.Kazakov, VM, Bogorodinski, DK, Skorometz, AA.Myogenic scapuloperoneal syndrome. Muscular dystrophy in the K Kindred. Reexamination of the K family described for the first time by Oranski in 1927. Eur Neorol 1975; 13: 350–359.CrossRefGoogle Scholar
3.Feigenbaum, JA, Munsat, TL.A neuromuscular syndrome of scapuloperoneal distribution. Bull Los Angeles Neurol Soc 1970; 35: 47–57.Google ScholarPubMed
4.Thomas, PK, Calne, DB, Elliott, CF.X-linked scapuloperoneal syndrome. J Neurol Neurosurg Psychiatr 1972; 35: 208–215.CrossRefGoogle ScholarPubMed
5.Serratrice, G, Pellissier, JF, Ponget, J, Gastaut, JL, et al. Les syn- dromes scapulo-péroniers. Rev Neurol (Paris) 1982; 138:691–711.Google Scholar
6.Stark, D.Étude clinique et génétique d’une famille atteinted’atrophie musculaire progressive neurale. J Genet Hum 1958; 7: 1–32.Google Scholar
7.Kaeser, HE.Scapuloperoneal muscular atrophy. Brain 1965; 88: 407–418.CrossRefGoogle ScholarPubMed
8.Davidenkow, S.Scapulo-peroneal amyotrophy. Arch Neurol Psychiatr 1939;41: 694–701.CrossRefGoogle Scholar
9.Harding, AE, Thomas, PK.Distal and scapuloperoneal distribution of muscle involvement occurring within a family with Type 1 hereditary motor and sensory neuropathy. J Neurol 1980; 224: 17–23.Google Scholar
10.Serratrice, G, Pellissier, JF, Pouget, J.Trois cas d’amyotrophie scapulopéronière neurogène (syndrome de Davidenkow). Rev Neurol (Paris) 1984; 140: 738–740.Google Scholar
11.Tsukagoski, H, Takasu, T, Yoshida, M, et al. A family with scapuloperoneal muscular atrophy. Clin Neurol (Tokyo) 1969; 9: 511–517.Google Scholar
12.Toghi, H, Tsukagashi, H, Toykura, Y.Neurogenic scapuloperoneal syndrome with autosomal recessive inheritance. Clin Neurol (Tokyo) 1971; 11: 215–220.Google Scholar
13.Schwartz, MD, Swash, M.Scapuloperoneal atrophy with sensory involvement: Davidenkow’s syndrome. J Neurol Neurosurg Psychiatry 1975; 38: 1003–1007.CrossRefGoogle ScholarPubMed