Hypertrophic neuropathy with spinal cord compression

doi:10.1016/0090-3019(82)90330-5

Surgical Neurology

Volume 18, Issue 4, October 1982, Pages 237-240

https://doi.org/10.1016/0090-3019(82)90330-5 Get rights and content

Abstract

The case of a 40-year-old patient with Dejerine-Sottas disease, who developed spinal cord compression from hypertrophic nerve roots, is presented. Six previously reported cases are reviewed. We discuss the characteristic myelographic changes seen in Dejerine-Sottas disease.

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Cited by (10)

Hypertrophied cauda equina presenting as intradural mass: Case report and review of literature
1998, Surgical Neurology
Background
Hereditary motor and sensory neuropathy types I and III usually lead to enlargement of peripheral nerves. Rarely, spinal nerve roots may also be involved, leading to radiculopathy and/or myelopathy.
Methods
This 44-year-old man with back and lower extremity radicular pain and distal lower extremity weakness and numbness was found to have a nonenhancing intradural mass that caused a nearly complete myelographic block from L1–L4. He underwent a decompressive laminectomy with intradural exploration.
Results
Hypertrophic but otherwise normal-looking nerve roots were observed. Subsequent electrodiagnostic testing and sural nerve biopsy confirmed that this patient had a previously unsuspected hereditary motor and sensory neuropathy (HMSN). His pain resolved, but at latest follow-up his weakness and numbness persisted.
Conclusions
Nonenhancing spinal intradural mass lesions may represent enlarged nerve roots, which have a number of potential etiologies. Electrodiagnostic studies and peripheral nerve biopsy are instrumental in establishing the diagnosis of HMSN.
Cervical spinal cord compression complicating the clinical course of Charcot-Marie-Tooth type 1
2015, BMJ Case Reports
Magnetic resonance imaging of suspected idiopathic bilateral C2 hypertrophic ganglioneuritis in dogs
2015, Journal of Small Animal Practice
Hypertrophic neuropathy of the cauda equina
2010, European Journal of Orthopaedic Surgery and Traumatology
Hypertrophic Neuropathy of the Cauda Equina: Case Report
2004, Neurosurgery
MR imaging of Dejerine-Sottas disease
1999, American Journal of Neuroradiology

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Hypertrophic neuropathy with spinal cord compression

Abstract

J Fac Radiol

Polynéurite chronique hypertrophique

Rev Neurol (Paris)

Observations on the syndrome of hypertrophic neuritis

Medicine (Baltimore)

Hypertrophic neuropathy, plain film and myelographic changes

Radiology

Zür kenntnis der hypertrophischen neuritis

Beitr Pathol Anat

Neuropathological changes in hereditary neu-ropathies: manifestations of the syndrome heredopathia atactica polyneuritiformis in the presence of the interstitial hypertrophic polyneuropathy

J Neuropathol Exp Neurol

A case of primary generalized amyloid disease with involvement of the veins

Brain

Lower motor and primary sensory neuron diseases with peroneal muscular atrophy

Arch Neurol

Peripheral Neuropathy

Hypertrophic interstitial neuropathy (Déjerine-Sottas Disease)

Cervical myelographic changes in hypertrophic interstitial polyneuropathy

Ann Neurol