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[Davidenkow syndrome].

R Alberca1, I Chinchón, E Rafel

  • 1Servicio de Neurología y Dto, de Anatomía Patológica, HU Virgen del Rocío, Sevilla.

Archivos De Neurobiologia
|March 1, 1990
PubMed
Summary
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This case study details a rare peripheral neuropathy initially diagnosed as Davidenkow's syndrome. Later involvement of cranial nerves and lower motor neurons expanded the diagnostic considerations beyond hereditary motor and sensory neuropathies.

Area of Science:

  • Neurology
  • Genetics
  • Clinical Neuroscience

Background:

  • Davidenkow's syndrome is a rare, inherited neurological disorder characterized by progressive muscle weakness and atrophy.
  • It typically affects the scapuloperoneal muscles and can involve distal sensory impairments.

Observation:

  • A 31-year-old female presented with lifelong right foot walking difficulties, progressing to right scapuloperoneal amyotrophy and distal sensory signs.
  • Nerve conduction studies and biopsy confirmed peripheral neuropathy, initially suggesting Davidenkow's syndrome.

Findings:

  • At age 41, cranial nerve involvement (V, VII, XI, XII) on the right side indicated lower motor neuron dysfunction.
  • This cranial nerve and lower motor neuron involvement complicated the diagnosis, moving beyond typical Hereditary Motor and Sensory Neuropathies (HMSN) presentations.

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Implications:

  • This case highlights the diagnostic challenges in rare neuropathies that may present with overlapping features.
  • It suggests that some cases initially presumed to be Davidenkow's syndrome might represent broader or atypical forms of motor neuron disease or peripheral neuropathy.
  • Further research is needed to delineate the spectrum of genetic and clinical variability in such disorders.