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Seitelberger's disease II

Related people

A rare familial form of demyalinating disease with early onset and absence of stainable myelin. The affected child is normal at birth and development is normal up to second year of life. Then the disturbance develops with difficulty in standing and walking; progressive deterioration of neurological function and speech; incontinence during sleep; nystagmus, strabismus, blindness, seizures, tendon hyporeflexia or areflexia; or initially pathologic reflexes, flexion, and deformation. Both sexes affected.

It is a variant of the Pelizaeus-Merzbacher syndrome and has some resemblance with the Hallervorden-Spatz disease.

See also:
Pelizaeus-Merzbacher syndrome (sudanophilic leukodystrophy) under Friedrich Pelizæus, German neurologist, 1850-1917.

Hallervorden-Spatz disease (progressive pallidal degeneration syndrome) under Julius Hallervorden, German neurologist, 1882-1965.

Bibliography

  • F. Seitelberger:
    e unbekannte Form von infantiler Lipoidspeicherkrankheit des Gehirns.
    Proceedings of the First International Congress of Neuropathology (Rome, September 8-13, 1952). Volume 3. Rosenberg and Sellier, Turin. 1952.
  • F. Seitelberger:
    Die Pelizaeus-Merzbachersche Krankheit. Klinisch-anatomische Untersuchung zum problem ihrer Stellung unter den diffusen Sklerosen.
    Wien Zschr Nervenh, 1954, 9: 228-289.

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