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Disorders of the Nervous Tissue01:28

Disorders of the Nervous Tissue

Nervous tissue is a vital component of the human body's communication system, enabling us to perceive and respond to stimuli. However, like all other tissues, it is vulnerable to disorders and diseases that can significantly impact our neurological functioning.
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A Battery of Motor Tests in a Neonatal Mouse Model of Cerebral Palsy
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Infantile neurological Degos disease.

Tong Hong Yeo1, Grace Vassallo, Mary Judge

  • 1Department of Paediatric Neurology, Royal Manchester Children's Hospital, Manchester M13 9WL, UK. yeo@doctors.org.uk

European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society
|July 28, 2010
PubMed
Summary

Degos disease, a rare vasculopathy, can present neonatally with skin and neurological symptoms. This case highlights its potential in childhood neurological disease differential diagnosis.

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Area of Science:

  • Rare diseases
  • Vasculopathy
  • Dermatology
  • Neurology
  • Pediatrics

Background:

  • Degos disease (malignant atrophic papulosis) is a rare vasculopathy, typically presenting in adulthood with skin lesions and potential systemic involvement.
  • Neurological manifestations are varied, including stroke, seizures, and optic atrophy, suggesting blood vessel integrity defects.
  • While primarily considered an adult disorder, rare infantile cases have been documented.

Observation:

  • A female neonate presented with Degos disease, exhibiting characteristic skin lesions, ptosis, and generalized weakness.
  • The patient experienced progressive neurodevelopmental and physical decline.
  • CT scans revealed intracranial calcification, a feature noted in previous rare cases.

Findings:

  • This report details a unique case of neonatal-onset Degos disease.
  • The case underscores the severe systemic and neurological impact of the disease from birth.
  • Intracranial calcification was observed, correlating with prior case reports.

Implications:

  • The findings emphasize the importance of considering Degos disease in the differential diagnosis of neonatal and childhood neurological disorders with cutaneous manifestations.
  • Early recognition and diagnosis are crucial for managing this rare and progressive condition.
  • Further research into the etiology and management of neonatal Degos disease is warranted.