[The Wohlfart-Kugelberg-Welander disease. A clinical case with nonobstructive myocardiopathy].
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Biomedical subjects
Publications and source records attributed to P Bakouche.
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We describe two original cases of internal carotid artery dysgenesis associated with a malformative spectrum, which includes transsphenoidal encephalocele, optic nerve coloboma, hypopituitarism, and hypertelorism. Cephalic neural crest cells migrate to various regions in the head and neck where they contribute to the development of structures as diverse as the anterior skull base, the walls of the craniofacial arteries, the forebrain, and the face. Data suggest that the link between these rare malformations is abnormal neural crest development.
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After presenting two personal observations, the authors underline the fact that the neurological manifestations of Behçet's disease are more frequent than they were supposed to be. Their symptoms are overly manifold but have in common constant abnormality in the cerebrospinal fluid when an attack occurs. The affection is a very serious one demanding an early and well established diagnosis only to be obtained thanks to a clinical examination. Corticosteroïd therapy (1 mg/kg per day) either alone or associated with immuno-suppressive therapy may check the attack. Preventing relapses implies a non-stop treatment. Immuno-suppressive therapy seems to be the most efficient one.