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Indian Journal of Psychiatry
Medknow Publications
Publication Date
  • Case Report
  • Medicine


Indian J. Psychiat. (1982), 24(2), 1 9 0 - 1 9 1 A CASE OF PYKNO-DYSOSTOSIS WITH PSYCHOSIS K. N. SRL\IVAS,i M.D., D.P.M. T. MURALI, ' M.B.B.S. P.S. SHIVAKUMAR,3 M.B.B.S. R. S. PANDEY,« M.D. B.Y.T. ARYA,' M.D. SUMMARY A rare case report of association of affective psychosis with pyknodysostosis is discussed. Pyknodysostosis is a rare mesodermal disorder first reported by Schuler in 1962. Maroteaux and Larmy (1965) later coined the term pyknodysostosis for the condition. The clinical features of this entity a r e : uni- form decrease in stature, frontal and parietal bossing, sutural diastasis, proptosis, beaked nose, dentate anomalies (double row of teeth), micrognathia, with short hands and feet. The diagnosis is confirmed by the roentegenography which reveals by increared density of all long bones, partial hypoplasia of distal phalanges, absence of ungual processes, open cranial sutures, small facial bones, narrow mandible flattened at its angle along with absence or atrophy of air sinuses. At times the lateral part of the clavicle may be absent. Laboratory investigations usually show no abnormali- ties. Elmore et al. (1966) have reported apparent deletion of the short arm of the small acroeintric chromosome 22 of the group G in one case-otherwise no chromo- somal abnormality has been reported so far. CASE REPORT Miss C, 50 years old unmarried lady, hailing from a rural Hindu nuclear family born out of a non-consanguineous union> brought by her brother to the psychiatry- walk-in-clinic at National Institute of Mental Heal th and Neurosciences, Banga- lore with the complaints of talking exces- sively, not taking food properly, neglecting personal hygiene and disturbed sleep of fifteen months duration. No history of mental re tardat ion, epilepsy or menta l illness in the family was reported. Birth and early developmental history not available. Pat ient reached the present status at the age of ten years. Menstrual history is reported to b

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