365. ELASTIC FIBER DEGENERATION IN THE COS- TELLO SYNDROME Masato Mori, Takanori Yamagata, Yuuko Mori, Mariko Momoi, Mituhiro Nokubi, Takeshi Saito, and Yoshimistu Fukushima, Tochigi and Saitama, Japan

The Costello syndrome is characterized by redundant skin le- sions especially on hands and palms, curly hair, distinct facial appearance, and mental retardation. We have performed patho- logic examinations on 2 patients with this syndrome, and found abnormal conformations of elastic fibers of various tissues. Pa- tient 1, a 6-month-old boy having typical features of the Costello syndrome (e.g., redundant skin lesions, deep palmer and planter crease, large for gestational age at birth, poor feeding, charac- teristic facial appearance, dysrhythmia, mental retardation) died of rhabdomyolysis. Pathologic findings revealed fragmentation and disruption of elastic fibers in the skin and mucosa of the pharynx and larynx, while elastic fibers in the aorta, bronchus, and alveolae were preserved. His cardiac conduction system was abnormal. The pulmonary arteries were narrowed, but there was no emphysematous change. Patient 2 is a 5-year-old boy whose condition was diagnosed as the Costello syndrome. Skin biopsies revealed the disruption of elastic fibers. The Costello syndrome is a rare disorder, with unknown pathogenesis. The pathologic examinations of 2 patients with this syndrome revealed abnormal construction of elastic fibers, which is a typical finding of cutis laxa. It is suggested that the underlying pathology of these 2 diseases are closely related.

366. ELECTROPHYSIOLOGIC ASSESSMENT OF CHILDREN WITH PERIPHERAL NERVE INJURY DUE TO WAR OR ACCIDENT N. Bari~i~, Z. Mitrovi6, D. Jureni~, M. ~'.agar, A. Julia, and M. ~ogtarko, Zagreb, Croatia

Twenty-seven children aged 3-15 years were studied clinically and electrophysiologically. Twelve of these children had been wounded during the war by bullets and shell particles. Brachial plexus injuries occurred in 6 of 12 children, radial nerve injuries in 3, and either peroneal or ischiadical injuries in 3. Fifteen children had traumatic accidents, which caused median nerve injury in 4 of 15 children, ulnar injuries in 5 of 15, and radial or peroneal nerve lesions in 3 of 15. The children were assessed 1-15 months after injury occurred. Axonal damage with very low or inelicitable compound muscle action potential and fibrillations or positive denervation potentials occurred in 11 of 12 patients with war injuries and in 11 of 15 children after accidental trauma with mostly bone fractures. Complete neuron loss and total ax-

onal damage occurred in 9 of 12 children with war trauma and in 7 of 15 children with accidentally acquired nerve injury. Partial denervation was registered in 4 children with accidentally ac- quired nerve injury and in 3 children with war-acquired nerve injury. Conduction block and demyelination with signs of pe- ripheral nerve compression occurred in 4 of 15 children with accidental nerve injury. Slight improvement occurred after 7-18 months on conservative treatment in war-injured children, and after 2-7 months in accidentally acquired injuries as well as after surgical repair in both groups. We conclude that in war-injured children complete denervation with total axonal damage oc- curred more often followed by a slower, delayed, and often complicated improvement course. In children with accidental nerve injuries the course was significantly better.

367. AKINETIC MUTISM DUE TO DIPHENYLHYDAN- TOIN TOXICITY Sarenur T0ttinciio~lu, Mehmet Kantar, Can Candan, and Tu~rul Ozgtir, Izmir, Turkey

Akinetic mutism (AM) is a rare special unconsciousness state. In AM, the patient seems awake, lacks mental activity, is unable to speak, and does not respond to environmental stimulus. Cyclical sleep and awake states are preserved. Incontinence is present. Craniopharyngoamatous cyst, cerebrovascular accident, cardiac arrest, severe hypoglycemia, carbon monoxide intoxication, sev- eral drugs, central pontine myelinolysis, radiotherapy, trauma, tumor, hydrocephaly, Wernicke-Korsakoff disease, and tubercu- lous endocarditis might cause AM. We present a 12-year-old girl with epilepsy since the age of 10 months. She has been receiving DPH, 300 mg/day, for convulsions. On admission, gait disorder, ataxia, dysarthria, and horizontal nystagmus were present. She displayed AM, seemed awake, was unable to speak and under- stand, and was not cooperative. Neither spontaneous nor noxious stimuli-induced extremity movements were observed. Orofacial dyskinesia and incontinence were also present. Known vascular, cardiac, toxic, traumatic, infectious, tumor, and demyelinating factors for AM were all excluded. The patient had only mild cerebellar atrophy on MRI. She had abnormal BAEP response, which itself was not sufficient for CPM diagnosis. She had been taking DPH for 11 years and the serum DPH level was >40 txg/ml. Her cerebellar findings were attributed to the toxic levels of DPH. In the management of this patient, DPH was replaced by carbamazepine. Motor and mental activity returned within 2 months and she was symptom free when last seen after 5 months. We could not find any known cause of AM in our patient and strongly believe that DPH might be responsible. No similar ad- verse effect of DPH has been previously reported.

180 PEDIATRIC NEUROLOGY Vol. 11 No. 2