241. MULTIFOCAL CNS LANGERHANS CELL HIS- TIOCYTOSIS WITHOUT SYSTEMIC INVOLVEMENT Atiya Khan, Margaret Lee, and Irving Fish, New York, New York

Langerhans cell histiocytosis (LCH) is a disorder with prolifer- ation of Langerhans cells into multiple organs, including skin, liver, spleen, bone marrow, lungs, and on rare occasions brain. Although solitary primary brain lesions have been reported we describe the first patient with multifocal cerebral LCH and no systemic involvement. An 18-month-old girl presented with fail- ure to thrive and was found to have diabetes insipidus. Work-up revealed a thickened pituitary stalk which, on biopsy, was con- sistent with LCH. Follow-up MRI scans when she was 31/2 years revealed multiple enhancing nodular lesions bilaterally in the caudate nuclei, temporal lobes, frontal lobes, and multiple subependymal lesions. The right frontal lesion was biopsied and again showed LCH confirmed by S100 stain and Birbeck gran- ules on electron microscopy. A complete systemic work-up, in- cluding skeletal survey, bone scan, bone marrow biopsy, CT scans of chest and abdomen, and MRI of the spine and CSF, was entirely negative for systemic LCH. She has failed to respond to chemotherapy, but the extent of her lesions has stabilized. She remains neurologically intact and 3 years after onset still does not have any systemic involvement.

242. AFFECTIVE ILLNESS IN CHILDREN: PATTERNS OF PRESENTATION IN RELATION TO PUBERTAL MATURATION AND FAMILY HISTORY Warren A. Weinberg, Caryn R. Harper, Caitlin Davies, and Roger A. Brumback, Dallas, Texas and Oklahoma City, Okla- homa

Affective illness is now recognized as a common problem in all age groups. In children, it causes behavioral problems and school difficulty. However, the patterns of affective illness, while well documented in adults, have not been assessed in children and changes in these patterns with aging have not been studied. We evaluated the pattern of affective illness in a group of 100 normally intelligent, middle class, caucasian children (72 boys and 28 girls; age range: 2-20 yrs; mean: 10 yrs) with af- fective illness consecutively referred for school failure to the Pediatric Behavioral Neurology Program. Each child was eval- uated using established criteria. Part of the evaluation included assessment of pubertal stage (Tanner stages), family history, and course and pattern of symptoms. There were 65 children in Tan- ner stage 1 (T1), 9 in Tanner stage 2 (T2), and 26 in Tanner stages 3-5 (T3). Seven patterns of affective illness were identi- fied. In the prepubertal children (T1), disorders with manic symptoms were most common (69%): hypomania (12%), cy- clothymia (17%), juvenile rapid-cycling bipolar disorder (JRCBD) (12%), and dysthymia with bipolar symptoms (28%). In contrast, pubertal children (T3) have a predominance of syn- dromes with depressive symptoms (61%): dysthymia (15%) and depression (46%), along with JRCBD (23%). Only 2 children (stage T1) had classic manic-depressive disease. In the family histories, affective illness, alcoholism, and drug abuse were fre- quent, regardless of the child's pattern of symptoms. Character disorder and Briquet syndrome were also common, but thought disorder, suicide, and homicide were infrequent. This study sup- ports the hypothesis that the presentation of affective illness changes with age; manic features predominate in younger chil- dren, while depressive symptoms are more evident after puberty.

243. VERTEBRAL ARTERY DISSECTIONmA STROKE REGISTRY IS NEEDED Divya S. Khurana, Carsten G. Bonnemann, Elizabeth C. Dool- ing, and Eileen M. Ouellette, Boston, Massachusetts

Vertebral artery dissection (VAD) is an uncommon cause of stroke in children. Accuracy of diagnosis by MR angiography (MRA) instead of invasive transfemoral angiography (TFA) has been controversial. Need for anticoagulation and duration of such therapy also is debated. We report 2 boys with VAD. Pa- tient 1, 7 years of age, presented with hemiparesis and seizures. Patient 2, 4 years of age, presented with ataxia. Cardiac, hema- tologic, metabolic, and infectious investigations were negative. Initially, their MRAs were not thought to demonstrate regions of occlusion to explain the posterior circulation infarcts seen on CT and MRI. Patient 1 was discharged on aspirin (ASA) and read- mitted because of new infarcts. MRA showed VAD and distal basilar occlusion confirmed by TFA. After a total of 5 weeks of heparin and warfarin, he was rehospitalized with a contralateral stroke due to a pontine infarct. MRA of Patient 2 showed a filling defect which was interpreted as artifact. VAD was con- firmed on TFA. He was treated for a total of 6 mos with heparin and warfarin and since has been on daily ASA. A high index of suspicion of VAD may allow diagnosis on the basis of MRA alone. Previous reports have indicated good outcomes of VAD in children and adults whether treated with anticoagulation or not. A pediatric stroke registry is needed to access data about diag- nosis and therapy and information on long-term outcomes.

244. PERIPHERAL FACIAL NERVE PALSIES HER- ALDING RARE DISEASES Divya S. Khurana and Elizabeth C. Dooling, Boston, Massachusetts

Peripheral facial nerve palsy usually is idiopathic, traumatic, or postinfectious in etiology. We report 2 boys with peripheral fa- cial nerve weakness as the initial manifestation of unusual dis- eases. A 16-year-old boy presented with right facial weakness and bilateral parotid swelling. Chest radiography revealed ade- nopathy and mediastinal enlargement which, on biopsy, were found to be T cell lymphoma. Cranial MRI and CSF were nor- mal. He has received chemotherapy with only partial recovery of facial weakness. A 3-year-old boy presented with right facial weakness which remitted on oral steroids. Two months later, he developed swelling of the right mastoid and right ear drainage. Mastoid biopsy was consistent with Langerhans cell (LC) his- tiocytosis. After temporal bone radiation, he developed lethargy, somnolence, vomiting, and intermittent fevers. CT was nega- tive. CSF showed moderate pleocytosis, elevated protein, and low glucose. Immunohistochemical markers for LC were posi- tive. MRI disclosed massive hydrocephalus, diffuse leptomen- ingeal enhancement and thickening, and enhancement of the pi- tuitary stalk. CSF pleocytosis persists despite chemotherapy. Al- though cerebellar lesions have been seen in histiocytosis X, cranial nerve involvement, hydrocephalus, and meningeal in- volvement are most unusual. Direct facial nerve inf'dtration or a paraneoplastic process may explain their facial palsies.

245. NEUROIMAGING IN FIRST FOCAL FEBRILE SEIZURES Divya S. Khurana, Carsten G. Bonnemann, and Elizabeth C. Dooling, Boston, Massachusetts

Febrile seizures occur in 2-5% of young children. It has been

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