A unique case of central diabetes insipidus (DI) associated with transient pituitary stalk enlargement: close observation over several years using magnetic resonance imaging (MRI) and hypophysial endocrine tests.

We had the opportunity to closely observe a unique case of central diabetes insipidus (DI), in which dramatic changes in both radiological findings and hypophysial functions were seen. A 63-year-old female developed central DI, and magnetic resonance imaging (MRI) revealed a mild thickening of the pituitary stalk and lack of hyperintense signal associated with normal neurohypophysis on T1-weighted images. About three months later, the stalk was found to be remarkably expanded like neoplasm; however, anterior pituitary functions were almost normal on that occasion, except for the absence of GH response to an insulin tolerance test. About nine months after the onset of DI, secondary hypoadrenalism and hypothyroidism, which required replacement therapy, developed transiently, but recovered about one year later. Results of hypophysial endocrine tests during this period showed that the dysfunction was predominantly suprapituitary in nature. As time passed, the stalk lesion began to shrink spontaneously and another MRI, obtained five years after the onset of DI, disclosed normal findings for the infundibulo-hypophysial system, except for lack of the hyperintense signal of the neurohypophysis. The patient has since been healthy, except for the DI, which has been controlled by treatment with vasopressin. We report here a unique case of central DI associated with transient pituitary stalk enlargement.

Clinical and electroencephalographic studies of postencephalitic epilepsy.

We retrospectively reviewed the clinical features and results of investigation of 62 patients with acute encephalitis or encephalopathy in the acute phase to identify the prognostic factors in the development of postencephalitic epilepsy. Patients who later developed epilepsy showed more marked disturbance of consciousness, convulsions, and seizure activities on EEG during the acute phase than those who did not. There was no relation between CSF abnormalities and postencephalitic epilepsy. Control of convulsions during the acute phase, especially of status epilepticus, seems to be important to improve the prognosis.

The effect of intraventricular interferon on subacute sclerosing panencephalitis.

Two patients with subacute sclerosing panencephalitis (SSPE) were treated with intraventricular alpha interferon (IFN-alpha) via an Ommaya reservoir for 20-57 months. The clinical course of the disease was followed for 20-67 months. Clinical improvement was observed after daily intraventricular administration of IFN in one case. There were no serious complications or side effects during interferon therapy except for the fever. Intraventricular administration of IFN appears superior to intrathecal administration for long-term treatment in several respects and is considered to be a potential therapeutic modality for SSPE.

Congenital muscular dystrophy of non-Fukuyama type with characteristic CT images.

A 9-year-old Japanese boy with congenital muscular dystrophy (CMD) with normal intelligence was presented. He was extremely floppy and had joint contractures since birth. Motor milestones were delayed and he did not learn to walk alone. Intellectual development was normal and no convulsions were observed. On physical examination at 9 years old, he had diffuse muscle weakness and atrophy and flexion contractures of joints. Creatine kinase was normal and IQ was 95. Biopsied muscle showed myopathic changes consistent with muscular dystrophy. CT scans of the head revealed diffuse low density area in the white matter of the cerebrum. These findings suggest central nervous system involvement in CMD is not confined to Fukuyama-type CMD.

Selective immunoglobulin M deficiency associated with disseminated molluscum contagiosum.

A 16-year-old girl with disseminated molluscum contagiosum (MC) was found to have a very low level of serum IgM, elevated levels of IgG and IgA, and a high level of IgE. She had normal numbers of peripheral blood IgM+, IgG+ and IgA+ B-lymphocytes but their terminal differentiation into plasma cells could not be induced by pokeweed mitogen (PWM) in vitro. On the other hand, the patient's T-cells showed normal helper functions in the PWM system and normal interferon (IFN) production in vitro. However, the IgM+ B-cells can be induced to differentiate into IgM secreting cells by Epstein-Barr virus (EBV), suggesting that the genetic mechanism for synthesis of the component immunoglobulin proteins is present. T-cell functions were impaired, as shown by delayed type cutaneous hypersensitivity (DTH) and mitogen response. The data suggest that the selective IgM deficiency of the patient is due mainly to defects in B-cells at the terminal differentiation stage, but immunological abnormalities are present in both B and T-cell systems. Neutrophil functions examined were normal. MC was treated by intravenous injection of IFN without any side effects; however, no clinical improvement was achieved.

Plasmapheresis in the treatment of the Guillain-Barré syndrome in childhood.

Four patients (two boys and two girls, 6 to 13 years of age) with the Guillain-Barré syndrome (GBS) exhibiting such pronounced symptoms as dyspnea and dysphagia were treated with plasmapheresis using an intermittent flow system or double filtration plasmapheresis. The duration of the disease before plasmapheresis varied between 4 and 11 days. Three of the four patients experienced marked improvement during the course of plasmapheresis, but one patient had only partial improvement. No serious complications occurred. Good clinical and electrophysiologic recovery enjoyed by most patients treated with plasmapheresis appears to confirm the efficacy of plasmapheresis in children with severe GBS.