Low-dose ACTH therapy for West syndrome: initial effects and long-term outcome.

BACKGROUND: Most Japanese pediatric neurologists attempt other treatments before using adrenocorticotropic hormone (ACTH) therapy for West syndrome (WS), and even then, they use only a low-dose synthetic ACTH to avoid serious adverse effects. In this multi-institutional study, the authors analyzed the initial effects, adverse effects, and long-term outcome in patients treated with low-dose synthetic ACTH in Japan. METHODS: The medical records of 138 patients with WS, who were treated with low-dose synthetic ACTH therapy for the first time at the authors' institutions between 1989 and 1998, were analyzed. RESULTS: At the end of ACTH therapy, excellent effect on seizures was noted in 106 of 138 (76%) patients, good effect in 23 (17%), and poor effect in 9 (7%). Initial effects on EEG were excellent in 53 of 138 (38%) patients, good in 76 (55%), and poor in 9 (7%). As for seizure prognosis at the time of follow-up, 51 of 99 (52%) patients were seizure-free, whereas 48 (48%) patients had seizures. Mental outcome was normal in 6 of 98 (6%) patients, mild mental retardation in 16 (16%), moderate mental retardation in 26 (27%), and severe mental retardation in 50 (51%). The initial effects of ACTH on seizures and long-term outcome were not dose dependent (daily dosage 0.005 to 0.032 mg/kg, 0.2 to 1.28 IU/kg; total dosage 0.1 to 0.87 mg/kg, 4 to 34.8 IU/kg). The severity of adverse effects correlated with total dosage of ACTH, and the severity of brain volume loss due to ACTH correlated well with the daily dosage and total dosage of ACTH. CONCLUSION: Low-dose synthetic ACTH therapy is as effective for the treatment of WS as the higher doses used in previous studies. The dosage of synthetic ACTH used in the treatment of WS can be decreased as much as possible to avoid serious adverse effects.

Elevated nerve growth factor levels in cerebrospinal fluid associated with progressive cortical atrophy.

Using a two-site enzyme-linked immunosorbent assay, we measured nerve growth factor (NGF) levels in cerebrospinal fluid (CSF) from 40 patients with or without brain atrophy due to various neurologic disorders. White matter and cortical atrophies were assessed by frontal horn index (FHI) and subarachnoid space area/inner skull space area (SSA/ISSA) ratio, respectively. CT findings of 40 patients were classified into 4 grades: normal (< mean + 2SD), grade I (mean + 2SD < or = or < mean + 4SD), grade II (mean + 4SD < or = < mean + 8SD), and grade III (mean + 8SD < or =). NGF levels in CSF were significantly elevated in 3 of 6 patients with grade III cortical atrophy and normal in 2 with grade II atrophy, 4 with grade I atrophy and 28 without atrophy. Cortical atrophy was progressive in the 3 with NGF elevation in CSF. With respect to white matter atrophy, NGF elevation was observed in none of 3 with grade III white matter atrophy, two of 4 with grade II atrophy, none of 3 with grade I atrophy and one of 30 without atrophy. The symptoms of the two of three with NGF elevation were progressive at the time of obtaining the CSF samples, while those of other patients without NGF elevation were non-progressive. The present study suggests that NGF elevation in CSF may reflect extensive cortical degenerative change.

IgA and IgG2 deficiency associated with zonisamide therapy: a case report.

PURPOSE: To report a previously undescribed adverse effect, IgA/IgG subclass deficiency associated with zonisamide (ZNS) therapy. METHODS: Serum IgA and IgG subclass levels were determined by the turbidimetric immunoassay and enzyme-linked immunosorbent assay, respectively, in a 2-year-old boy with postmeningitis sequelae who was treated by ZNS. RESULTS: Four months after initiation of ZNS, combined deficiency of IgA and IgG2 was noted. After cessation of ZNS, serum IgA level was promptly increased. IgG2 level was gradually increased, but remained subnormal after 7 months. CONCLUSIONS: This case documents, for the first time, the action of ZNS on IgG immune system as well as IgA system. If patients with ZNS therapy showed IgA deficiency and recurrent infections, it is preferable to check serum IgG subclass concentrations as well.

Intracranial germinoma with Down's syndrome: a case report and review of the literature.

BACKGROUND: Down's syndrome's association with malignancies such as leukemia is well known, but its association with brain tumor appears to be rare. We reviewed such rare cases of Down's syndrome and intracranial germ-cell tumor. CASE REPORT: A 10-year-old boy with Down's syndrome and intracranial germinoma located in the left basal ganglia is reported. The patient presented with right hemiparesis and was treated with a combination of surgery, chemotherapy with cisplatin and etoposide, and irradiation. CONCLUSIONS: We speculate that the percentage of germ-cell tumors is high among Down syndrome patients with brain tumors and that the most common site is the basal ganglia.

[Taste disturbance as an initial manifestation of Guillain-Barré syndrome in a 14-year-old girl].

We reported a 14-year-old girl who showed taste disturbance as an initial manifestation of Guillain-Barré syndrome (GBS). After an upper respiratory infection, she initially complained of taste disturbance, diplopia and salivation, followed by weakness in the legs and numbness in the extremities. On admission, she showed taste deficiency for sweet and sour sense, and a decline of salty and bitter taste. Weakness in the legs and disappearance of deep tendon reflexes were also noticed. We considered that the findings of electrophysiological examinations would conform to demyelinating features. Laboratory examinations revealed an increase of memory T cells in the peripheral blood and an elevated level of myelin basic protein in the cerebrospinal fluid. Based on clinical features and laboratory data, the diagnosis of GBS was made. We should keep taste disturbance in mind as one of the signs of GBS.