Successful treatment of subacute sclerosing panencephalitis with long-term intrathecal large dose of alpha-interferon--a case report
Horiguchi, Y.; Ohya, T.
No to Hattatsu 27(3): 231-237
1995
ISSN/ISBN: 0029-0831 PMID: 7544989 Document Number: 442635
Recent effective treatments for subacute sclerosing panencephalitis (SSPE) are oral inosiplex and intrathecal interferon therapy. The former seems to be effective for life expectancy but not for neurological disability, and the latter is a debating antiviral treatment although favorable reports are increasing. A 12 year-10 month-old boy with SSPE was presented as a successful case of prolonged effectiveness in both neurological symptoms and life expectancy. There are no significant side effects by the 200 weeks treatment of intrathecal alpha-interferon of large dose (6 million unit/dose/week) and ordinary dose of inosiplex (100 mg/kg/day). Neurological disability index (Dyken) improved from 70% to 10%; speech and higher cerebral functions improved from aphasia and almost vegetative states to verbal communicable level; motor dysfunction from bedridden to wheel chair level; and myoclonic and other seizures were controlled. Laboratory data also improved; periodic synchronized discharge (PSD) and other paroxysmal discharges disappeared and alpha activity that had once disappeared in the background activity of active stages reappeared in the EEGs. Measles antibodies in serum and CSF improved, and oligoclonal bands disappeared. However, diffuse brain atrophy remained on neuroimaging. Long-term intrathecal large dose treatment with alpha-interferon is effective and safe. It should be started as soon as the diagnosis is made, and a trial of large-dose and long-term therapy may be worthy even for more advanced cases irrespective of progress of neuroimages.