patients with amyotrophic lateral sclerosis and other motor neuron diseases. Neurology 1991;41(Suppl 1):314 7 Younger DS, Rowland LP, Latov N, et al. Lymphoma, motor neuron diseases and amyotrophic lateral sclerosis. Ann Neurol 1991;29:78-86 8. Hemachudha T, Phanuphak P, Johnson RT, et al. Neurologic complications of Semple-typerabies vaccine: clinical and immunologic studies. Neurology 1987;37:5 50-5 56 9. Rubin RH, Hattwick MAW, Jones S, et al. Adverse reactions to duck embryo rabies vaccine. Range and incidence. Ann Intern Med 1973;78:643-649 10. Javier RS, Kunishita T, Koike F, Tabira T. Semple rabies vaccine: presence of myelin basic protein and proteolipid protein and its activity in experimental allergic encephalomyelitis. J Neurol Sci 1989;93:221-230 11. Ubol D, Hemachudha T, Whitaker JN, Griffin DE. Antibody to peptides of human myelin basic protein in post-rabies vaccine encephalomyelitis sera. J Neuroimmunol 1990;26:107-111 12. Gluck R, Matthieu JM, Wegmann A, Mean F. Absence of myelin basic protein in an improved purified duck embryo rabies vaccine. Neurochem Pathol 1986;4:69-75 13. Arnason BG, Asbury AK. Idiopathic polyneuritis after surgery. Arch Neurol 1968;18:500-507
Intraventricdar Interteron Treatment for Subacute Sclerosing Panencephalitis I. Wirguin, MD, I. Steiner, MD, T. Brenner, PhD, and 0. Abramsky, MD, P h D In a recent issue of the Annuls of Neurology, Miyazaki and associates [l] report marked improvement in an 8-year-old boy with subacute sclerosing panencephalitis (SSPE) who was treated experimentally with intrathecal alpha interferon (IFN). The clinical response appeared to be IFN dosedependent. We have had experience with 5 SSPE patients who were treated with intrathecal IFN over prolonged periods. The first 3 patients, previously reported 123 though uncited by Miyazaki and associates, were given IFN doses of 106units twice weekly for up to 2 years. Clinical stabilization or mild improvement was observed in all 3. In a fourth patient, an 8 year-old-buy who was treated by a similar regimen for 6 months, disease progression was not halted. Treatment was discontinued when the boy reached a vegetative state. In the fifth patient, a 19-year-old man, apparent stabilization occurred until therapy was temporarily discontinued due to severe ventriculitis necessitating removal of the Ommaya reservoir. Subsequently IFN therapy was resumed via a spinal implantable continuous infusion pump (Shiley Infuseaid Inc). The patient has been receiving a weekly dose of 30 x lo6 units of IFN for over 2 years. Progression of the disease seems to be arrested but major improvement has not occurred. SSPE is characterized by considerable variability of the clinical course 131. The response to IFN administration may likewise be variable. Some patients may exhibit remarkable remissions, whereas the majority of patients show clinical stabilization or no response. Prolonged intrathecal IFN therapy entails high costs and effort and is associated with major
complications (see below). Because the number of anecdotal reports is rising, a controlled clinical trial is indicated to settle the issues of IFN efficacy in SSPE and the optimal dose and administration schedule. Finally, prolonged intrathecal IFN administration is not an innocuous procedure. In our 5 patients we observed 4 episodes of life-threatening ventriculitis 141, and 2 patients developed hemiparesis secondary to localized leukoencephalopathy around the intraventricular catheter. Therefore, novel means of administration, with an incorporated microfilter, such as an intrathecal infusion pump, should be employed to minimize possible deleterious side effects.
Department of Nearoloa Hudzssah University Hospital JeruJalem. Israel
References 1. Miyazaki M, Hashimoto T, Fujino K, er al. Apparent response of subacute sclerosing panencephalitis to intrathecal interferon alpha. Ann Neural 1991;29:97-99 2. Steiner 1, Wirguin I, Morag A, Abramsky 0. Intraventdcular interferon treatment for subacute sclerosing panencep;ialitis. J Child Neural 1989;4:20-23 3. Risk WS, Haddad FS. The variable natural history of subacute sclerosing panencephalitis: a study of 118 cases from the middle east. Arch Neural 1979;36:610-614 4. Siegal T, Pfeffer R, Steiner I. Antibiotic therapy for infected Ommaya reservoir systems. Neurosurgery 1988;22:97-100
Treatment of Subacute Sclerosing Panencephalitis with Alpha Interferon Generoso G. Gascon, MD," Soad Yamani, MD," A. Cafege, Pharm D,? L. Flock, RPh, MSc,t S. Al-Sedairy, PhD,f R. S. Parhar, MS,S J. Crowell, RN, REEGT,§ Michael Nester, PhD,* Imad Kanaan, MD," and Mohammed A. Jallu, MD" Miywaki and colleagues 11) reported a single patient with subacute sclerosing panencephalitis (SSPE) whose neurological deterioration was reversed and recovery enhanced by increasing weekly doses of intrathecal human alpha interferon (IFN-a) (1,500,OOO-3,OOO,OOO IU); the patient remains in remission. In addition to those referenced in Miyazaki's article, others 12-51 have reported varying results in IFN-a administered intrathecally o r intraventricularly, but none with such remarkable reversal of hndings and sustained improvement. SSPE is endemic in Saudi Arabia. We have seen 40 patients in 3 years. O n e of our patients, a 12-year-old boy, began deteriorating in school performance 22 months ago, followed by falls and jerky neck and arm movements, forgetfulness, and easy crying. He was first seen for a neurological examination 18 months ago in early Jabbour stage I1 (Freeman stage IB). Cerebrospinal fluid (CSF) antimeasles antibody (AMA) titer and CSF IgG were increased. ElectroencephaIogram (EEG) showed preserved background activity Annals of Neurology
Vol 30 No 2
August 1991 227
Intraventricdar Interteron Treatment for Subacute Sclerosing Panencephalitis I. Wirguin, MD, I. Steiner, MD, T. Brenner, PhD, and 0. Abramsky, MD, P h D In a recent issue of the Annuls of Neurology, Miyazaki and associates [l] report marked improvement in an 8-year-old boy with subacute sclerosing panencephalitis (SSPE) who was treated experimentally with intrathecal alpha interferon (IFN). The clinical response appeared to be IFN dosedependent. We have had experience with 5 SSPE patients who were treated with intrathecal IFN over prolonged periods. The first 3 patients, previously reported 123 though uncited by Miyazaki and associates, were given IFN doses of 106units twice weekly for up to 2 years. Clinical stabilization or mild improvement was observed in all 3. In a fourth patient, an 8 year-old-buy who was treated by a similar regimen for 6 months, disease progression was not halted. Treatment was discontinued when the boy reached a vegetative state. In the fifth patient, a 19-year-old man, apparent stabilization occurred until therapy was temporarily discontinued due to severe ventriculitis necessitating removal of the Ommaya reservoir. Subsequently IFN therapy was resumed via a spinal implantable continuous infusion pump (Shiley Infuseaid Inc). The patient has been receiving a weekly dose of 30 x lo6 units of IFN for over 2 years. Progression of the disease seems to be arrested but major improvement has not occurred. SSPE is characterized by considerable variability of the clinical course 131. The response to IFN administration may likewise be variable. Some patients may exhibit remarkable remissions, whereas the majority of patients show clinical stabilization or no response. Prolonged intrathecal IFN therapy entails high costs and effort and is associated with major
complications (see below). Because the number of anecdotal reports is rising, a controlled clinical trial is indicated to settle the issues of IFN efficacy in SSPE and the optimal dose and administration schedule. Finally, prolonged intrathecal IFN administration is not an innocuous procedure. In our 5 patients we observed 4 episodes of life-threatening ventriculitis 141, and 2 patients developed hemiparesis secondary to localized leukoencephalopathy around the intraventricular catheter. Therefore, novel means of administration, with an incorporated microfilter, such as an intrathecal infusion pump, should be employed to minimize possible deleterious side effects.
Department of Nearoloa Hudzssah University Hospital JeruJalem. Israel
References 1. Miyazaki M, Hashimoto T, Fujino K, er al. Apparent response of subacute sclerosing panencephalitis to intrathecal interferon alpha. Ann Neural 1991;29:97-99 2. Steiner 1, Wirguin I, Morag A, Abramsky 0. Intraventdcular interferon treatment for subacute sclerosing panencep;ialitis. J Child Neural 1989;4:20-23 3. Risk WS, Haddad FS. The variable natural history of subacute sclerosing panencephalitis: a study of 118 cases from the middle east. Arch Neural 1979;36:610-614 4. Siegal T, Pfeffer R, Steiner I. Antibiotic therapy for infected Ommaya reservoir systems. Neurosurgery 1988;22:97-100
Treatment of Subacute Sclerosing Panencephalitis with Alpha Interferon Generoso G. Gascon, MD," Soad Yamani, MD," A. Cafege, Pharm D,? L. Flock, RPh, MSc,t S. Al-Sedairy, PhD,f R. S. Parhar, MS,S J. Crowell, RN, REEGT,§ Michael Nester, PhD,* Imad Kanaan, MD," and Mohammed A. Jallu, MD" Miywaki and colleagues 11) reported a single patient with subacute sclerosing panencephalitis (SSPE) whose neurological deterioration was reversed and recovery enhanced by increasing weekly doses of intrathecal human alpha interferon (IFN-a) (1,500,OOO-3,OOO,OOO IU); the patient remains in remission. In addition to those referenced in Miyazaki's article, others 12-51 have reported varying results in IFN-a administered intrathecally o r intraventricularly, but none with such remarkable reversal of hndings and sustained improvement. SSPE is endemic in Saudi Arabia. We have seen 40 patients in 3 years. O n e of our patients, a 12-year-old boy, began deteriorating in school performance 22 months ago, followed by falls and jerky neck and arm movements, forgetfulness, and easy crying. He was first seen for a neurological examination 18 months ago in early Jabbour stage I1 (Freeman stage IB). Cerebrospinal fluid (CSF) antimeasles antibody (AMA) titer and CSF IgG were increased. ElectroencephaIogram (EEG) showed preserved background activity Annals of Neurology
Vol 30 No 2
August 1991 227
