Aylin Öztürk

Subacute sclerosing panencephalitis: clinical and magnetic resonance imaging evaluation of 36 patients.

We studied 36 patients (24 males, 12 females), all of whom had definite subacute sclerosing panencephalitis with typical periodic complexes in their electroencephalograms and increased titers of measles antibody in serum and cerebrospinal fluid. Their clinical and laboratory findings on admission were reviewed retrospectively. The age at onset of symptoms varied from 4 to 23 years. The average age at onset of disease was 13.1 ± 4.18 years. The mean of the duration from the infection to the onset of subacute sclerosing panencephalitis was 9 years. Unusual symptoms, especially in the early periods of disease, included hemiparesis (7 patients), headache (3), generalized tonic-clonic seizures (6), absence seizure (1), nausea (3), and vomiting (3). Twenty-six cranial magnetic resonance imaging (MRI) and 12 computed tomography examinations were performed. Nine patients had normal MRI. In the early stages, lesions usually involved parieto-occipital corticosubcortical regions asymmetrically. In time, symmetric periventricular white-matter changes became more prominent. In addition to the common clinical findings in cases of subacute sclerosing panencephalitis reported in the literature, there were some different clinical features of the disease. Eventually, we concluded that there seems to be no correlation between the clinical stages and either the duration from the onset of subacute sclerosing panencephalitis or the MRI findings. (J Child Neurol 2002;17:25-29).

Correlation between clinical stages and EEG findings of subacute sclerosing panencephalitis

In this retrospective study 67 patients with SSPE seen between the years 1980 and 1998 were reviewed. Using the criteria of SSPE diagnosis (clinical signs, characteristic EEG patterns, high titres of measles antibodies in the serum and CSF), the patients were divided into two groups. Group A fulfilled all criteria, however, due to the inability of measuring measles antibody before 1987, it was not possible to observe the third criterion in Group B. Among 67 patients, groups A and B consisted of 51 boys and 16 girls ranging in age between 1 to 23 years, mean age 13.1. The male/female ratio was 3.1. The periodic EEG complexes (PCs) were usually bilateral, synchronous and symmetrical. PC amplitude asymmetry was seen in 12 patients and 2 patients had no PC synchronization between the hemispheres. Six patients had more than one form of PC. Delta activity in anterior hemispheres after PC was seen in 40 patients, mostly in stage 2A. Thirty-two patients had focal epileptiform abnormalities in multiple locations at every stage but most frequently in frontal, central and temporal regions. One patient had PC over both hemispheres and periodic lateralized epileptiform discharges (PLEDs) over the right hemisphere. The EEG findings described and observed in our study do not seem to be specific to SSPE but these findings were not atypical or unusual.

Lafora Body-Like Inclusions in a Case of Progressive Myoclonic Ataxia Associated with Coeliac Disease

Lafora’s disease is a rare familial disorder characterised by sei-zures, myoclonus and dementia and caused by mutations in theEPM2A gene, which codes for laforin, a protein with unknown func-tion [1]. The demonstration of periodic acid-Schiff (PAS)-positiveinclusions in sweat gland ducts of the skin has been established as apractical method for a reliable diagnosis [2, 3].Coeliac disease, another disorder associated with progressivemyoclonic ataxia, is fairly common. However, reported cases withboth biopsy-proven gluten-sensitive enteropathy and progressivemyoclonic ataxia have accumulated in previous years and the coexis-tence of neurologic manifestations in coeliac disease seems morethan a chance association [4, 5].