Kalbiye Yalaz

Intraventricular interferon and oral inosiplex in the treatment of subacute sclerosing panencephalitis

We treated 22 patients with subacute sclerosing panencephalitis (SSPE) with intraventricular α-interferon (IFN) and inosiplex PO and followed them for 2 to 54 months. Three deaths occurred. Clinical improvement, demonstrated by decreasing scores on the Neurological Disability Index, occurred in 11/22 (50%); five patients became stable, and the progression rate of the disease decreased in three. The remission rate was significantly higher than untreated controls from the same institution. Patients who had a slowly progressive disease responded best to treatment. Serious side effects were rare. We recommend intraventricular IFN, combined with oral inosiplex, in the treatment of SSPE.

MRI findings in subacute sclerosing panencephalitis

Thirty-four MRI studies of 26 patients with subacute sclerosing panencephalitis are reported. Lesions of high signal intensity on T2-weighted images are the most common finding; they frequently involve the periventricular or subcortical white matter. Lesions tend to start in the cortex-subcortical white matter and progress with periventricular white matter involvement and diffuse cerebral atrophy. Pial and parenchymal contrast enhancement, local mass effect of parenchymal lesions, and involvement of the splenic portion of the corpus callosum are not infrequent. Basal ganglia and brainstem lesions were rare in this series. Although cortical and subcortical lesions have some correlation with clinical findings, the extent and location of the periventricular white matter lesions and cerebral atrophy did not reflect the neurologic status in many patients. NEUROLOGY 1996;47: 1278-1283

Cranial MR findings in Wilson's disease

Purpose: to define various cranial Mr appearances in Wilsons disease (WD). Material and Methods: MR examinations of 30 patients (9–44 years old) with WD were retrospectively reviewed. Six patients were asymptomatic siblings. Three other patients had isolated hepatic involvement, one with no symptoms. the remaining 21 patients had neurological involvement, 7 of whom had the mixed form of the disease. Nine patients had hepatic dysfunction, the 3 with isolated hepatic involvement and 6 of the 7 with the mixed form. Results: All symptomatic patients (n=23) had abnormal MR examinations. Atrophy was present in the majority of them. the most frequently involved sites were putamen (18/21) and pons (18/21) in patients with neurological abnormality. the putaminal lesions showed a consistent pattern of symmetric, bilateral, concentric-laminar T2 hyperintensity. Putaminal lesions were lacking in only 3 patients with neurological involvement, all of whom were relatively old and had had the disease for a longer duration. Most of the patients with hepatic dysfunction (8/9) had increased T1 signal intensity in the basal ganglia, particularly in the globus pallidus. Pontine involvement always included the dorsal aspect of the pons, however, in some cases the central portion of pons was also affected but ventrolateral longitudinal fibers were spared. Midbrain (16/21), thalamic (10/21) and caudate nucleus lesions (9/21) were also encountered. in a few patients cortical and subcortical white matter lesions were present with a predilection to the frontal lobe, particularly the precentral region. in one patient, a hemorrhagic focus was identified within the white matter lesion. Conclusion: on T2-weighted images, WD is suggested by: atrophy; putaminal lesions with a pattern of symmetric, bilateral, concentric-laminar T2 hyperintensity; and the involvement of the pars compacta of the substantia nigra, periaqueductal gray matter, the pontine tegmentum and the thalamus. the hepatic component of WD may cause increased T1 signal intensity in basal ganglia. in the adult age group, the basal ganglia lesions may be different from those in the pediatric group; the putaminal lesions may not be present; the globus pallidus and substantia nigra may show increased hypointensity on T2-weighted images. Cortical and subcortical lesions may also be present with a predilection to the frontal lobe.

Long-term follow-up of patients with subacute sclerosing panencephalitis treated with intraventricular alpha-interferon

Article abstract-We treated 22 patients with subacute sclerosing panencephalitis (SSPE) with intraventricular alpha-interferon (alpha-IFN) and oral inosiplex between 1986 and 1991. The follow-up for 56 to 108 months demonstrates a higher survival rate in these patients compared with those who did not receive alpha-IFN. However, eight of 11 patients whose condition improved after alpha-IFN treatment and five of five patients whose condition stabilized after alpha-IFN experienced neurologic deterioration 6 to 90 months after treatment; three of 11 and four of five died. The use of inosiplex did not influence the prognosis. Re-administration of the same regimen was not effective in one patient. Treatment-induced remissions in SSPE can be temporary, analogous to spontaneous remissions. Longer treatment with higher doses, or combinations of drugs, may be required. NEUROLOGY 1997;48: 526-528

Tissue inflammatory response in subacute sclerosing panencephalitis (SSPE).

The pattern of inflammatory infiltration was studied in the frontal brain biopsies of 28 cases with subacute sclerosing panencephalitis (SSPE) by immunohistochemistry. Lymphocytic infiltration and gliosis were common pathologic findings. CD4+ T lymphocytes were often observed in perivascular areas and CD8+ lymphocytes in the parenchyma. B lymphocytes were located in large perivascular cuffs associated with longer and slower disease. Major histocompatibility complex antigens, interferon-γ, and tumor necrosis factor-α (TNF-α) were expressed in endothelial and glial cells. The inflammatory lesions in subacute sclerosing panencephalitis consist of various cell subtypes and cytokines localized in particular areas of the brain tissue and show certain associations with clinical course. (J Child Neurol 2001;16:895-900).

Myasthenia gravis in childhood

Clinical features, serum acetylcholine receptor antibody (AChRAb) titres and course were reviewed in a series of 25 congenital (CMG) and 30 juvenile (JMG) myasthenia gravis cases to recognize characteristics of childhood‐onset myasthenia and its subgroups. The initial symptom for CMG is ptosis accompanied or followed by generalized weakness; myasthenic crises do not occur and spontaneous remissions are rare. In JMG, the distribution of weakness remains the same, but the severity fluctuates: spontaneous remissions (6 patients) and myasthenic crises (10 patients) are observed. Good response to anticholinesterase drugs is slightly more frequent in JMG (62 versus 41 %). AChRAbs were present in 9/26 JMG tested, girls with onset after 11 years being more likely to be Ab‐positive. Since patients with autoimmune myasthenia and a young age of onset are often seronegative, clinical features such as changing distribution of weakness, fluctuating severity, or response to treatment might be considered as supportive criteria for differentiating JMG from CMG.

Occidental type cerebromuscular dystrophy: a report of eleven cases.

Occidental type cerebromuscular dystrophy (OCMD) forms a substantial distinct group within congenital muscular dystrophy (CMD). These patients invariably present with amyotrophy, multiple joint contractures, facial muscle involvement, normal or nearly normal intelligence, leukodystrophic appearance on CT scan, and dystrophic changes in muscle.

Long-Term Prognosis After Neonatal Tetanus

Twenty‐four Turkish children who had had neonatal tetanus were evaluated by means of physical and neurological examinations and psychometric tests at four to 15 years of age. Enuresis, mental retardation and growth retardation were frequent findings. The prevention of neonatal tetanus is important not only because of the high mortality rate but also because of its sequelae.

β-Interferon Plus Inosiplex in the Treatment of Subacute Sclerosing Panencephalitis

We treated seven patients with subacute sclerosing panencephalitis with β-interferon and oral inosiplex for 2 to 15 months. Stabilization or improvement was observed in three patients. The effect of treatment was equivocal in two other patients who became stable. The disease continued its progression in the remaining two patients who died. Treatment shorter than 2 months was not effective. Changes in electroencephalograms (EEG), magnetic resonance images (MRI), or cerebrospinal fluid measles antibody levels did not have a close correlation with clinical course. These results suggest that β-interferon might be efficient in some patients with subacute sclerosing panencephalitis and justify its trial in larger studies with longer follow-up. (J Child Neurol 1998; 13:557-559).

Phenylketonuria in Pediatric Neurology Practice: A Series of 146 Cases

The neurologic manifestations of patients with phenylketonuria treated at different ages are illustrated in this series of 146 cases, including 9 sib pairs. In addition to well-known findings such as mental retardation, autistic features, microcephaly, and tremor, motor retardation was common and responded promptly to dietary treatment. Hypotonia and diminished reflexes were more frequent findings than hypertonia. Four sib pairs showed divergent features, such as the later-treated sibling having higher function than the early-treated one. Because siblings have a similar genotype and similar environmental and dietary conditions, this observation can be explained by differences in phenylalanine transport to the brain or additional metabolic or perinatal factors influencing the neurologic outcome. (J Child Neurol 2006;21:987—990; DOI 10.2310/7010.2006.00228).