Subacute sclerosing panencephalitis: clinical and magnetic resonance imaging evaluation of 36 patients.
TL;DR: It is 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.
Abstract: 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.
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TL;DR: Recent developments in diagnostics may expedite disease diagnosis and outbreak containment, while progress in understanding the molecular biology of Nipah virus could lead to novel therapeutics and vaccines for this deadly pathogen.
122 citations
TL;DR: Nipah virus (NiV) is an emerging zoonosis that frequently results in high case‐fatality and long‐term neurological assessments of survivors are limited.
Abstract: Objective
Nipah virus (NiV) is an emerging zoonosis. Central nervous system disease frequently results in high case-fatality. Long-term neurological assessments of survivors are limited. We assessed long-term neurologic and functional outcomes of 22 patients surviving NiV illness in Bangladesh.
Methods
During August 2005 and May 2006, we administered a questionnaire on persistent symptoms and functional difficulties to 22 previously identified NiV infection survivors. We performed neurologic evaluations and brain magnetic resonance imaging (MRI).
Results
Twelve (55%) subjects were male; median age was 14.5 years (range 6–50). Seventeen (77%) survived encephalitis, and 5 survived febrile illness. All but 1 subject had disabling fatigue, with a median duration of 5 months (range, 8 days–8 months). Seven encephalitis patients (32% overall), but none with febrile illness had persistent neurologic dysfunction, including static encephalopathy (n = 4), ocular motor palsies (2), cervical dystonia (2), focal weakness (2), and facial paralysis (1). Four cases had delayed-onset neurologic abnormalities months after acute illness. Behavioral abnormalities were reported by caregivers of over 50% of subjects under age 16. MRI abnormalities were present in 15, and included multifocal hyperintensities, cerebral atrophy, and confluent cortical and subcortical signal changes.
Interpretation
Although delayed progression to neurologic illness following Nipah fever was not observed, persistent fatigue and functional impairment was frequent. Neurologic sequelae were frequent following Nipah encephalitis. Neurologic dysfunction may persist for years after acute infection, and new neurologic dysfunction may develop after acute illness. Survivors of NiV infection may experience substantial long-term neurologic and functional morbidity. Ann Neurol 2007
113 citations
Cites result from "Subacute sclerosing panencephalitis..."
...Similar to the neuroimaging findings in several of our patients, MRI in SSPE is characterized by diffuse cortical and subcortical hyperintensities.(38,39) Follow-up CSF was obtained on only one of our patients (Subject 7), and we were unable to detect NiV or NiV-specific antibodies in this specimen....
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TL;DR: The differential diagnosis for multiple sclerosis (MS) in childhood and adolescence includes infectious, inflammatory, and neoplastic disorders as well as metabolic neurogenetic leukodystrophies, toxic leukaemia, and vascular conditions.
Abstract: The differential diagnosis for multiple sclerosis (MS) in childhood and adolescence includes infectious, inflammatory, and neoplastic disorders as well as metabolic neurogenetic leukodystrophies, toxic leukodystrophies, and vascular conditions. The evaluation is determined by the clinical and neuroradiologic presentation. A minimal diagnostic battery is proposed. More expanded evaluations are indicated for specific or atypical clinical presentations.
58 citations
TL;DR: Irrespective of the incidence of occurrence of SSPE in a community, a clinician should be aware of the wide spectra of EEG findings and provide valuable insight into the structural and clinical correlates of EEG changes in S SPE.
55 citations
Cites background from "Subacute sclerosing panencephalitis..."
...Imaging findings in SSPE are usually not correlated with the clinical stage of illness (Ozturk et al., 2002)....
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Journal Article•
TL;DR: MR spectroscopy showed findings suggestive of inflammation in stage II and findings of demyelination, gliosis, cellular necrosis, and anaerobic metabolism in stage III, which could be a promising technique for early diagnosis and treatment planning in cases of SSPE.
Abstract: BACKGROUND AND PURPOSE: Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, inflammatory neurodegenerative disease. Our aim was to determine the metabolic abnormalities of brain in early- and late-stage SSPE by using MR spectroscopy and to assess areas of involvement in the early stages when MR imaging findings were normal. METHODS: Children with stage II (n = 3) or III (n = 3) SSPE and 10 healthy, age-matched children underwent MR imaging, multivoxel MR spectroscopy, and short-echo single-voxel MR spectroscopy (SVS). Areas of involvement in the brain were determined with chemical shift imaging. For SVS, 2 × 2 × 2-cm voxels were placed in the frontal subcortical white matter (FSWM) and parieto-occipital white matter (POWM). N-acetylaspartate (NAA)/creatine (Cr), choline (Cho)/Cr, myo-inositol (Ins)/Cr, and NAA/Cho ratios were calculated. RESULTS: Comparisons of NAA/Cr, Cho/Cr, Ins/Cr and NAA/Cho ratios between patients and control subjects showed significant differences in FSWM and POWM (P CONCLUSION: MR spectroscopy showed findings suggestive of inflammation in stage II and findings of demyelination, gliosis, cellular necrosis, and anaerobic metabolism in stage III. MR spectroscopy could be a promising technique for early diagnosis and treatment planning in cases of SSPE.
54 citations
References
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Journal Article•
182 citations
TL;DR: A variable natural history was found in a standardized follow-up study of 118 patients with subacute sclerosing panencephalitis (SSPE) and only 20% followed the sequence:behavior change, mental deterioration, periodic attacks, severe debility, and death within a year.
Abstract: • A variable natural history was found in a standardized follow-up study of 118 patients with subacute sclerosing panencephalitis (SSPE). Only 20% followed the sequence: behavior change, mental deterioration, periodic attacks, severe debility, and death within a year. Six stages were identified: O, subtle psychointellectual symptoms; 1, obvious psychointellectual and neurological changes; 2, stereotyped attacks; 3, vegetative psychomotor condition; 4, improvement; 5, relapse. Illness began in stage 0 for 32%; 1, 36%; 2,19%; 1 and 2 simultaneously, 13%. In early development, 77% were hyperactive, and 27% had psychointellectual difficulties one to five years prior to obvious illness. The average duration of each stage was as follows: stage O, two years; 1, 2.5 months; 2, 7.5 months; 3, 5.5 months; 4, 3.5 years; 5, 1.5 years. Noteworthy improvements and plateaus occurred in more than half of the patients. In stage 4, improvement was long-term and substantial for 5% and modest for 18%. Survival followed an exponential curve with an average half-life of 1.8 years; 41% of the patients survived beyond two years. Half of the patients passed through neither stage 0 nor stage 4; their half-life was 0.7 years. The remainder had a half-life of 3.0 years.
150 citations
TL;DR: Subacute sclerosing panencephalitis is a neurodegenerative disease of childhood that is due to a persistent measles infection that is coexistent with the decline in natural measles infection.
136 citations
TL;DR: In conclusion, intraventricular IFN, combined with oral inosiplex, in the treatment of SSPE is recommended, and patients who had a slowly progressive disease responded best to treatment.
Abstract: We treated 22 patients with subacute sclerosing panencephalitis (SSPE) with intraventricular alpha-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.
119 citations
TL;DR: 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.
Abstract: Thirty-four MRI studies of 26 patients with subacute sclerosing panencephalitis are reported. Lesions of high signal intensity on T 2-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
93 citations