Features Of Subacute Sclerosing Panencephalitis Research Articles

Seizures as Presenting Feature of Subacute Sclerosing Panencephalitis: a Systematic Review of Case Reports and Case Series

Seizures as Presenting Feature of Subacute Sclerosing Panencephalitis: a Systematic Review of Case Reports and Case Series

The spectrum of MRI findings in subacute sclerosing panencephalitis with clinical and EEG correlates

Reports of magnetic resonance imaging (MRI) findings in subacute sclerosing panencephalitis (SSPE) with clinical and electroencephalography (EEG) correlation are few. To describe the MRI features of SSPE and correlate with its clinical features and EEG. Clinical, MRI and EEG data of 33 patients (male/female 19/14; age: 11.4 ± 5.3 yr) were reviewed. All patients had raised IgG anti-measles antibody (≥ 1:625) in cerebrospinal fluid. Their clinical features were myoclonus (100%), seizures (42%), visual disturbances (18%), pyramidal signs (30%), extrapyramidal symptoms (27%), and cognitive impairment (79%) and behavioral changes (21%). EEGs were universally abnormal: diffuse slowing of background activity-all; periodic complexes-27 (symmetrical): High voltage delta waves (55%) and sharp & slow wave discharges (45%). MRI findings included – normal (9%), cerebral atrophy (78%), white matter (76%) and basal ganglia (6%) changes. Restricted diffusion in periventricular/subcortical region was noted in 4/23 patients (stage 2). Magnetic resonance spectroscopy showed decreased N-acetyl aspartate peaks and elevated choline peaks in 75% and lactate peak in 25% (stage 3) of patients. Correlation of background delta activity among patients with MRI signal abnormalities and those without any signal change was noted (P=0.02). There was significant correlation between visual deficits and bilateral parieto-occipital white matter signal changes (P=0.03). There was no other EEG-MRI or clinical-MRI correlation. MRI abnormalities in SSPE are diverse and may or may not reflect underlying pathology. Visual deficits were observed in patients with parieto-occipital white matter signal change. Restricted diffusion of signal abnormalities and lactate peak in magnetic resonance spectroscopy were uncommon and it may add to better understanding.

臨床調査個人票からみた亜急性硬化性全脳炎 (SSPE) の疫学像

臨床調査個人票からみた亜急性硬化性全脳炎 (SSPE) の疫学像

Catatonia as the Initial Presenting Feature of Subacute Sclerosing Panencephalitis

Catatonia as the Initial Presenting Feature of Subacute Sclerosing Panencephalitis

Atypical Presentations of Subacute Sclerosing Panencephalitis in Two Neurologically Handicapped Cases

Subacute sclerosing panencephalitis (SSPE) is a neurodegenerative disorder caused by persistent measles infection. Here, we report two neurologically handicapped cases presenting with atypical features of SSPE. Patient 1 who had mild mental retardation manifested acute encephalopathy with partial seizures and hemiplegia, mimicking encephalitis. He showed a fulminant course without myoclonia or a periodic electroencephalogram complex. Although SSPE is usually associated with an increased diffusion pattern, diffusion-weighted imaging of our patient showed decreased diffusion in the right hippocampus. Patient 2 with infantile hemiparesis presented with secondary generalized seizures, followed by asymettrical myoclonias involving the side contralateral to the hemiparesis. A periodic electroencephalogram complex was absent on the previously damaged brain regions. Our findings show that preexisting neurological disorders may modify the clinical or electrophysiological findings of SSPE, leading to atypical presentations. SSPE should be considered in the differential diagnosis of acute encephalopathy with lateralizing signs or unidentified seizures. Decreased diffusion resolution in diffusion-weighted-imaging may correlate with rapid clinical progression in SSPE.

Bilateral Macular Retinitis as the Presenting Feature of Subacute Sclerosing Panencephalitis

Two patients presented with retinitis as the initial clinical manifestation of subacute sclerosing panencephalitis (SSPE), a delayed neurologic complication of measles. In one patient, the ocular involvement preceded the neurologic symptoms by 4 weeks and in the other patient by 4 years. The diagnosis of SSPE was suspected when neuropsychiatric manifestations appeared and was confirmed by the typical panencephalitic electroencephalography changes, neuroimaging features of panencephalitis, and high titers of measles antibodies in serum and cerebrospinal fluid. Although SSPE is an untreatable illness, recognition of this unusual presentation is valuable to allow earlier diagnosis and institution of palliative measures.

A case of subacute sclerosing panencephalitis presenting as depression.

Subacute sclerosing panencephalitis (SSPE) is rare in adult patients. Clinical presentation in intial phases of SSPE may be non-specific leading to diagnostic delay. We present a 24-year-old patient with depressive syndrome of five months' duration prior to the onset of typical features of SSPE, which is a rare presentation. This patient had responded partially to Sertraline, for a brief period, before he was diagnosed to have SSPE. This case illustrates affective symptoms can be the presenting features of SSPE in adults.

Changing Clinical Features of Subacute Sclerosing Panencephalitis (SSPE)

The early clinical features, age at onset, and relation to immunization of 32 patients (24 males, 8 females) with SSPE are reported from Grant Medical College, Mumbia, India.

Chorioretinitis as a possible very early manifestation of subacute sclerosing panencephalitis

Ocular changes occur in up to 50% of cases of subacute sclerosing panencephalitis (SSPE) usually at the same time as other neurological signs. Occasionally, visual symptoms precede other manifestations by a few weeks or months. Fundal changes (most commonly a macular chorioretinitis) are the most frequent ocular manifestation of SSPE. Inclusion bodies occur in the retina of patients with chorioretinitis and SSPE and it is believed that the lesions are due to actual viral invasion. We report a case of SSPE in which an atypical chorioretinitis preceded the onset of the typical clinical features of the disease by almost two years. Our case is unusual because of the long interval between the visual symptoms and the onset of other features of SSPE. We wish to emphasise the importance of thinking of SSPE in cases of chorioretinitis which are atypical or occur in young patients. Although treatment of SSPE thus far has been mostly disappointing, earlier diagnosis may offer the hope of better results.