Subacute sclerosing panencephalitis: case based review

Subacute sclerosing panencephalitis (SSPE) is a chronic debilitating condition that occurs in children affected with measles. SSPE is broadly distinguished as typical SSPE, the more rampant form, occurring over a period of years following primary measles infection, while the atypical has a more rapidly progressive course over weeks to months. SSPE can present with cognitive, epileptic, autonomic, pyramidal and ophthalmologic manifestations with scholastic decline being the primary feature. The management of SSPE focuses on improvement of quality of life and prolongation of survival which can be achieved with the use of supportive care modalities and immunomodulators respectively. This is a comprehensive review which discusses several parameters of SSPE such as epidemiology, pathophysiology, clinical presentations, and detailed management protocol for this condition. As part of this review, we also discuss a case of rapidly progressive, fulminant and atypical SSPE in a five-year-old male presenting clinically with myoclonic jerks of lower extremities.

Movement Disorder Early in the Presentation of Two Children with Subacute Sclerosing Pan-Encephalitis

Subacute sclerosing panencephalitis(SSPE) is a progressive degenerative disease caused by measles infection with overall poor prognosis in spite multiple modalities of treatment. The course of the disease is characterized by progressive neurological decline in the form of behavioral and personality changes followed by a stage of characteristic periodic myoclonic spasms followed by a stage of quadriplegia movement disorder, vegetative state and frequently early death. Here we report two cases with atypical presentation of early rather than late movement disorder during illness and the unusual association of central precocious puberty preceding the course of illness in one of the cases

A long-term subacute sclerosing panencephalitis survivor treated with intraventricular interferon-alpha for 13 years / 소아과

Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, and fatal central nervous system disorder resulting from persistent measles virus infection. Long-term data are scarce, with a maximum follow-up period of 10 years. Interferon-alpha (IFN-α) is a protein that exerts its antiviral activity via enhancement of cellular immune response and is reported to be an effective drug for the treatment of SSPE. However, there is currently no consensus regarding the optimal duration of IFN-α therapy. Here, we present a case report of a patient with SSPE treated with long-term intraventricular IFN-α therapy, which facilitated clinical improvement and neurological stabilization without causing serious adverse effects. To the best of our knowledge, this is one of the longest follow-up studies investigating a patient with SSPE receiving intraventricular INF-α treatment. Further studies are necessary to validate the benefits and safety of long-term intraventricular IFN-α treatment in patients with SSPE.

Retinitis as the presenting feature of subacute sclerosing panencephalitis in an Indian male: A case report

Measles virus is a rare but important cause for acute retinitis as it can eventually lead to the fulminant complication of SSPE. We report a case of a young Indian male with acute viral retinitis who subsequently developed SSPE. It is of paramount importance to consider measles virus and SSPE as a cause in an immunocompetent young adult with necrotizing viral retinitis

Subacute sclerosing panencephalitis resembling Rasmussen's encephalitis on magnetic resonance imaging

Subacute sclerosing panencephalitis (SSPE) is a rare, slowly progressing but invariably fatal disease that is related to a prior measles virus infection and most commonly affects paediatric patients. Magnetic resonance (MR) imaging is the modality of choice for determining such changes in white matter. SSPE typically demonstrates bilateral but asymmetric periventricular and subcortical white matter involvement. We herein report a rare case of unilateral white matter involvement in a 13-year-old boy with SSPE that closely simulated Rasmussen's encephalitis. To the best of our knowledge, this is the first report of an atypical presentation on MR imaging in which SSPE was a rare cause of unilateral brain parenchymal involvement in a patient with intractable seizures.

Subacute Sclerosing Panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a rare, invariably fatal degenerative disease of central nervous system developing after measles infection. Neurological symptoms are initial presenting symtoms but rere reports of pure psychiatric symtoms preceding neurological symtoms have been reported.. We here report a case of 18 years old male patient suffering from Subacute sclerosing panencephalitis (SSPE) who initially had behavioural changes and then subseqently devloped neurological symptoms.

Atypical Subacute Sclerosing Panencephalitis With Short Onset Latency.

An 11-month-old boy presented with focal seizures, myoclonic jerks and altered sensorium of one month duration, with a history of measles at eight months of age. A diagnosis of Subacute sclerosing panencephalitis (SSPE) was made on the basis of typical EEG changes and presence of anti-measles antibody in cerebrospinal fluid. A differential diagnosis of SSPE should be considered in all forms of acute encephalopathy in infants for early diagnosis and management.

Measles Viral Infection in PD-1 Gene Knockout Mice / 소아감염

PURPOSE: Subacute sclerosing panencephalitis (SSPE) is a neurodegerative disease due to persistent measles virus infection. We investigated the role of programmed death-1 (PD-1) molecule which is related with chronic viral infection in developing SSPE in mouse. METHODS: We adopt the PD-1-/-, PD-1-/+, and wild type BALB/c 3 week old mice to make an animal model of SSPE by injecting measles virus (SSPE strain) intraventricularly. Three months after infusion of virus, the mice were sacrificed and examined if the typical pathologic lesions had been progressed. The sera were collected from each group of mice and the serum level of IL-21 was measured with ELISA kit. RESULTS: The necrotic lesions on white matter and gliosis were found in focal areas in wild type BALB/c. The extent of lesion was smaller in heterotype BALB/c. Scanty lesions were found in PD-1-/- mice. The sera level of IL-21 was not elevated in all three groups. CONCLUSION: Our data suggest that the PD-1 molecule may play a role in persistent viral infection.

Basal ganglia lesions in subacute sclerosing panencephalitis / Lesões nos gângliios da base em panencefalite esclerosante subaguda

The parieto-occipital region of the brain is the most frequently and severely affected in subacute sclerosing panencephalitis (SSPE). The basal ganglia, cerebellum and corpus callosum are less commonly involved. We describe apatient with SSPE confirmed by neuropathology based on brain magnetic resonance imaging showing extensive basal ganglia involvement and no significant involvement of other cortical structures. Though rarely described in SSPE, clinicians should be aware of this involvement. SSPE should be kept in mind when changes in basal ganglia signal are seen on brain magnetic resonance imaging with or without involvement of other regions of the human brain to avoid erroneous etiological diagnosis of other pathologies causing rapidly progressive dementia.

A região parietooccipital é mais frequente e gravemente acometida na panencefalite esclerosante subaguda(PEESA). Os gânglios da base, cerebelo e corpo caloso são menos envolvidos. Descrevemos um paciente com PEESA confirmada por neuropatologia com imagens de ressonância magnética (RNM) evidenciando acometimento extenso dosgânglios da base sem envolvimento de outras estruturas corticais. Embora raramente descritas nesta doença, deve-se ficar atento para tal acometimento e PEESA deve ser lembrada quando alterações de sinal nos gânglios da base são vistas naRNM com ou sem acometimento de outras regiões do cérebro a fim de evitar outros diagnósticos etiológicos errôneos de patologias que cursam com demência rapidamente progressiva.

Acute bilateral vision loss as the presenting feature of subacute sclerosing panencephalitis.

A 12-yr-old boy with an atypical presentation of subacute sclerosing panencephalitis (SSPE) is described. Bilateral macular chorioretinitis preceded the neurological symptoms by 3 weeks. Both visual and neurological features had an acute onset. Clinicians need to be aware that macular chorioretinitis in a child may be the heralding feature of SSPE.

Atypical subacute sclerosing panencephalitis: case report

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disorder of the central nervous system with both poor prognosis and high mortality. The disease has been related to a persistent and aberrant measles virus infection and no effective treatment has been available. We report a case of SSPE with atypical features including seizures at onset and a fulminant course in a 8 years-old boy who had been previously immunized against measles.

Panencefalite esclerosante subaguda (PES) é uma doença inflamatória e progressiva do sistema nervoso central com prognóstico reservado e alta mortalidade. A doença tem sido relacionada com a infecção persistente e anormal pelo vírus do sarampo e não há tratamento específico disponível. Relatamos um caso de PES com características atípicas representadas por início do quadro com crises convulsivas e apresentação fulminante em menino de 8 anos previamente imunizado contra o vírus do sarampo.

Subacute Sclerosing Panencephalitis: Clinical Experience of 6 Cases

PURPOSE: Subacute sclerosing panencephalitis(SSPE) is a severe and usually fatal neurodegenerative disorder of childhood and adolescence. The etiology is related to previous measles infection especially during the first 2 years of life. Since recent measles epidemics in Korea may increase the late risk of SSPE, the authors investigated the clinical characteristics of SSPE focusing on brain MRI. METHODS: Six cases(4 males, 2 females) of SSPE patients were retrospectively reviewed for clinical, EEG, laboratory and brain MRI findings. RESULTS: Four of 6 had a history of measles infection in the first year of life. Clinical manifestations were as follows:myoclonus(6), falling(4), ataxia(4), dysarthria(3), seizures (2), involuntary movements(2), tremor(2), head drop(1), sleep disturbance(1). In all cases, CSF IgG, CSF IgG/albumin ratio, and CSF/serum IgG index increased, oligoclonal bands were positive, and CSF antimeasles antibodies were positive. Frontal high amplitude sigma activities and anteriorly-accentuated multifocal epileptiform discharges were noted on EEG. Brain MRI revealed T2-weighted high signal intensity of the deep white matter. CONCLUSION: The diagnosis of SSPE depends on characteristic clinical features and elevation of measles antibodies in CSF, supported by others including EEG, CSF and brain MRI findings. We hope the clinical characteristics we mentioned may be useful for the early diagnosis and active management of SSPE in Korea.

A Case of Subacute Sclerosing Panencephalitis

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disease of the central nervous system (CNS) caused by a persistent, aberrant measles virus infection. The outcome is usually fatal. After a latent period of 6 to 7 years, there is subtle and slow cognitive decline and visuospatial disorientation develops followed by myoclonic jerks, extrapyramidal symptoms, ataxia, and seizures, progressing to coma or vegetative state. The diagnosis is based on at least three of the following criteria 1) clinical manifestations 2) abnormal EEG 3) hyperglobulinorrachia, elevated serum or spinal fluid measles antibody 4) histologic features. No therapeutic maneuver has been proven conclusively to be of value. We have diagnosed and experienced a case of subacute sclerosing panencephalitis (SSPE) in a 5-year-old child with the chief complaint of myoclonic seizure and mental deterioration. We report a case and the brief review of related literature.