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Subacute Sclerosing Panencephalitis


Presentation

  • An atypical presentation of subacute sclerosing panencephalitis can lead to a delay in diagnosis, thus hindering early treatment. Herein, we describe a 14-year-old girl who presented with insomnia, amnesia, auditory and visual hallucinations.[ncbi.nlm.nih.gov]
  • Subacute sclerosing panencephalitis (SSPE) typically presents with progressive mental deterioration, behavioral changes, and myoclonic jerks. Atypical presentations are not unknown and may result in diagnostic delays.[ncbi.nlm.nih.gov]
  • We report herein the case of a previously healthy 13-years-old girl with an unusual radiological presentation.[ncbi.nlm.nih.gov]
  • Visual complaints, if present, generally antedate the onset of neurological symptoms by a few weeks or months. Here, we report two cases of SSPE presenting with ocular findings and their prognoses. Case reports.[ncbi.nlm.nih.gov]
  • The present study retrospectively reviewed 56 cases history, neurologic symptoms, and clinical and laboratory data, as well as the outcomes.[ncbi.nlm.nih.gov]
Weakness
  • , such as those in the family… Read More disorder of nervous system In nervous system disease: Slow viruses Subacute sclerosing panencephalitis is characterized by the slowly increasing loss of mental abilities, brief, shocklike jerking of the body, weakness[britannica.com]
  • Over the next few days she started developing progressive unsteadiness of gait, weakness of both lower limbs followed by weakness of trunk and then both upper limbs. She went on to develop loss of bladder control, mutism and myoclonic jerks.[ijo.in]
  • If you develop SSPE, you may experience: gradual changes in your behavior unusual behavior a decrease in your cognitive and social abilities difficulty completing schoolwork or work dementia lethargy muscle spasms or jerking tense or lax muscles weakness[healthline.com]
  • A 25-year-old woman presented with a history of sudden visual loss in her right eye 5 years ago, intermittent jerking of the body for 10 months, insidious onset of weakness of the left upper limb 10 months back, weakness of the left lower limb 8 months[ijri.org]
  • There was no history of redness, lacrimation, diplopia, headache, vomiting, seizures, and motor weakness. After 7 days of onset of vision loss, she also developed abnormal behaviour in the form of excessive smile and emotional lability.[atmph.org]
Hyperthermia
  • In the final phases, hypothalamic involvement may cause intermittent hyperthermia, diaphoresis, and pulse and BP disturbances. Serologic testing EEG Neuroimaging SSPE is suspected in young patients with dementia and neuromuscular irritability.[merckmanuals.com]
  • The symptoms of SSPE can include the following: Coma Death Seizures Irritability Dementia Blindness Spasticity Memory loss Optic atrophy Hyperthermia Unsteady gait Abnormal EEG Myoclonic jerks Cortical blindness Brain inflammation Behavioral changes Very[disabled-world.com]
  • Over the two weeks prior to death he assumed a decebrate posture with preterminal hyperthermia. He died of cardiopulmonary arrest seven months following admission. Fig. 2.[healio.com]
  • The specific cause for his sudden deterioration was not clear with infection, malignant hyperthermia or dystonic spasm directly related to SSPE considered. He died within two weeks of re-admission and no autopsy was performed.[ncbi.nlm.nih.gov]
Tachycardia
  • She had frequent bouts of hyperpyrexia, tachycardia, hypertension, and irregular breathing. Electroencephalography was performed and revealed diffuse symmetrical slow wave activity.[doi.org]
Exanthema
  • , Intern Med, 2006 , vol. 45 (pg. 841 - 2 ) 11 Inflammatory/post-infectious encephalomyelitis, J Neurol Neurosurg Psychiatry, 2004 , vol. 75 Suppl. 1 (pg. i22 - 8 ) 12 Acute disseminated encephalomyelitis preceding measles exanthema, J Child Neurol, 2011[doi.org]
  • […] evidence that measles virus infection of brain occurs soon after the acute infection with subsequent spread throughout the brain. 18 Measles virus is thought to reach the brain through infection of cerebral endothelial cells, perhaps during the acute exanthema[doi.org]
Diplopia
  • There was no history of loss of consciousness, headache, vomiting, diplopia or any motor deficits. On examination, his vitals were stable. Nervous system examination revealed conscious, disoriented boy with impaired recent memory.[atmph.org]
Psychiatric Manifestation
  • Psychiatric manifestation of SSPE. J Neuropsychiatry Clin Neurosci 2006;18:560. [ PUBMED ] 3. Kartal A, Kurt AN, Gürkas E, Aydin K, Serdaroglu A. Subacute sclerosing panencephalitis presenting as schizophrenia with an alpha coma pattern in a child.[ijpm.info]
  • In the earlier stages of the disease, psychiatric manifestations, such as behavioral changes, mood swings, depression, and cognitive decline, are noted.[cureus.com]
  • Kayal M, Varghese ST, Balhara YP (2006) Psychiatric manifestation of SSPE. J Neuropsychiatry Clin Neurosci 18:560 PubMed Google Scholar 63.[link.springer.com]
Auditory Hallucination
  • hallucination) led to initial misdiagnosis of our case as possible schizophrenia.[ijpm.info]
Abnormal Behavior
  • Isolation Lymph node biopsies from 32-year-old male with visual loss, abnormal behavior, delusions, hallucinations, myoclonic seizu Product Format frozen Storage Conditions -60 C Comments HA produced in rhesus monkey RBC.[atcc.org]
Seizure
  • It occurred mainly in the first year, while in three cases seizures appeared between 1 and 5 years of the disease evolution. Myoclonus was present independently from seizures.[ncbi.nlm.nih.gov]
  • Atypical forms of presentation such as generalized seizures and acute or subacute coma can be observed rarely. This report describes a 5-year-old boy with an atypical feature of SSPE, generalized seizures.[ncbi.nlm.nih.gov]
  • Abstract Subacute sclerosing panencephalitis is a progressive disorder which also presents with various types of seizures, mainly myoclonic jerks, atonic attacks and tonic-clonic seizures.[ncbi.nlm.nih.gov]
  • During the terminal phase of the disease, no other type of seizures and movement disorders were recognized, except epilepsia partialis continua.[ncbi.nlm.nih.gov]
  • Patient 2 with infantile hemiparesis presented with secondary generalized seizures, followed by asymettrical myoclonias involving the side contralateral to the hemiparesis.[ncbi.nlm.nih.gov]
Myoclonic Jerking
  • For 1 month his only symptom was seizures, followed by personality and behavioral changes and myoclonic jerks.[ncbi.nlm.nih.gov]
  • Features like myoclonic jerks, cognitive decline and typical EEG findings lead to a strong suspicion of SSPE. Here, we describe the stagewise progression of a case of SSPE in a 14-year-old girl who had myoclonic jerks and cognitive decline at onset.[ncbi.nlm.nih.gov]
  • The authors report a case of 10 year-old boy, previously healthy with complete immunization, presenting with frequent myoclonic jerks, abnormal movements, spasticity and altered mental status.[ncbi.nlm.nih.gov]
  • It causes mental deterioration, myoclonic jerks, and seizures. Diagnosis involves EEG, CT or MRI, CSF examination, and measles serologic testing. Treatment is supportive.[merckmanuals.com]
  • Despite oral inosiplex and supportive care, patient developed generalized seizures with frequent myoclonic jerks and rapidly progressed into coma.[ncbi.nlm.nih.gov]
Irritability
  • The initial symptoms of SSPE are subtle and include mild mental deterioration (such as memory loss) and changes in behavior (such as irritability) followed by disturbances in motor function, including uncontrollable involuntary jerking movements of the[ninds.nih.gov]
  • Because these patients may be unable to express symptoms, increased intracranial pressure should be considered in the presence of irritability or frequent myoclonia.[ncbi.nlm.nih.gov]
  • Etiology SSPE is caused by a mutant measles virus that provokes persistent brain inflammation (swelling and irritation).[orpha.net]
  • Serologic testing EEG Neuroimaging SSPE is suspected in young patients with dementia and neuromuscular irritability. EEG, CT or MRI, CSF examination, and measles serologic testing are done.[merckmanuals.com]
Ataxia
  • A 9-year-old girl presented with poor balance and ataxia following an episode of upper respiratory tract infection. Neurological examination revealed mild hemiparesis and ataxia.[ncbi.nlm.nih.gov]
  • A 5-year-old boy presented with acute-onset cerebellar ataxia without associated encephalopathy, focal motor deficits, seizures or cognitive decline.[ncbi.nlm.nih.gov]
  • This study describes a 15-year-old boy with SSPE presenting with visual agnosia, prosopagnosia, simultanagnosia, optic ataxia, and oculomotor apraxia which are compatible with Balint syndrome.[ncbi.nlm.nih.gov]
  • Here it is presented a 7-years-old boy with involuntary movements in both hands, drop attacks while walking, ataxia and stupor.[ncbi.nlm.nih.gov]
  • Myoclonus, ataxia, autonomic dysfunction and dementia gradually develop. Stupor and coma precede the death.[orpha.net]
Mental Deterioration
  • Subacute sclerosing panencephalitis, a progressive neurodegenerative disorder of the central nervous system, can present atypically with uncharacteristic electroencephalographic (EEG) features at its onset albeit typically with progressive mental deterioration[ncbi.nlm.nih.gov]
  • Subacute sclerosing panencephalitis (SSPE) typically presents with progressive mental deterioration, behavioral changes, and myoclonic jerks. Atypical presentations are not unknown and may result in diagnostic delays.[ncbi.nlm.nih.gov]
  • The initial symptoms of SSPE are subtle and include mild mental deterioration (such as memory loss) and changes in behavior (such as irritability) followed by disturbances in motor function, including uncontrollable involuntary jerking movements of the[ninds.nih.gov]
  • Prominent findings at presentation were atonic/myoclonic seizures (57.9%) and mental deterioration with behaviour alteration (30.3%).[ncbi.nlm.nih.gov]
  • It causes mental deterioration, myoclonic jerks, and seizures. Diagnosis involves EEG, CT or MRI, CSF examination, and measles serologic testing. Treatment is supportive.[merckmanuals.com]

Workup

  • Extensive medical and laboratory workup was done to rule out other diseases.[ijri.org]
  • Laboratory workup did not reveal any abnormality in blood and urine. Cranial computed tomography was normal. A possibility of eclamptic encephalopathy was considered and she was treated accordingly.[doi.org]
EEG Periodic Complexes
  • Yakub demonstrated that video-split EEG monitoring is a more sensitive technique for early diagnosis and detection of atonia or myoclonus, 11 which are time related to EEG periodic complexes.[doi.org]
Generalized Periodic Patterns
  • Hallmarks of this entity include progressive cognitive decline, myoclonia, a generalized periodic pattern on EEG and deep white matter abnormalities on MRI. However, imaging can be normal in early stages.[ncbi.nlm.nih.gov]
White Matter Lesions
  • CT or MRI may show cortical atrophy or white matter lesions. CSF examination usually reveals normal pressure, cell count, and total protein content; however, CSF globulin is almost always elevated, constituting up to 20 to 60% of CSF protein.[merckmanuals.com]
  • MRI can reveal multifocal white-matter lesions. Treatment The course of SSPE is variable, but it can last for years. There is no definitive treatment. Long-term seizure management is often required. 96, 193 Adapted from: Goldstein MA and Harden CL.[epilepsy.com]
  • Lab tests showing high Rubeola Ig G Antibody Titres in serum, elevated measles antibody titers in the CSF, brain CT or MRI scan may show cortical atrophy and white matter lesions.[secure.ssa.gov]
  • Brain MRI demonstrated periventricular white matter lesions. EEG reported typical findings suggestive of SSPE (i.e., high-voltage slow-wave complexes interspersed with short rhythmic waves) [Figure 1].[ijpm.info]

Treatment

  • In spite of antipsychiatric treatment (olanzapine 20 mg/d) for 3 months, a progressive deterioration in neurologic function was observed.[ncbi.nlm.nih.gov]
  • No effective treatment has a proven role; oral isoprinosine and intrathecal administration of alpha-interferon may prolong survival.[ncbi.nlm.nih.gov]
  • Treatment for SSPE is still undetermined. A combination of oral isoprinosine (Inosiplex) and intraventricular interferon alfa appears to be the best effective treatment. Patients responding to treatment need to receive it life long.[doi.org]
  • It is caused by a persistent measles virus infection of the brain without any available treatment to date. The physiopathology of the disease is largely unknown.[ncbi.nlm.nih.gov]
  • Subacute sclerosing panencephalitis: Diagnosis and drug treatment options.[ncbi.nlm.nih.gov]

Prognosis

  • Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disease of the central nervous system with poor prognosis and high mortality.[ncbi.nlm.nih.gov]
  • Subacute sclerosing panencephalitis (SSPE) is a progressive neurodegenerative disease with high mortality and poor prognosis. This is caused by persistent defective measles virus infection.[ncbi.nlm.nih.gov]
  • Prevention, in the form of measles vaccination, is the only real "cure" for SSPE. x Prognosis Most individuals with SSPE will die within 1 to 3 years of diagnosis.[ninds.nih.gov]
  • Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disorder of the central nervous system with both poor prognosis and high mortality.[ncbi.nlm.nih.gov]
  • Subacute sclerosing panencephalitis is a neurodegenerative disease with a poor prognosis. We report a case of a 5 1/2-year-old boy who had emotional lability, cognitive difficulties, and myoclonia after a mild closed head injury.[ncbi.nlm.nih.gov]

Etiology

  • […] in serum and cerebrospinal fluid, ultrastructural observation of intranuclear and intracytoplasmic inclusions in neurons and neuroglia in three patients, and alteration of serum and CSF immunoglobulins indicate that the measles myxovirus may be the etiological[jamanetwork.com]
  • Etiology SSPE is caused by a mutant measles virus that provokes persistent brain inflammation (swelling and irritation).[orpha.net]
  • The pronounced and prolonged elevation of antimeasles antibodies suggests a possible etiologic relation between the virus and this patient's illness and is of special interest in the light of the occurrence of slow-virus infections in the nervous systems[nejm.org]

Epidemiology

  • Abstract Investigators conducted a retrospective epidemiological study of subacute sclerosing panencephalitis, a fatal disease caused by measles infection, over the past few years in Japan.[ncbi.nlm.nih.gov]
  • In this Grand Round, we review the clinical features and treatment of subacute sclerosing panencephalitis, and the epidemiological and public health aspects of the case.[ncbi.nlm.nih.gov]
  • Molecular epidemiology of measles virus in Japan, Pediatr Int, 2004 , vol. 46 (pg. 214 - 23 ) 38.[doi.org]
  • The current epidemiological situation is that autochthonous SSPE will disappear in the United States, assuming measles vaccination rates remain high. Copyright 2018 Elsevier Ltd. All rights reserved.[ncbi.nlm.nih.gov]
  • Extract from report of GACVS meeting of 1-2 December 2005, published in the WHO Weekly epidemiological Record on 13 January 2006 The Committee reviewed the epidemiology of subacute sclerosing panencephalitis (SSPE) and the purported relationship between[who.int]
Sex distribution
Age distribution

Pathophysiology

  • MR spectroscopy (MRS) may illustrate the pathophysiological features of SSPE. To the best of our knowledge, this is the second report of MRS findings of corpus callosum involvement in a stage 3 SSPE case.[ncbi.nlm.nih.gov]
  • This review examines the pathophysiology of measles infection and the presentations, diagnosis and treatment of the four types of measles-induced encephalitis including primary measles encephalitis, acute post-measles encephalitis, measles inclusion body[ncbi.nlm.nih.gov]
  • For the interested reader a discussion of the pathophysiology of this unusual infection is dealt with in several important references but will not be discussed in this article (3–8).[ncbi.nlm.nih.gov]
  • (B) Interferon alfa The pathophysiology of natural remissions and relapses in SSPE is unknown.[doi.org]

Prevention

  • The success of global programs to eliminate measles will not only prevent the severe complications and death associated with acute cases of measles but will also prevent the devastating disease SSPE Acknowledgments We thank Ms.[doi.org]
  • Prevention, in the form of measles vaccination, is the only real "cure" for SSPE.[ninds.nih.gov]
  • Physicians taking care of children need to be aware of atypical presentations of subacute sclerosing panencephalitis and must have a high index of suspicion to prevent diagnostic delays and avoid unnecessary diagnostic and therapeutic interventions.[ncbi.nlm.nih.gov]
  • Electronic address: lpalliva@uci.edu. 2 Department of Population Health and Disease Prevention, University of California, 653 E. Peltason Drive, Irvine, CA 92697, United States. Electronic address: noymer@uci.edu.[ncbi.nlm.nih.gov]
  • Measles and SSPE are preventable by maintenance of high rates of immunization in the population. Copyright 2013 Elsevier B.V. All rights reserved.[ncbi.nlm.nih.gov]

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