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]

• Epilepsy

  Intractable epilepsy developed in four patients. CONCLUSION: Atypical SSPE presentation can include mainly focal intractable seizures. Epilepsy developed during clinical course in 52.6% cases. [ncbi.nlm.nih.gov]

  Managing epilepsy and co-existing disorders. Boston: Butterworth-Heinemann; 2002;83-133. With permission from Elsevier ( www.elsevier.com ). [epilepsy.com]

  […] diagnosis A wide variety of neurological conditions should be considered in the differential diagnosis of SSPE: Creutzfeldt-Jacob disease, anoxic encephalopathy, metabolic encephalopathy, hepatic failure, drug toxicity, thyrotoxicosis, progressive myoclonic epilepsy [orpha.net]

• 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]

  Although the cause of death is usually pneumonia, the pneumonia results from the extreme weakness and abnormal muscle control caused by the disease. [msdmanuals.com]

  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]

  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]

• Hyperthermia

  In the final phases, hypothalamic involvement may cause intermittent hyperthermia, diaphoresis, and pulse and blood pressure disturbances. [msdmanuals.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]

• Collapse

  At this stage, patients frequently die due to hyperpyrexia, cardiovascular collapse, or hypothalamic disturbances. 51 DIAGNOSIS Once myoclonus is evident the clinical diagnosis is seldom a problem. [doi.org]

• Anemia

  Ribavirin was well tolerated except for mild hemolytic anemia in patient #1. CSF ribavirin levels were 75% of serum at 7 days, and remained above 66% for 6 weeks. [nature.com]

• Fecal Incontinence

  The patient presented with an altered mental status, generalized muscle rigidity, and episodes of urinary and fecal incontinence that started two days earlier. [cureus.com]

• Muscle Rigidity

  The patient presented with an altered mental status, generalized muscle rigidity, and episodes of urinary and fecal incontinence that started two days earlier. [cureus.com]

• Tachycardia

  She had frequent bouts of hyperpyrexia, tachycardia, hypertension, and irregular breathing. Electroencephalography was performed and revealed diffuse symmetrical slow wave activity. [doi.org]

• Muscle Spasm

  Stage II: There may be uncontrolled movement problems including jerking and muscle spasms. Other symptoms that may occur in this stage are loss of vision, dementia, and seizures. [nlm.nih.gov]

  Stage 2: This stage may involve jerking, muscle spasms, seizures, loss of vision, and dementia. Stage 3: Jerking movements are replaced by writhing (twisting) movements and rigidity. At this stage complications may result in death. [en.wikipedia.org]

  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]

• 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

  and possible auditory 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

  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]

  Definition of subacute sclerosing pan en ceph a li tis : a usually fatal neurological disease of children and young adults caused by infection of the brain by a previously latent measles virus that is marked especially by behavioral changes, myoclonic seizures [merriam-webster.com]

• Myoclonus

  We suggest that the generalized periodic discharges associated with the myoclonus contributed to the patient's encephalopathy. [ncbi.nlm.nih.gov]

  On subsequent follow up, the child still has myoclonus and he was treated with carbamazepine. 4. [pubs.sciepub.com]

• 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]

  This response leads to brain inflammation (swelling and irritation) that may last for years. SSPE has been reported in all parts of the world, but in western countries it is a rare disease. [nlm.nih.gov]

• 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]

  It is characterized by diffuse inflammation of brain tissue, personality change, seizures, ataxia myoclonus, visual disturbances, dementia, fever, and death. Live measles virus can be cultured from brain tissue. [medical-dictionary.thefreedictionary.com]

• 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]

  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]

  Subacute sclerosing panencephalitis, a progressive and usually fatal brain disorder, is a rare complication of measles that appears months or years later and causes mental deterioration, muscle jerks, and seizures. [msdmanuals.com]

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

  Figure 1 EEG showing a periodic pattern with slow wave complexes recurring at intervals of 4–6 seconds. [doi.org]

• Gliosis

  Brain tissue biopsy showed histologic evidence of encephalitis with eosinophilic intranuclear inclusion bodies (Cowdry Type A and B), intracytoplasmic inclusion bodies, perivascular lymphoplasmacytic infiltration and gliosis. [ncbi.nlm.nih.gov]

  Histopathological findings in brain biopsy or autopsy include perivascular cuffing, eosinophilic cytoplasmic inclusions, neurophagia, and fibrous gliosis. [orpha.net]

  MR spectroscopy may demonstrate 1,3 decreased NAA: from neuronal loss increases in choline: from demyelination increase myo -inositol: from active gliosis elevated lactate: from macrophagic infiltration [radiopaedia.org]

  Depending on duration of the process the active inflammation and/ or chronic destructive- reparative changes can be seen as diffuse demyelination, intense fibrillary gliosis, neuronal loss and brain atrophy. [path.upmc.edu]

• White Matter Lesions

  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]

  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. [msdmanuals.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]

In spite of antipsychiatric treatment (olanzapine 20 mg/d) for 3 months, a progressive deterioration in neurologic function was observed. [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]

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disease of the central nervous system with poor prognosis and high mortality. [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]

[…] 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]

We have added two cases to the existing literature on adult‐onset SSPE, compared them with a more classic juvenile presentation, and extensively reviewed those reports that were published after the etiological link with the measles virus had been established [onlinelibrary.wiley.com]

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]

We conducted a comprehensive review of the impact of measles immunization on the epidemiology of SSPE and examined epidemiological evidence on whether there was any vaccine-associated risk. [ncbi.nlm.nih.gov]

Molecular epidemiology of measles virus in Japan, Pediatr Int, 2004, vol. 46 (pg. 214 - 23 ) 38. [doi.org]

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]

[…] courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology Subacute sclerosing panencephalitis Microchapters Home Patient Information Overview Historical Perspective Pathophysiology [wikidoc.org]

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 [doi.org]

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]