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]

• 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]

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

• Hyperthermia

  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]

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

• 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 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]

  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]

  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]

• 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]

• Auditory Hallucination

  hallucination) led to initial misdiagnosis of our case as possible schizophrenia. [ijpm.info]

• 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]

• Myoclonus

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

• Irritability

  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]

  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]

  Etiology SSPE is caused by a mutant measles virus that provokes persistent brain inflammation (swelling and irritation). [orpha.net]

  According to the Merck Manual: "SSPE is suspected in young patients with dementia and neuromuscular irritability. EEG, CT or MRI, CSF examination, and measles serologic testing are done. [en.wikipedia.org]

• 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]

  It causes mental deterioration, myoclonic jerks, and seizures. Diagnosis involves EEG, CT or MRI, CSF examination, and measles serologic testing. Treatment is supportive. [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

  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. [en.wikipedia.org]

  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]

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]

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]

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]

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

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]

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 [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]

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]

Prevention, in the form of measles vaccination, is the only real "cure" for SSPE. [ninds.nih.gov]