Related Experiment Video

Updated: May 31, 2026

Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
26:48

Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis

Published on: July 4, 2007

Fulminant adult-onset subacute sclerosing panencephalitis: a case report

Anthony Faivre1, Jean-Baptiste Souraud, Aileen McGonigal

  • 1HIA Sainte-Anne, Service de Neurologie, Boulevard St-Anne, Toulon 83000, France.

BMJ Case Reports
|June 21, 2011
PubMed

Insights

This case highlights acute subacute sclerosing panencephalitis (SSPE) presenting as severe status epilepticus in an adult. Early diagnosis is crucial, even in developed nations, for this rare and aggressive neurological condition.

Area of Science:

  • Neurology
  • Infectious Diseases
  • Pathology

Background:

  • Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological disorder.
  • Acute SSPE is an exceptionally rare variant with diverse and atypical presentations.
  • Unexplained acute encephalopathies require a broad differential diagnosis.

Purpose of the Study:

  • To report a unique case of adult-onset acute SSPE presenting with status epilepticus.
  • To emphasize the diagnostic challenges posed by atypical SSPE presentations.
  • To highlight the importance of considering SSPE in unexplained encephalopathies.

Main Methods:

  • Clinical case presentation of a young adult with rapid neurological decline.
  • Cerebrospinal fluid (CSF) analysis including IgG synthesis and measles antibodies.
  • Magnetic Resonance Imaging (MRI) and Electroencephalography (EEG) findings.
  • Brain biopsy for definitive histopathological confirmation.

Main Results:

  • The patient presented with acute encephalopathy and severe status epilepticus.
  • CSF revealed markedly increased intrathecal IgG synthesis and high antimeasles antibodies.
  • MRI and EEG findings were atypical for classic SSPE.
  • Brain biopsy confirmed SSPE diagnosis.
  • The patient deteriorated rapidly, leading to death within 6 weeks.

Conclusions:

  • This case represents the first reported instance of SSPE presenting with status epilepticus in adults.
  • Acute SSPE can manifest with protean and atypical neurological symptoms.
  • SSPE should be considered in the differential diagnosis of unexplained acute encephalopathies, even in developed countries.

More Related Videos

A Metadata Extraction Approach for Clinical Case Reports to Enable Advanced Understanding of Biomedical Concepts07:50

A Metadata Extraction Approach for Clinical Case Reports to Enable Advanced Understanding of Biomedical Concepts

Published on: September 20, 2018

Sub-acute Cerebral Microhemorrhages Induced by Lipopolysaccharide Injection in Rats06:39

Sub-acute Cerebral Microhemorrhages Induced by Lipopolysaccharide Injection in Rats

Published on: October 17, 2018

Related Concept Videos

Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
Brain Abscess l: Introduction01:26

Brain Abscess l: Introduction

A brain abscess is a focal, intracerebral infection characterized by a localized collection of pus within the brain parenchyma, resulting from microbial invasion and the body’s inflammatory response. It progresses through stages: early and late cerebritis, followed by early and late capsule formation, reflecting tissue destruction, immune response, and eventual encapsulation.Etiology and PathogenesisCausative organisms vary with source and host factors, often involving polymicrobial infections,...
Bacterial Meningitis I: Introduction01:22

Bacterial Meningitis I: Introduction

Bacterial meningitis is a severe, life-threatening inflammation of the meninges, particularly the pia mater and arachnoid mater, affecting the subarachnoid space, ventricles, and cerebrospinal fluid (CSF). If untreated, it can lead to significant neurological complications or death.Causative AgentsCommon pathogens vary with age and immune status. In adults, major organisms include Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae. Streptococcus agalactiae (group B...
Multiple Sclerosis l: Introduction01:19

Multiple Sclerosis l: Introduction

Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
Rocky Mountain Spotted Fever01:26

Rocky Mountain Spotted Fever

Rocky Mountain Spotted Fever (RMSF) is a severe tick-borne illness caused by Rickettsia rickettsii, a Gram-negative, coccobacillary bacterium. This pathogen is an obligate intracellular parasite, requiring a host cell for replication. Transmission occurs through the bite of an infected tick. In the United States, the most important vectors are Dermacentor variabilis (American dog tick) and Dermacentor andersoni (Rocky Mountain wood tick), though other tick species may also serve as vectors.