Related Experiment Video
Updated: Jun 30, 2026
06:24
Mouse Models of Periventricular Leukomalacia
Published on: May 18, 2010
Reversible extensive leukoencephalopathy in Sweet disease: a case report
Kazuhiro Fukushima1, Akiyo Hineno, Minori Kodaira
1Department of Internal Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan. fuku@hsp.md.shinshu-u.ac.jp
Journal of the Neurological Sciences
|September 19, 2008
Summary
Sweet disease, a rare inflammatory condition, can affect the central nervous system, causing acute encephalopathy. This case highlights reversible leukoencephalopathy in neuro-Sweet disease, responding well to steroid therapy.
Area of Science:
- Neurology
- Dermatology
- Immunology
Background:
- Sweet disease (acute febrile neutrophilic dermatosis) is an idiopathic multisystem inflammatory disorder.
- Neuro-Sweet disease is a rare manifestation involving the central nervous system alongside characteristic skin lesions.
Observation:
- A case of Sweet disease complicated by acute encephalopathy is presented.
- The patient exhibited extensive cerebral white matter lesions on MRI and elevated CSF protein without pleocytosis.
Findings:
- The leukoencephalopathy observed was primarily attributed to reversible edema, not destructive parenchymal changes.
- Complete clinical recovery and normalization of MRI and CSF findings were achieved after steroid therapy.
Implications:
- This case underscores the potential for reversible neurological involvement in neuro-Sweet disease.
- Prompt steroid treatment may prevent long-term neurological deficits in affected patients.
Related Concept Videos
01:19Encephalitis 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...
01:29Hepatic Encephalopathy
DefinitionHepatic encephalopathy is a reversible neurologic syndrome that results from advanced liver dysfunction or portosystemic shunting. It leads to disturbances in cognition, behavior, and motor function due to the brain’s exposure to gut-derived toxins that the liver fails to detoxify.EtiologyThis condition develops either in the setting of acute fulminant hepatitis or progressively during chronic liver disease, such as cirrhosis and portal hypertension. Portosystemic shunting—including...
01:26Encephalitis 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...
01:22Cerebral Edema ll: Pathophysiology
Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this barrier loses...
01:28Rabies
Rabies is a lethal zoonotic disease caused by a single-stranded, negative-sense RNA virus of the Lyssavirus genus, within the family Rhabdoviridae. Its primary mode of transmission to humans is through bites or saliva-contaminated scratches from infected mammals such as dogs, bats, raccoons, or foxes. Transmission can also occur if infectious saliva contacts abraded skin or intact mucous membranes, including the conjunctiva.Viral Entry and Early ReplicationOnce introduced at the bite or scratch...
01:19Cerebral Edema l: Introduction
Cerebral edema is a pathological increase in brain water content that disrupts intracranial pressure regulation and impairs neurological function. Because the cranial vault is rigid, even modest increases in tissue volume can compromise cerebral perfusion, distort neural structures, and initiate secondary injury. Cerebral edema develops through four principal mechanisms: vasogenic, cytotoxic, interstitial, and ionic.Vasogenic EdemaVasogenic edema arises from disruption of the blood–brain...