Anti-N-Methyl-D-Aspartate Receptor Encephalitis with Favorable Outcome Despite Prolonged Status Epilepticus

Background

To describe a case of auto-immune encephalitis in an adolescent with favorable outcome despite prolonged status epilepticus.

Methods

A 17 year old Asian man without previous medical history developed alteration of consciousness and partial seizures. The diagnosis of anti-N-methyl-D-aspartate receptor encephalitis was confirmed by the detection of specific antibodies in both cerebrospinal fluid and serum.

Results

The clinical course was complicated by prolonged status epilepticus which was refractory to a large number of antiepileptic drugs, including barbiturate coma. Immunomodulatory therapy included steroids, plasma exchanges, and intravenous immunoglobulins. After 86 days of intensive therapy, the patient regained consciousness progressively. Brain magnetic resonance imaging never demonstrated any lesion. Extensive search for a tumor was negative. At 12 month follow-up, the patient had made an excellent recovery.

Conclusion

Auto-immune encephalitis is likely underdiagnosed in adolescents. In their most severe presentation, seizures may be resistant to a large number of anti-epileptic drugs, and the clinical improvement seems to be mainly because of the immunomodulatory therapy. Relapse is possible, as well as the delayed development of a teratoma or other tumor.     

Anti-N-Methyl-D-Aspartate Receptor Encephalitis in Korea: Clinical Features,Treatment, and Outcome

Background and Purpose

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common type of autoimmune synaptic encephalitis and it often responds to treatment. We analyzed the clinical characteristics of anti-NMDAR encephalitis in Korea.

Methods

Serum and/or cerebrospinal fluid (CSF) of adult patients (aged ≥18 years) with encephalitis of undetermined cause were screened for anti-NMDAR antibodies using a cell-based indirect immunofluorescence assay. The patients came from 41 university hospitals.

Results

Of the 721 patients screened, 40 were identified with anti-NMDAR antibodies and clinical details of 32 patients were obtained (median age, 41.5 years; 15 females). Twenty-two patients (68.8%) presented with psychiatric symptoms, 16 (50%) with seizures, 13 (40.6%) with movement disorders, 15 (46.9%) with dysautonomia, 11 (34.4%) with memory disturbance, and 11 (34.4%) with speech disturbance. Magnetic resonance imaging, electroencephalography, and CSF examinations yielded nonspecific findings. Tumor information was only available for 22 patients: 5 patients had tumors, and 2 of these patients had ovarian teratomas. Twenty-two patients received immunotherapy and/or surgery, and therapeutic responses were analyzed in 21 patients, of which 14 (66.7%) achieved favorable functional outcomes (score on the modified Rankin Scale of 0-2).

Conclusions

This study investigated the clinical characteristics of adult anti-NMDAR encephalitis in Korea. Currently, elderly patients who do not have tumors are commonly diagnosed with this condition. Understanding the detailed clinical characteristics of this disease will improve the early detection of anti-NMDAR encephalitis in patients both young and old.     

Antiglutamic Acid Decarboxylase Antibodies in Children With Encephalitis and Status Epilepticus

Encephalitis may present with seizures or status epilepticus, and the etiology is usually presumed. Specific antibodies to ion channels, receptors, and other synaptic proteins were identified during the past decade. However, only a few studies investigated antiglutamic acid decarboxylase antibodies and antibodies to cell membrane ion channels or surface antigens in pediatric encephalitis and status epilepticus. We examined antibodies to glutamic acid decarboxylase and cell membrane ion channels or surface antigens in acute-phase serum from 17 children with encephalitis and status epilepticus. Antiglutamic acid decarboxylase antibody titers were compared with those of control children manifesting therapy-resistant epilepsy. Antiglutamic acid decarboxylase antibody titers were significantly higher in those with encephalitis and status epilepticus than in those with therapy-resistant epilepsy. No patient demonstrated antibodies to cell membrane ion channels or surface antigens. Six children exhibited positive antiglutamic acid decarboxylase antibodies. One child died, three manifested postencephalitic epilepsy with neurologic deficits, and two recovered well. Higher antiglutamic acid decarboxylase antibody titers were evident in the children with encephalitis and status epilepticus. Clinicians should be aware of the potential role of antiglutamic acid decarboxylase antibodies in children with encephalitis and status epilepticus.     

Chorea and Developmental Regression Associated With Human Herpes Virus-6 Encephalitis

We report a 14-month old child with multiple episodes of febrile status epilepticus, followed by chorea and developmental regression, caused by human herpes virus–6 encephalitis. Chorea has been described as a complication of relapsing herpes simplex virus I infection, but not as a manifestation of human herpes virus–6 infection. It is uncertain whether the chorea was an autoimmune phenomenon or a direct effect of the virus. The child was treated with levetiracetam, intravenous immunoglobulin, and foscarnet. The seizures and chorea resolved with treatment, but developmental regression, with loss of language skills, persisted 6 months after the illness. This child illustrates a new clinical presentation of human herpes virus–6 encephalitis, adds to the spectrum of disorders caused by this virus, and strengthens the case for routine identification of specific viral agents in all cases of childhood viral infections with central nervous system symptoms to determine optimal treatment and prognosis.     

Young girl with abnormal behavior: Anti-N-Methyl-D-Aspartate receptor immune encephalitis

Anti N Methyl D Aspartate receptor immune encephalitis (Anti NMDARE) is a recently defined, under-recognized and often misdiagnosed disease, which typically occurs in young females and may be associated with an underlying tumor, usually ovarian teratoma. If diagnosed early, initiation of immunotherapy and tumor removal (if present) may result in recovery. We report a case of a 17 years old girl with Anti NMDARE who was initially misdiagnosed as Functional psychosis, Neuroleptic Malignant Syndrome and Sepsis syndrome. To the best of our knowledge, this is only the second case of anti NMDARE being reported from India. This case report underscores the need for a greater awareness of this entity across multiple specialties, e.g., general medicine, psychiatry and neurology, to ensure a heightened diagnostic suspicion, which can lead to timely diagnosis and adequate therapy of this treatable disease.     

An Autopsy Case of Protracted Necrotic Encephalitis With Marked Atrophy of Unilateral Temporal Lobe

Clinical and neuropathological findings in a case of an unusually protracted encephalitis have been described. The patient was a 44-year-old man who experienced mental deterioration, right abducens and facial paresis, right and, subsequently, left hemiparesis, and consciousness disturbance with an intermittent low grade fever and occasional headache during one year and three months. Electroenphalograms showed periodic lateralized epileptiform discharges. Virus titers including herpes simplex virus were all normal. Neuropathological examinations revealed marked atrophic changes in the right temporal lobe, insular lobe and hippocampus with minimal inflammatory signs. The distribution of the lesions was almost identical with that of acute necrotic encephalitis or herpes simplex encephalitis despite its unusually protracted course.     

Encephalitis.

Encephalitis is an acute infection of brain parenchyma characterized clinically by fever, headache, and an altered level of consciousness. There may also be focal or multifocal neurologic deficits, and focal or generalized seizure activity. This article discusses the clinical presentation, diagnosis, and treatment of herpes simplex virus (HSV) encephalitis, the arthropodborne viral encephalidities, Rocky Mountain spotted fever, viral encephalitis in the immunocompromised patient, and postinfectious encephalomyelitis.     

Anti-N-methyl-D Encephalitis

Anti-N-methyl-D-aspartate receptor encephalitis is a rare and serious autoimmune disorder, usually part of a paraneoplastic syndrome. Patients present with a change in mental status, bizarre behavior, and seizures. There are many neoplastic and infectious etiologies, with 90 percent of documented cases in young women, frequently with an ovarian teratoma. Damage to limbic brain receptors is most often induced by teratoma cell-produced anti-N-methyl-D-aspartate antibodies. Treatment targets the specific oncologic or infectious cause and/or employs steroids, plasmapheresis, or other anti-inflammatory therapies. Neurological sequellae persist 75 percent of the time, relapse occurs in 20 percent of cases, and the mortality rate is 25 percent.     

Die Encephalitis

Ohne Zusammenfassung