Accare, locatie de Ruyterstee, Fazantenlaan 1, 9422 EZ Smilde. firstname.lastname@example.org
We describe the case of a 17-year-old girl with anti-NMDA-receptor encephalitis. She had the characteristic psychiatric symptoms such as hallucinations, delirious and bizarre behaviour, and catatonic symptoms. She later also displayed neurological symptoms such as epileptic seizures, dyskinesias and sensitivity disturbances. After treatment with corticosteroids and immunoglobulins patient recovered completely. Potentially lethal symptoms can arise from the autonomic dysregulation. The incidence of this disorder has not yet been established. The catatonic features should be treated adequately with high doses of benzodiazepines. Because patients usually present with psychiatric symptoms, treatment often starts in a psychiatric setting. In view of the serious somatic complications it is desirable that the patient be treated in a general hospital. Early diagnosis and treatment are very important because the chances of recovery are thereby enhanced.
Servicio de Psiquiatría, Hospital Clínico Universitario Lozano Blesa, Zaragoza, España. email@example.com
The clinical diagnosis of encephalitis due to anti-glutamate receptor N-methyl D-aspartate (NMDA) antibodies (anti-NMDA encephalitis) is made more complex by the fact that psychiatric clinical features are usually predominant in the early stages. This can lead to a delay in the diagnosis, treatment and prognosis of the disease.
We report on two clinical cases attended by the Child/Youth Psychiatry Section of our hospital, in collaboration with the Paediatric and Neurology services. Case 1: a 4-year-old male who was referred owing to behavioural alterations and the regression of previously acquired skills. Case 2: a 13-year-old female who was admitted due to a behavioural disorder within the context of a possible initial psychotic episode. In both cases appropriate complementary tests were performed, including lumbar puncture and anti-NMDA antibodies, which were positive. Once the diagnosis of anti-NMDA encephalitis had been reached, treatment was started: in the first case, with intravenous perfusion of corticoids and immunoglobulins, while in the second rituximab had to be associated. Both patients progressed towards clinical improvement.
Over the last two years there has been an increase in the number of cases of anti-NMDA encephalitis diagnosed in the child/youth population. It is a neuropsychiatric, autoimmune condition, which can correspond to a paraneoplastic syndrome. Follow-up and transvaginal ultrasonography as well as thoracoabdominal computerised axial tomography scans are recommended for at least two years after diagnosis. Early diagnosis and treatment are important, which means that a multi- and interdisciplinary approach is required.
Oddział Neurologii, SPSK nr 7, Śląski Uniwersytet Medyczny w Katowicach, ul. Ziołowa 45-47, 40-635 Katowice.
We present a patient with multiple sclerosis, diagnosed at the age of 16.5, in whom mental and orientation disturbances, strange behaviour as well as bizarre dyskinesias of the face and extremities occurred at age 20. After several days, tonic-clonic status epilepticus developed. Head computed tomography showed no abnormalities. Lumbar puncture revealed a pleocytosis of 20/3, which became normal after treatment. Seizures were brought under control, but the psychiatric symptoms persisted; they subsided after a dozen or so weeks. Magnetic resonance of the head and cervical spinal cord did not show any new abnormalities. After another several months, all symptoms recurred. A wide range of laboratory tests, as well as positron emission tomography, did not reveal any abnormalities. Suspicion of autoimmune encephalitis led to a test for serum anti-NMDA-receptor antibodies that confirmed the diagnosis. After immunotherapy, our patient improved and was transferred for rehabilitation.
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a recently recognized autoimmune neurologic disorder that presents with severe neuropsychiatric symptoms in previously healthy children. A 4-year-old Lebanese girl presented with new-onset behavioral changes, orofacial dyskinesias, fluctuation in consciousness, inability to walk, and mutism. Antibodies directed cerebrospinal fluid. Prompt treatment with a single course of intravenous immunoglobulin resulted in early complete recovery. This is the first case report of a Middle Eastern child affected with this condition.
1Department of Pediatric Medico-surgical, Pediatric Neurology and Neurorehabilitation Unit, CHUV, Lausanne, Switzerland.
Anti-N-methyl-d-aspartate (anti-NMDA) receptor encephalitis likely has a wider clinical spectrum than previously recognized. This article reports a previously healthy 16-year-old girl who was diagnosed with anti-NMDA receptor encephalitis 3 months after onset of severe depression with psychotic features. She had no neurological manifestations, and cerebral magnetic resonance imaging (MRI) was normal. Slow background on electroencephalogram and an oligoclonal band in the cerebrospinal fluid prompted the search for anti-NMDA receptor antibodies. She markedly improved over time but remained with mild neuropsychological sequelae after a trial of late immunotherapy. Only a high index of suspicion enables recognition of the milder forms of the disease masquerading as primary psychiatric disorders.
Department of Psychiatry, Hokkaido University.
Recently, paraneoplastic encephalitis associated with ovarian teratoma, which predomi nantly affects young women, has been reported. Its symptoms are severe but often treatment-responsive and reversible. Various psychiatric symptoms occurring shortly after onset are characteristic of this encephalitis. A 22-year-old female who had a fever and common cold-like symptoms presented with short-term memory loss. She was suspected to have viral encephalitis, but the cerebrospinal fluid (CSF) showed no marked change. Shortly after that, she developed delusions and hallucinations and presented with psychomotor excitement. She was suspected of having schizophrenia and admitted to the psychiatry department. However, several days after admission, she showed upper limb convulsion, orofacial dyskinesias, and central hypoventilation and became unresponsive. The results of laboratory tests were within the normal range, and there was no marked elevation of anti-viral antibody titers. Brain imaging was normal, but a solid tumor containing soft tissue and calcified components, probably an ovarian teratoma, was discovered on an abdominal CT scan. Anti-N-methyl-D-aspartate (NMDA) receptor antibody was positive in her CSF and blood serum, and we diagnosed her with “Anti-NMDA receptor encephalitis”. Gamma globulin was very effective, and the ovarian teratoma was removed. Finally, she could be discharged from our hospital without any sequela, and returned to her job. Psychiatrists often encounter encephalitis patients with psychiatric symptoms. If the type of encephalitis is unknown, we should keep this disease in mind.
Department of Emergency and Critical Care Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa 259-1193, Japan. firstname.lastname@example.org.
A previously healthy 21-year-old woman, transported to our medical emergency center for excluding organic brain disease, had undergone medical examination 9 days before for trembling in her left hand, which was caused by stress. The patient exhibited fever and strange behaviors, e.g., wandering around, babbling, and making smoking gestures; hence, psychiatric examination was performed. The patient’s Glasgow Coma Scale score was 4-3-5, and involuntary movement was observed. Cerebrospinal fluid examination revealed increased cell count; hence, we suspected anti-N-methyl-d-aspartate (NMDA) receptor encephalitis. We conducted an abdominal CT scan, which revealed a neoplastic lesion with calcification in the right ovary. Early steroid pulse therapy was started. On hospital day 25, she tested positive for anti-NMDA receptor antibodies; hence, anti-NMDA receptor encephalitis and concomitant ovarian teratoma was diagnosid. She underwent right adnexectomy; subsequently, immunotherapy was performed. The patient recovered and was discharged on hospital day 105. Anti-NMDA receptor encephalitis is not uncommon; however, this disease must be considered for young encephalitis patients exhibiting psychiatric symptoms. If patients (aged ≤ 30 years) presents with encephalitis of uncertain etiology, psychiatric symptoms, seizures, movement disorders, or psychosis, clinicians should consider anti-NMDA encephalitis as a possible diagnosis. Clinical diagnosis should be waged early to ensure timely treatment.
Department of Psychiatry, Royal College of Surgeons in Ireland, RCSI Education and Research Centre, Beaumont Hospital, Dublin 9, Ireland.
We present four cases of confirmed anti-NMDA receptor encephalitis; three presented initially with serious psychiatric symptoms and the other developed significant psychiatric symptoms during the initial phase of illness. Brain biopsy findings of one patient are also described. Psychiatrists should consider anti-NMDA receptor encephalitis in patients presenting with psychosis and additional features of dyskinesias, seizures and catatonia, particularly where there is no previous history of psychiatric disorder.
Hospital Universitario 12 de Octubre, Madrid, Espana. email@example.com
Autoimmune encephalitis against N-methyl-D-aspartate (NMDA) receptors is being diagnosed more and more frequently in the paediatric age. It should be suspected in children with psychiatric symptoms, encephalopathy, abnormal movements or epileptic seizures. Paraneoplastic cases are less frequent than in adults.
We report the case of a boy, 2.5 years of age, with subacute encephalopathic signs and symptoms and epileptic seizures followed by behaviour disorders, neurological regression, dyskinesias and insomnia. Results of a cerebrospinal fluid study were normal, the magnetic resonance scan of the head revealed a focal periventricular lesion and diffuse leptomeningeal uptake; moreover, the serial electroencephalograms showed high-amplitude delta activity interspersed with generalised intercritical epileptiform activity. The patient was given empirical treatment with high doses of corticoids and intravenous immunoglobulins with no response. After showing up positive for antibodies against the NMDA receptor, plasmapheresis was begun, which led to his swift and spectacular recovery. After more than 18 months’ follow-up, his sequelae are limited to mild behavioural and language alterations. He has had no relapses and has not needed any kind of maintenance treatment.
Anti-NMDA encephalitis is a treatable disorder and, sometimes, the first evidence of an underlying neoplasia, which makes its early recognition and treatment essential. Treatment of the non-paraneoplastic forms are based on immunotherapy: glucocorticoids, intravenous immunoglobulins, plasmapheresis and immunosuppressants. Plasmapheresis can bring about a fast, spectacular improvement.