Anti-N-methyl-d-aspartate receptor encephalitis associated with carcinosarcoma with neuroendocrine differentiation of the uterus

Journal of Neurology -     

Anti-N-methyl-d-aspartate receptor encephalitis associated with intracranial Angiostrongylus cantonensis infection: a case report

Neurological Sciences - Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is a recently described paraneoplastic syndrome with prominent neuropsychiatric symptoms. Many of these cases are...     

Anti-N-methyl-d-aspartate receptor encephalitis in children: Incidence and experience in Hong Kong

Aim

The study aims to analyze the incidence, clinical features, investigation findings and treatment outcomes of anti-N-methyl-d-aspartate receptor encephalitis in children from Hong Kong.

Progressive cortical and sub-cortical alterations in patients with anti-N-methyl-d-aspartate receptor encephalitis

Journal of Neurology - Advanced structural analyses are increasingly being highly valued to uncover pathophysiological understanding of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis....     

Electroencephalograms of children with permanent cortical visual impairment

The electroencephalograms of 40 children with permanent cortical visual impairment (CVI) were studied. CVI was diagnosed in the presence of severe visual loss, normal or minimal ocular findings and clinical, electrodiagnostic and CT evidence of postgeniculate lesions involving the visual cortex. All patients had a multidisciplinary evaluation, including extensive neurological and ophthalmological investigations. The records did not contain any specific features which would be diagnostic of CVI. Isolated occipital spikes were rare and more commonly multifocal EEG disturbance was seen because of diffuse cerebral involvement. Photic stimulation was of little use in the diagnosis. Multihandicapped children with profound CVI tended to show multifocal abnormalities, no alpha rhythm and a suppressed posterior waking background in their EEGs, whereas those with more residual vision usually had an alpha rhythm. The presence or absence of alpha rhythm seemed to reflect the intactness of the striate cortex. However, the lack of alpha rhythm was not always associated with total blindness and not all children with useful residual vision had alpha rhythm.     

Long-term outcome of children with cortical visual impairment

Cortical visual impairment (CVI) is now the most common cause of visual impairment in children. Little is known about the long-term visual outcome. This study evaluates the outcome of children with congenital CVI. Using medical records, 423 children (225 males, 198 females) were identified with congenital CVI. Of these children, 259 had follow-up visual acuity assessments. The children's gestational age varied with 32 weeks or less representing 15.9%; 33 to 36 weeks representing 10.7%; 37 to 42 weeks representing 61.2%; and 43 weeks or greater representing 0.9% (11.3% of patients' gestational age was unrecorded). Clinical data were extracted and information regarding outcome was gathered. The majority of children showed improvement in their visual acuity levels after 2 or more years of follow-up. For the 194 children initially assessed before 3 years of age, 97 had improved, 75 were unchanged, 18 had deteriorated, and 4 had sub-optimal assessments. For the 74 children initially assessed at 3 or more years of age, 23 had improved, 44 remained unchanged, 3 had deteriorated, and 4 had sub-optimal testing. Children with better visual acuity levels at follow-up were more likely to have favourable cognitive outcomes (non-mental retardation) in 12.2% versus 2.8% (p<0.01). Similarly, favourable motor outcomes (independent ambulation) were present in 20.1% for those with better visual acuities versus 7.9% for those with poorer visual acuities (p<0.01). Our study demonstrates that the majority of children with CVI underwent improvement in visual acuity. Additional disabilities were common, but those children with better visual acuity outcomes faired better. Given the frequency of comorbid conditions, appropriate diagnostic assessment services are needed.     

抗N-甲基-D-门冬氨酸受体脑炎

抗N-甲基-D-门冬氨酸受体脑炎(anti-N-methyl-D-aspartate-receptor encephalitis),即抗NMDAR脑炎,是一种抗体介导的脑炎,其发病机制与NMDAR抗体有关,常伴有卵巢畸胎瘤的发生,临床症状十分复杂,病情严重,甚至可危及生命,但是通过积极的治疗,大部分患者可以治愈[1-4].抗NMDAR脑炎最早发现于卵巢畸胎瘤相关性边缘性脑炎(ovarian teratoma-associated limbic encephalitis,OTLE)患者.早在1992年,Sebire等首先报道了该病的临床特征,1997年日本报道了2例OTLE,对其临床表现进行了详细描述,但是对该病的发病机制尚不明确.2005年Dalmau和他的研究团队首次在OTLE患者脑组织中发现了一种新的神经元细胞膜抗原抗体,2年后,他们又鉴定了NMDAR的NR1/NR2B异聚体,揭示了OTLE的发生与NMDAR抗体有关[5-7].     

Anti-NMDA receptor encephalitis associated with ictal asystole

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis usually presents with psychiatric symptoms, behavioural changes, impaired consciousness, seizures and autonomic instability. Ictal asystole is a rare phenomenon associated with complex partial seizures. It is implicated as a potential cause of sudden unexpected death in epilepsy. We report a 41-year-old woman who presented with anti-NMDAR encephalitis. During continuous video electroencephalogram and cardiac monitoring, an episode of ictal asystole was detected. We discuss the potential link between anti-NMDAR encephalitis and ictal asystole. Treatment options for ictal asystole in the setting of anti-NMDAR encephalitis are also discussed.     

Serum NfL associated with anti-NMDA receptor encephalitis

Neurological Sciences - Neurofilament light chains (NfL) have been reported as potential markers for neuronal-axonal injury in neuroinflammatory diseases. In the current study, we describe serum...     

Motor impairment in liver cirrhosis without and with minimal hepatic encephalopathy

Butz M, Timmermann L, Braun M, Groiss SJ, Wojtecki L, Ostrowski S, Krause H, Pollok B, Gross J, Südmeyer M, Kircheis G, Häussinger D, Schnitzler A. Motor impairment in liver cirrhosis without and with minimal hepatic encephalopathy.
Acta Neurol Scand: 2010: 122: 27–35.
© 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Aim – Manifest hepatic encephalopathy (HE) goes along with motor symptoms such as ataxia, mini‐asterixis, and asterixis. The relevance of motor impairments in cirrhotics without and with minimal HE (mHE) is still a matter of debate. Patients and methods – We tested three different groups of patients with liver cirrhosis: no signs of HE (HE 0), mHE, and manifest HE grade 1 according to the West Haven criteria (HE 1). All patients (n = 24) and 11 healthy control subjects were neuropsychometrically tested including critical flicker frequency (CFF), a reliable measure for HE. Motor abilities were assessed using Fahn Tremor Scale and International Ataxia Rating Scale. Fastest alternating index finger movements were analyzed for frequency and amplitude. Results – Statistical analyses showed an effect of HE grade on tremor and ataxia (P < 0.01). Additionally, both ratings yielded strong negative correlation with CFF (P < 0.01, R = ?0.5). Analysis of finger movements revealed an effect of HE grade on movement frequency (P < 0.03). Moreover, decreasing movement frequency and increasing movement amplitude parallel decreasing CFF (P < 0.01, R = 0.6). Conclusion – Our results indicate that ataxia, tremor, and slowing of finger movements are early markers for cerebral dysfunction in HE patients even prior to neuropsychometric alterations becoming detectable.     

Adult‐onset Rasmussen encephalitis associated with focal cortical dysplasia

Rasmussen encephalitis is a rare, devastating condition, typically presenting in childhood. Cases of adult‐onset Rasmussen have also been described, but the clinical picture is less defined, rendering final diagnosis difficult. We present a case of adult‐onset Rasmussen encephalitis with dual pathology, associated with focal cortical dysplasia and encephalitis. We interpreted the Rasmussen encephalitis to be caused by severe and continuous epileptic activity due to focal cortical dysplasia. The best therapeutic approach for such cases remains unclear.     

Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma

OBJECTIVE: To report the autoantigens of a new category of treatment-responsive paraneoplastic encephalitis. METHODS: Analysis of clinical features, neuropathological findings, tumors, and serum/cerebrospinal fluid antibodies using rat tissue, neuronal cultures, and HEK293 cells expressing subunits of the N-methyl-D-aspartate receptor (NMDAR). RESULTS: Twelve women (14-44 years) developed prominent psychiatric symptoms, amnesia, seizures, frequent dyskinesias, autonomic dysfunction, and decreased level of consciousness often requiring ventilatory support. All had serum/cerebrospinal fluid antibodies that predominantly immunolabeled the neuropil of hippocampus/forebrain, in particular the cell surface of hippocampal neurons, and reacted with NR2B (and to a lesser extent NR2A) subunits of the NMDAR. NR2B binds glutamate and forms heteromers (NR1/NR2B or NR1/NR2A/NR2B) that are preferentially expressed in the adult hippocampus/forebrain. Expression of functional heteromers (not single subunits) was required for antibody binding. Eleven patients had teratoma of the ovary (six mature) and one a mature teratoma in the mediastinum; five of five tumors examined contained nervous tissue that strongly expressed NR2 subunits and reacted with patients' antibodies. Tumor resection and immunotherapy resulted in improvement or full recovery of eight of nine patients (paralleled by decreased antibody titers); two of three patients without tumor resection died of neurological deterioration. Autopsies showed extensive microgliosis, rare T-cell infiltrates, and neuronal degeneration predominantly involving, but not restricted to, the hippocampus. INTERPRETATION: Antibodies to NR2B- and NR2A-containing heteromers of the NMDAR associate with a severe but treatment-responsive encephalitis. Our findings provide a diagnostic test and suggest a model of autoimmune NMDAR-related encephalitis with broad implications for other immune-mediated disorders of memory, behavior, and cognition.     

N-Methyl D-aspartate receptor antibody encephalitis associated with myelitis

Encephalitis associated with antibodies to the N-methyl D-aspartate receptor (NMDA-R) was first described in young women with ovarian teratoma. It has subsequently been described in men, children and in those without an underlying tumour. Characteristic clinical features include neuropsychiatric symptoms, seizures, movement disorders, hypoventilation and autonomic instability. Spinal cord disease in association with other typical clinical features has been described in only one patient previously. We report a patient presenting with myelitis, with typical features of NMDA-R associated encephalitis manifesting 3 months later.     

Anti-NMDA receptor encephalitis with associated catatonia during pregnancy

Anti-NMDA-R encephalitis has been described as a cause of acute psychosis in young females. It is rare during pregnancy. We describe a primigravida 32-year-old woman with acute onset psychosis during the first trimester. Eight weeks after becoming pregnant, the patient became psychotic with associated catatonia and autonomic disturbance. Serum anti-NMDA-R antibodies were found. She responded to plasma exchange. At caesarean section, a healthy baby boy was born and a benign mature cystic teratoma was removed from the left ovary. Catatonia associated with psychosis may occur in pregnancy secondary to anti-NMDA-R encephalitis. Prompt and aggressive treatment can lead to a good outcome for both baby and mother.