Summary Background Some encephalitides or seizure disorders once thought idiopathic now seem to be immune mediated. We aimed to describe the clinical features of one such disorder and to identify the autoantigen involved. Methods 15 patients who were suspected to have paraneoplastic or immune-mediated limbic encephalitis were clinically assessed. Confocal microscopy, immunoprecipitation, and mass spectrometry were used to characterise the autoantigen. An assay of HEK293 cells transfected with rodent GABAB1 or GABAB2 receptor subunits was used as a serological test. 91 patients with encephalitis suspected to be paraneoplastic or immune mediated and 13 individuals with syndromes associated with antibodies to glutamic acid decarboxylase 65 were used as controls. Findings All patients presented with early or prominent seizures; other symptoms, MRI, and electroencephalography findings were consistent with predominant limbic dysfunction. All patients had antibodies (mainly IgG1) against a neuronal cell-surface antigen; in three patients antibodies were detected only in CSF. Immunoprecipitation and mass spectrometry showed that the antibodies recognise the B1 subunit of the GABAB receptor, an inhibitory receptor that has been associated with seizures and memory dysfunction when disrupted. Confocal microscopy showed colocalisation of the antibody with GABAB receptors. Seven of 15 patients had tumours, five of which were small-cell lung cancer, and seven patients had non-neuronal autoantibodies. Although nine of ten patients who received immunotherapy and cancer treatment (when a tumour was found) showed neurological improvement, none of the four patients who were not similarly treated improved (p=0.005). Low levels of GABAB1 receptor antibodies were identified in two of 104 controls (p<0.0001). Interpretation GABAB receptor autoimmune encephalitis is a potentially treatable disorder characterised by seizures and, in some patients, associated with small-cell lung cancer and with other autoantibodies. Funding National Institutes of Health.

Brain-computer interface training appears to have yielded some improvement in motor function and brain plasticity. Further controlled research is needed to clarify the role of the brain-computer interface system.

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Amyotrophic lateral sclerosis (ALS) is a rare disease involving selective and progressive degeneration and disappearance of motor neurons. Oxidative stress is believed to contribute to its pathogenesis. We have investigated the efficacy and safety of edaravone, a free radical scavenger previously approved for treatment of acute cerebral infarction, in ALS patients. Within an open trial design, 20 subjects with ALS received either 30 mg (5 subjects) or 60 mg (15 subjects) of edaravone via intravenous drip once per day. Two weeks of administration was followed by a two-week observation period. This four-week cycle was repeated six times. The primary endpoint was the change in the revised ALS functional rating scale (ALSFRS-R) score, while the secondary endpoint was 3-nitrotyrosine (3NT) level in cerebrospinal fluid (CSF). Efficacy was evaluated in the 60 mg group. During the six-month treatment period, the decline in the ALSFRS-R score (2.3+/-3.6 points) was significantly less than that in the six months prior to edaravone administration (4.7+/-2.1 points); the difference between the two was 2.4+/-3.5 points (Wilcoxon signed rank test, p = 0.039). In almost all patients, CSF 3NT, a marker for oxidative stress, was markedly reduced to almost undetectable levels at the end of the six-month treatment period. Data from the present study suggest that edaravone is safe and may delay the progression of functional motor disturbances by reducing oxidative stress in ALS patients.

Recent studies have shown that scalp electroencephalogram (EEG) based brain-computer interface (BCI) has a great potential for motor rehabilitation in stroke patients with severe hemiplegia. However, key elements in BCI architecture for functional recovery has yet to be clear. We in this study focused on the type of feedback to the patients, which is given contingently to their motor-related EEG in a BCI context. The efficacy of visual and somatosensory feedbacks was compared by a two-group study with the chronic stroke patients who are suffering with severe motor hemiplegia. Twelve patients were asked an attempt of finger opening in the affected side repeatedly, and the event-related desynchronization (ERD) in EEG of alpha and beta rhythms was monitored over bilateral parietal regions. Six patients were received a simple visual feedback in which the hand open/grasp picture on screen was animated at eye level, following significant ERD. Six patients were received a somatosensory feedback in which the motor-driven orthosis was triggered to extend the paralyzed fingers from 90 to 50°. All the participants received 1-h BCI treatment with 12–20 training days. After the training period, while no changes in clinical scores and electromyographic (EMG) activity were observed in visual feedback group after training, voluntary EMG activity was newly observed in the affected finger extensors in four cases and the clinical score of upper limb function in the affected side was also improved in three participants in somatosensory feedback group. Although the present study was conducted with a limited number of patients, these results imply that BCI training with somatosensory feedback could be more effective for rehabilitation than with visual feedback. This pilot trial positively encouraged further clinical BCI research using a controlled design.

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A new method for the evaluation of stroke patients, designated the Stroke Impairment Assessment Set (SIAS) is presented. The SIAS primarily employs single task assessment of various functions and rates performance on scales of 0 to 5 or 0 to 3. The items evaluated include motor function, muscle tone, sensation, range of motion, pain, trunk control, visuospatial perception, aphasia and functions on the unaffected side. Scores for each item are plotted on a radar chart, so that deficits can be identified at a glance. The inter-observer variation in SIAS scores is acceptable and assessment can be performed as part of a routine clinical examination.

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