Status epilepticus suspected autoimmune: Neuronal surface antibodies and main clinical features.

OBJECTIVES: Status epilepticus (SE) can be associated with neuronal surface antibodies (NS-Abs) but NS-Ab detection rate remains unknown in patients with SE of unclear etiology at symptom presentation but suspected of having an autoimmune etiology (SE suspected autoimmune). We aimed to determine the NS-Ab detection rate and the clinical features that predict the presence of NS-Abs in patients with SE suspected autoimmune. METHODS: We retrospectively reviewed the clinical information of 137 patients with SE suspected autoimmune who underwent testing for NS-Abs between January 2007 and September 2020. NS-Abs were examined in both serum and cerebrospinal fluid (CSF) obtained at symptom onset with established assays. We classified brain magnetic resonance imaging (MRI) findings into unremarkable, autoimmune limbic encephalitis (ALE) (bilateral abnormalities highly restricted to the medial temporal lobes), ALE-Plus (ALE pattern and additional extramedial temporal lobe abnormalities), multifocal cortico-subcortical (MCS), or other pattern. We compared the clinical features between patients with and without NS-Abs. RESULTS: Forty-four patients (32.1%) had NS-Abs, including 35 N-methyl-d-aspartate receptor (NMDAR) (one with concurrent γ-aminobutyric acid B receptor [GABAbR] and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor [AMPAR]), 5 γ-aminobutyric acid A receptor (GABAaR), 2 leucine-rich glioma-inactivated 1(LGI1), 1 GABAbR, and 1 unknown antigens. Compared with NS-Ab-negative patients, NS-Ab-positive patients were more likely to have a preceding headache (56.8% vs 26.7%), preceding psychobehavioral or memory alterations (65.9% vs 20.4%), involuntary movements (79.5% vs 16.1%), CSF pleocytosis (81.8% vs 62.0%), elevated immunoglobulin G (IgG) index (45.2% vs 15.6%), oligoclonal bands (51.5% vs 9.5%), tumor (47.7% vs 8.6%), and higher APE2 score (median of 9 vs 7), and they were less likely to have an ALE-Plus pattern (2.3% vs 23.7%). However, preceding fever and ALE or MCS pattern were not different between the two groups of patients. SIGNIFICANCE: When an autoimmune etiology was suspected, there was a relatively high likelihood (one of three patients) of identifying NS-Abs. Some clinical features (preceding symptoms, inflammatory CSF) predict a higher likelihood of finding NS-Ab positivity, but the ALE-Plus MRI pattern is more likely suggestive of NS-Ab negativity.

Different atrophic patterns in early- and late-onset Alzheimer's disease and evaluation of clinical utility of a method of regional z-score analysis using voxel-based morphometry.

BACKGROUND/AIMS: We evaluated the differential patterns of brain atrophy in early- and late-onset Alzheimer's disease (AD) by measuring regional z-scores of voxel-based morphometry and assessed the availability of the method for clinical use. METHODS: The first 50 patients with probable AD were compared to 83 age-matched control subjects to identify the brain atrophy. Regions of interest were set in the areas showing z-scores >4. To find substantial differences in the atrophy pattern, principal component analysis was performed. The second group of 56 patients with memory complaints entered the study for evaluation of the clinical use of the model. RESULTS: The centers of the regions of interest were the amygdala, anterior hippocampi, posterior hippocampi, temporal cortices and subcallosal cortex, and left posterior cingulate cortex (PCC). Eigenvectors of the temporal cortices and left PCC showed counter-directions to those of patient age, suggesting that patients with younger onset age were preferentially associated with atrophy of those regions. Differential analyses of the second group showed high availability for the detection of abnormal brain atrophy in people with subjective memory complaints. CONCLUSION: AD with earlier onset is preferentially related to PCC and temporal lobe atrophy. Voxel-based morphometry can be statistically analyzed, and this method has the potential for bias-free assessment of brain atrophy.

Cervical Cord Infarction Caused by Dissection of the Intracranial Segment of the Vertebral Artery.

Cervical cord infarction is uncommon but has been increasingly reported as a complication of vertebral artery dissection (VAD). A 54-year-old woman presented with neck pain and neurological deficit following sudden neck movement. Radiological findings suggested cervical cord infarction in the anterior spinal artery territory at the C5-C6 vertebral level and dissection of the intracranial segment of the right vertebral artery. Cervical cord infarction due to VAD is usually caused by dissection of its extracranial segment. The present case indicates that dissection of the intracranial segment of the vertebral artery can also cause cervical cord infarction.

Subacute subdural hygroma and presyrinx formation after foramen magnum decompression with duraplasty for Chiari type 1 malformation.

A 15-year-old girl developed a rare case of subdural hygroma after foramen magnum decompression for Chiari type 1 malformation manifesting as rapid symptom deterioration around 10 days after uncomplicated operation with uneventful immediate postoperative course. Progressive enlargement of the subdural hygroma in both supra- and infratentorial spaces was followed by the development of hydrocephalus. Syringomyelia improved shortly after the first operation but then deteriorated with massive presyrinx formation. Reoperation with wide opening of the arachnoid membrane lead to a rapid resolution of the hydrocephalus and the presyrinx. The present case shows that wide opening of the arachnoid membrane is an effective therapeutic option.