Simultaneous Four Supratentorial Lesions Predict Tube Dependency Due to an Impaired Anticipatory Phase of Ingestion.

This study aimed to identify the neuroanatomical predictors of oropharyngeal dysphagia and tube dependency in patients with supratentorial or infratentorial ischemic strokes. Patients with acute ischemic stroke were enrolled and were classified into 3 groups: right supratentorial (n = 61), left supratentorial (n = 89), and infratentorial stroke (n = 50). Dysphagia was evaluated by a modified water swallowing test and the Food Intake LEVEL Scale to evaluate oropharyngeal dysphagia and tube dependency, respectively. As two dysphagia parameters, we evaluated the durations from onset of stroke to (1) success in the modified water swallowing test and to (2) rating 7 points or above on the Food Intake LEVEL Scale: patients regained sufficient oral intake and were not tube-dependent. Voxel-based lesion-symptom mapping analysis was performed for a spatially normalized lesion map of magnetic resonance imaging to explore the anatomies that are associated with the two dysphagia parameters for each stroke group. The right precentral gyrus and parts of the internal capsule are associated with oropharyngeal dysphagia. The four supratentorial areas are associated with tube dependency. The dorsal upper medulla is associated with both oropharyngeal dysphagia and tube dependency. These results suggest that supratentorial stroke patients can be tube-dependent due to an impaired anticipatory phase of ingestion. The simultaneous damage in the four supratentorial areas: the inferior part of the precentral gyrus, lenticular nucleus, caudate head, and anterior insular cortex, predicts tube dependency. In contrast, infratentorial stroke patients can be tube-dependent due to oropharyngeal dysphagia caused by lesions in the dorsal upper medulla, damaging the swallowing-related nucleus.

Correlation of callosal angle at the splenium with gait and cognition in normal pressure hydrocephalus.

OBJECTIVE: Idiopathic normal pressure hydrocephalus (iNPH) is characterized by ventricular enlargement that deforms the corpus callosum, making the callosal angle (CA) small. The authors aimed to evaluate the clinical usefulness of the CA in different planes in iNPH. METHODS: Forty patients with iNPH were included in the study. As a control group, 241 patients with other neurological diseases and 50 healthy controls were included. The subjects had been seen at the authors' institutions from 2010 to 2020. The Timed Up and Go (TUG) test total time and Mini-Mental State Examination (MMSE) total score were evaluated. CAs were measured in the axial plane at the splenium and genu and in the coronal plane at the anterior commissure and posterior commissure by using 3-dimensional T1-weighted MR images. As other hydrocephalus parameters, the Evans index, frontal-occipital horn ratio, and third ventricular width were also measured in patients with iNPH. Associations between each CA or hydrocephalus parameter and clinical parameters were evaluated. The classification efficacy of each CA in differentiating between iNPH and other neurological diseases and healthy controls was evaluated. RESULTS: The CA at the splenium, but no other hydrocephalus parameters, was correlated with TUG total time or MMSE total score in patients with iNPH. Receiver operating characteristic analysis showed that a CA of 71.1° at the splenium has 90.0% sensitivity and 89.0% specificity in discriminating iNPH from other neurological diseases and healthy controls. Probabilistic tractography analysis showed that neuronal fibers via the splenium connect the superior parietal lobules, temporal lobes, and occipital lobes. CONCLUSIONS: The study results suggest that interhemispheric disconnections at the splenium are, at least in part, responsible for gait and cognitive disturbance in iNPH. The CA at the splenium is a unique morphological feature that correlates with gait and cognition in iNPH, and it is useful for discriminating iNPH from other neurological diseases and healthy controls.

[A Case of an Elderly Diabetic Patient Developing Wernicke Encephalopathy without Alcohol Abuse or an Unbalanced Diet].

A 70-year-old man with a 28-year history of type 2 diabetes mellitus was admitted due to persistent vomiting and neurological abnormalities in Nov 2012. He had developed gait disturbance and diplopia for six months during antiplatelet therapy, which was initiated following the diagnosis of a cerebellar infarction in June 2012. He had nystagmus, truncal ataxia and an ocular motility disorder, and the MRI study showed increased FLAIR and DWI signals in the peri-third ventricle and periaqueductal region, in addition to the cerebellar vermis. Wernicke encephalopathy was suspected according to his symptoms, and thiamine administration dramatically improved his condition. He did not have a history of alcohol abuse or poor eating habits; however, various coexisting factors, including diabetes mellitus, pyloric stenosis and the use of antiulcer drugs and insulin, were considered to be responsible for Wernicke encephalopathy. This case demonstrates the importance of distinguishing Wernicke encephalopathy from cerebrovascular disease in elderly patients.

A novel mutation of PDE8B Gene in a Japanese family with autosomal-dominant striatal degeneration.

BACKGROUND: Autosomal-dominant striatal degeneration is a rare autosomal-dominant neurodegenerative movement disorder characterized by slowly progressive parkinsonism. Recently, a mutation of the cyclic nucleotide phosphodiesterase 8B gene was reported to be a causal gene mutation of this disease. METHODS: We report on the clinical characteristics of 2 patients of a Japanese family with autosomal-dominant striatal degeneration and the result of gene mutation analysis of this family. RESULTS: Clinical features of the patients are slowly progressive parkinsonism and brain MRI showing high signal intensity in T2-weighted images in the striatum. We found a heterozygous nonsense mutation in the first exon of cyclic nucleotide phosphodiesterase 8B gene, which is predicted to disrupt all important functional domains of the cyclic nucleotide phosphodiesterase 8B protein. CONCLUSIONS: This family is the second family with autosomal-dominant striatal degeneration after the first German family, confirming that cyclic nucleotide phosphodiesterase 8B gene is the causative gene for this disease.