Cortical thickness across the cingulate gyrus in schizophrenia and its association to illness duration and memory performance.

Schizophrenia has been associated with structural brain abnormalities and cognitive deficits that partly change during the course of illness. In the present study, cortical thickness in five subregions of the cingulate gyrus was assessed in 44 patients with schizophrenia-spectrum disorder and 47 control persons and related to illness duration and memory capacities. In the patients group, cortical thickness was increased in the posterior part of the cingulate gyrus and related to illness duration whereas cortical thickness was decreased in anterior parts unrelated to illness duration. In contrast, cortical thickness was related to episodic and working memory performance only in the anterior but not posterior parts of the cingulate gyrus. Our finding of a posterior cingulate increase may point to either increased parietal communication that is accompanied by augmented neural plasticity or to effects of altered neurodegenerative processes in schizophrenia.

Cerebellar Contribution to Context Processing in Extinction Learning and Recall.

Whereas acquisition of new associations is considered largely independent of the context, context dependency is a hallmark of extinction of the learned associations. The hippocampus and the prefrontal cortex are known to be involved in context processing during extinction learning and recall. Although the cerebellum has known functional and anatomic connections to the hippocampus and the prefrontal cortex, cerebellar contributions to context processing of extinction have rarely been studied. In the present study, we reanalyzed functional brain imaging data (fMRI) of previous work investigating context effects during extinction in a cognitive associative learning paradigm in 28 young and healthy subjects (Lissek et al. Neuroimage. 81:131-3, 2013). In that study, event-related fMRI analysis did not include the cerebellum. The 3 T fMRI dataset was reanalyzed using a spatial normalization method optimized for the cerebellum. Data of seven participants had to be excluded because the cerebellum had not been scanned in full. Cerebellar activation related to context change during extinction learning was most prominent in lobule Crus II bilaterally (p < 0.01, t > 2.53; partially corrected by predetermined cluster size). No significant cerebellar activations were observed related to context change during extinction retrieval. The posterolateral cerebellum appears to contribute to context-related processes during extinction learning, but not (or less) during extinction retrieval. The cerebellum may support context learning during extinction via its connections to the hippocampus. Alternatively, the cerebellum may support the shifting of attention to the context via its known connections to the dorsolateral prefrontal cortex. Because the ventromedial prefrontal cortex (vmPFC) is critically involved in context-related processes during extinction retrieval, and there are no known connections between the cerebellum and the vmPFC, the cerebellum may be less important during extinction recall.

Boucher-Neuhäuser syndrome: cerebellar degeneration, chorioretinal dystrophy and hypogonadotropic hypogonadism: two novel cases and a review of 40 cases from the literature.

The combination of progressive cerebellar degeneration, hypogonadotropic hypogonadism and chorioretinal dystrophy defines the rare Boucher-Neuhäuser syndrome (BNS), which has recently been linked to autosomal-recessive mutations in the PNPLA6 gene in four index patients. Here we present two novel unrelated patients with BNS, where we identified four recessive PNPLA6 mutations (3 of them novel) as the genetic cause, using a targeted high-throughput approach. This finding provides the first replication from independent families that BNS is caused by PNPLA6 and, moreover, highlights PNPLA6 as the major gene leading to BNS. Given the fact that the major gene causing BNS has thus now been identified, we summarize the spectrum of clinical presentations and phenotype evolution of BNS based on a systematic in-depth review of the literature of previously published cases (n = 40). Both the two cases presented here and our review of the literature propose that the clinical presentation of BNS can be variable regarding both the age (ranging from 1 to 40 years) and the clinical symptoms at onset (cerebellar ataxia in 38 %; vision loss in 36 %; delayed puberty in 26 %). A substantial fraction of BNS cases may present with relatively selective atrophy of the superior and dorsal parts of the cerebellar vermis along with atrophy of the cerebellar hemispheres on MRI, while brainstem or cortical changes on MRI seem to be present only in small fractions. Also in the literature, no other major genetic causes of BNS other than PNPLA6 mutations were identified.

[Cannabis hyperemesis syndrome]. / Cannabis-Hyperemesis-Syndrom.

We present the course of cyclical hyperemesis most likely induced by cannabis in a young cannabis-dependent, but otherwise healthy female adult. Cyclical hyperemesis developed in parallel to increasing cannabis inhalation, and remitted completely within a few days of abstinence in a protective inpatient setting. Just as in those increasing cases which can be found in literature, the hyperemesis improved by taking a hot shower or bath at the beginning of the detoxification. This thermosensitivity, along with the detection of a central disturbance of the thyroid axis, points to the hypothalamic cannabinoid system being involved in cannabis-induced cyclical hyperemesis. The patient was followed up for 4 months without any re-occurrence of the syndrome during controlled cannabis abstinence.

Microbleeds and free active MMP-9 are independent risk factors for neurological deterioration in acute lacunar stroke.

BACKGROUND AND PURPOSE: in this prospective study, we evaluated mutual relationships amongst microbleeds, matrix metalloproteinase-9 (MMP-9) and neurological deterioration in patients with their first acute lacunar stroke. METHODS: based on diffusion-weighted image findings, we recruited 206 patients with their first acute lacunar stroke. Those without a MRI scan were excluded. Small (a maximum lesion diameter of 15 mm) areas of subcortical gray and white matter with increased signals were considered as lacunar infarctions. GRE images were obtained within 24 h of the onset of stroke symptoms. Venous blood was sampled at base line (within 24 h). Clinical, biochemical, rheological and inflammatory parameters, neurological scales and free, active MMP-9 levels were compared between patients with and without microbleeds. Neurological deterioration was defined as an increase in more than two points on the National Institutes of Health Stroke Scale Score baseline 14 days after the onset of lacunar stroke. RESULTS: of the patients, 79 (38.3%) had microbleeds and 48 (23.3%) showed neurological deterioration. Free, active MMP-9 and C-reactive protein (CRP) levels were significantly increased amongst patients with microbleeds (P < 0.001 and P = 0.047, respectively). Existence of microbleeds (RR = 2.47, 95% CI = 1.25-3.83) and increased free, active MMP-9 (RR = 1.10 per 10 ng/ml, 95% CI = 1.03-1.19) were identified as independent risk factors for neurological deterioration after adjusting for potential confounders. DISCUSSION: ncreased levels of active MMP-9 and the existence of microbleeds might be useful in predicting the deterioration following an initial acute lacunar stroke.

[A case of cannabis-induced mania]. / Manie durch Cannabis--ein Fallbericht.

We present the course of a mania most likely induced by cannabis in a young cannabis-dependent patient, but apart from this, healthy male adult. Mania developed in parallel to increasing cannabis inhalation and remitted quickly and completely within one week of abstinence without any antimanic medication in a protective inpatient setting. Simultaneously, the THC-COOH level decreased in serum and urine.

A case of unilateral hemispheric encephalitis.

Herein we report a case of encephalitis involving the unilateral hemisphere, with a clinical course different from that of Rasmussen syndrome. A 44-year-old man visited us because of headache and language abnormality. Cerebrospinal fluid examination showed lymphocytosis with increased level of protein. Brain MRI showed abnormal findings limited to the unilateral hemisphere. The symptoms and signs improved without any specific antiviral treatment in a week. However, language disturbance and right hemiparesis developed after a week. Steroid therapy was effective. He recovered without any neurologic sequelae. Our case was unusual encephalitis involving the unilateral hemisphere, which was benign and steroid-responsive.

Identification of the full-length huntingtin- interacting protein p231HBP/HYPB as a DNA-binding factor.

Neurodegeneration in Huntington's disease (HD) is associated with an elongated glutamine tract in the widely expressed huntingtin protein. Although the pathogenic mechanisms are still unknown, the distinct physical properties of mutant huntingtin in the brain suggest that other factors including huntingtin-interacting proteins might play a specific role. We have previously identified a DNA-binding motif in the proximal E1A promoter of adenovirus serotype 12 as responsible for E1A autoregulation. Here, we identified the p231HBP protein as a DNA-binding factor, the C-terminal portion of which has recently been characterized as the huntingtin-interacting protein HYPB of unknown function. We have determined the full-length cDNA sequence, identified several domains supporting its gene regulatory functions, and mapped the HBP231 gene to chromosome 3p21.2-p21.3. Our results provide an interesting molecular link between huntingtin and a DNA-binding factor, implicating that this interaction might result in the alteration of cellular gene expression involved in HD pathogenesis.

Localization of Claude's syndrome.

Although the red nucleus often has been suggested to be the lesion site responsible for Claude's syndrome, the precise localization of the syndrome is uncertain. Lesion sites were reviewed in six patients with Claude's syndrome and compared with other patients with the syndrome identified by a literature search. The findings strongly suggest that Claude's syndrome occurs because of a lesion of the superior cerebellar peduncle just below and medial to the red nucleus. Midbrain infarction and partial oculomotor nerve palsy was common.

Superior sagittal sinus thrombosis and transient ischemic attacks: possible mechanism.

Clinical manifestations of superior sagittal sinus (SSS) thrombosis are nonspecific but characterized by headache, papilledema, seizures, focal deficits, progressive coma and death. Recurrent transient focal neurologic deficit is an extremely rare manifestation in superior sagittal sinus thrombosis and the mechanism is unknown. A 45-year-old man presented with headache for two weeks and four episodes of transient (5-10 minutes) right or left hemiparesis for two days. Magnetic resonance image and transfemoral cerebral angiography revealed superior sagittal sinus thrombosis with numerous prominent collateral venous channels. There was no parenchymal lesion. After four days of heparinization, no further transient focal neurologic deficits developed. Follow-up angiography showed partial recanalization of the SSS. Possible mechanism of transient ischemic attacks in this patient is thought to be a transient functional disturbance due to a temporal reduction of tissue perfusion in the process of operating fully-enough collateral channels.

Encephalitis and polyradiculoneuritis following rubella virus infection--a case report.

Amongst neurological complications of rubella virus infection, polyradiculoneuritis as well as encephalitis is very rare. Only one case of postrubella polyradiculoneuritis combined with encephalitis has been reported to our knowledge. A 17-years-old male presented with suspected meningoencephalitis in a recent epidemic of rubella in a southern district of Korea. He developed symmetrical hyporeflexic weakness of all four extremities with urinary disturbance several days later. Rubella IgM antibody titer (enzyme linked immunosorbent assay) was 58 AU/mL in serum and 12 AU/mL in cerebrospinal fluid. Electrophysiologic studies showed peripheral polyradiculoneuropathy with multifocal conduction block. Considering the involvement of the central nerve as well as the peripheral nerve in an adult patient, this case is thought to be valuable in view of the pathophysiology of neurologic complication in rubella virus infection.

A case of intracerebral air embolism following acupuncture.

Recently, the reported causes of cerebral air embolism are more from invasive medical procedures than decompressive sickness. We report a patient with homolateral cerebral air embolism probably due to acupuncture to the anterior neck. A 64-year-old man was admitted with early seizure followed by unconsciousness and focal neurologic deficits. He had subcutaneous emphysema into the left anterior neck and chest wall. Several hours before admission, he underwent the acupuncture in the area of left anterior neck for the treatment of previous hemiplegia. Initial brain computed tomography (CT) showed multiple, small (2-8 mm in diameter) air bubbles in the corticomedullary junction area of the left cerebral hemisphere. He regained consciousness 2 days later. A delayed CT scan, taken 6 days after the event, showed no air bubbles in the cerebral hemisphere but massive cerebral edema. The patient's condition continued to improve during his hospital stay. He was discharged 3 weeks later with minimal neurologic deficits. Unilateral cerebral artery air embolism in this patient is thought to be caused by direct infiltration of air to the common carotid artery following acupuncture.

A case of aneurysm of the vein of Galen.

Aneurysm of the vein of Galen is a very rare disease. The authors present a case of secondary aneurysm of the vein of Galen which was confirmed by characteristic clinical symptoms, brain CT and angiographic findings. The patient was a 14-year-old right handed girl with intermittent headache, nausea, vomiting, dysphasia and gait disturbance. Neurologic examination revealed dysarthria, nasal voice, blurring of both margins of optic discs, truncal ataxia and dysdiadochokinesia. Sensory function was normal but right hemiparesis was seen. Roentgenogram of the skull revealed diffuse thinning of the calvarium, widening of sella turcica and erosion of clinoid processes. Computed tomogram of the brain showed dilatation of all ventricles and round hyperdense mass behind the third ventricle in the midline. The lesion was enhanced markedly and homogeneously. Left and right internal carotid angiograms showed arteriovenousmal-formation with drainage to the aneurysm of the vein of Galen.

Principles of Food Dehydration.

The basic principles involved in the food dehydration are reviewed. Major emphasis is placed on types of water held in foods, heat of vaporization of water in food product, equilibrium moisture and water activity relationship and dehydration rate mechanism.