Dementia Screening Based on SVM Using Qualitative Drawing Error of Clock Drawing Test.

In the welfare of the elderly, it is important to detect the signs of dementia at an early stage and prevent it from becoming serious. We evaluated the performance of SVM-based cognitive function classification models and investigated the drawing features that contribute to distinguishing the severity of cognitive functions. Clock drawing test (CDT) was conducted on three groups of elderly people with different degrees of cognitive impairment. Feature selection was applied to the qualitative drawing features of the CDT, and a two-class classification model was constructed using support vector machine. The results showed that the five features related to conceptual deficits and spatial and planning deficits could be used to classify the dementia group and healthy control group with 79 % accuracy, and all the features showed statistically significant differences. It is suggested that these qualitative drawing features of the CDT can be applied to dementia screening.

Acute isolated bilateral mydriasis: case reports and review of the literature.

Isolated bilateral internal ophthalmoplegia is a rare and problematic condition affecting activities of daily living. Herein, we describe the cases of 2 patients with postinfectious isolated bilateral internal ophthalmoplegia, i.e., mydriasis without external ophthalmoplegia. One patient demonstrated no other neurological symptom, while the other patient showed mild gait ataxia. Magnetic resonance imaging revealed no abnormal findings in the brain or brainstem. Light-near dissociation of the pupils was not recognized in either patient, and supersensitivity to dilute pilocarpine was observed in 1 of the 2 patients. An increased titer of the anti-GQ1b IgG antibody was noted in 1 patient. A review of the literature revealed five similar cases; the symptomatic characteristics, ratio of positive anti-GQ1b IgG antibody, and effective treatment are discussed.

Acute lumbosacral transverse myelitis.

We report two patients with idiopathic acute lumbosacral myelitis, a rare form of acute transverse myelitis. Both patients developed urinary retention, moderate motor and sensory paresis of the lower extremities, severe sensory deficit in the anogenital region and reduced deep tendon reflexes. Steroid pulse therapy was initiated within 2 days after onset, and progress of the symptoms stopped immediately after administration in both of our patients. The sequelae of the sensory deficits in the sacral dermatome distribution and urinary retention impaired daily functioning. Immediate immunosuppressive therapy, including high-dose steroid treatment, is important to improve the prognosis of acute lumbosacral transverse myelitis.

Intravenous immunoglobulin (IVIg) with methylprednisolone pulse therapy for motor impairment of neuralgic amyotrophy: clinical observations in 10 cases.

BACKGROUND: Neuralgic amyotrophy (NA) is a distinct peripheral nervous system disorder characterized by attacks of acute neuropathic pain and rapid multifocal weakness and atrophy unilaterally in the upper limb. The current hypothesis is that the episodes are caused by an immune-mediated response to the brachial plexus, however, therapeutic strategies for NA have not been well established. METHODS AND RESULTS: We retrospectively reviewed 15 case series of NA; 10 of the 15 patients received intravenous immunoglobulin (IVIg) with methylprednisolone pulse therapy (MPPT) and 9 of these 0 patients showed clinical improvement of motor impairment. CONCLUSION: Our clinical observations do not contradict the possibility that IVIg with MPPT may be one of the potential therapeutics for NA, however the efficacy remains to be established. Further confirmatory trials are needed in patients with various clinical severities and phases of NA. Further basic research and confirmatory trials should be performed to survey the efficacy of such immunomodulation therapy for NA.

Pallidotomy for severe tardive jaw-opening dystonia.

Bilateral pallidotomy was performed in a schizophrenic patient with severe jaw-opening dystonia developed after chronic neuroleptic treatment. The dystonia led to sustained mandibular joint dislocation, and tracheotomy was performed after suffocation. The jaw-opening dystonia disappeared immediately following pallidotomy; the tracheotomy was closed, and he regained eating and speech ability. Analysis of the neuronal firing of the globus pallidus revealed low neuronal firing rates in the internal pallidum (GPi) and an irregular burst pattern of the GPi cells compared to those in Parkinson's disease. These results suggest that pallidotomy is a treatment option for tardive jaw-opening dystonia and that dystonia of this type is driven by abnormal neural activities in the GPi.

Calciphylaxis as a Catastrophic Complication in a Patient with POEMS Syndrome.

Calciphylaxis is a vascular calcification-cutaneous necrosis syndrome, usually seen in patients with end-stage renal disease and secondary hyperparathyroidism. We report a 57-year-old polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome patient complicated with extensive skin ulcers due to calciphylaxis. He first noted a painful cutaneous ulcer on his left thigh, and then skin lesions rapidly worsened, resulting in multiple intractable ulcers with gangrene on his legs and trunk in a few months. Serum vascular endothelial growth factor (VEGF) was markedly elevated. Biopsy samples from his skin ulcers showed the deposition of calcium in the medial layer of cutaneous vessels, this finding being compatible with calciphylaxis. This is the second reported case with POEMS syndrome complicated with calciphylaxis. Both patients had no evidence of renal failure, hyperparathyroidism, or clotting disorders. The pathogenic link between POEMS syndrome and calciphylaxis is still unclear, but VEGF is known to regulate vascular calcification, in cooperation with bone morphogenetic proteins. Further, corticosteroid and several proinflammatory cytokines activate nuclear factor-κB pathway, known as the final common pathway leading to vascular calcification. Taken together, we consider that POEMS syndrome can be an independent risk condition for calciphylaxis.

Cerebral amyloid angiopathy-related hemorrhage in a middle-aged patient with Down's syndrome.

A patient with Down's syndrome (DS) died of cerebral hemorrhage at age 52. At autopsy, a large sub-cortical hematoma was present in the right frontal lobe, and microscopic examination showed numerous senile plaques and neurofibrillary tangles in an extensive area of neocortex and also disclosed heavy involvement of vascular walls by amyloid deposition. These senile plaque and vascular amyloid deposits were specifically stained with an antibody to Abeta. His APOE genotype was epsilon4/epsilon4. This is a rare case of DS with cerebral amyloid angiopathy (CAA)-related cerebral hemorrhage. Genetic factors, such as APOE genotype, conceivably determine the risk of vascular rupture in individuals with CAA, even among patients with DS.

AL amyloidosis with spontaneous hepatic rupture: successful treatment by transcatheter hepatic artery embolization.

A 42-year-old male patient with primary AL amyloidosis experienced spontaneous hepatic rupture, producing diffuse widespread intrahepatic hemorrhage. Transcatheter hepatic artery embolization saved his life. Hepatic rupture infrequently develops in patients with systemic amyloidosis, but survival after this complication is rare because surgical treatments seldom succeed. Non-invasive emergent transcatheter embolization should be considered for hepatic rupture caused by massive deposits of amyloid.

Cortical petechial hemorrhage, subarachnoid hemorrhage and corticosteroid-responsive leukoencephalopathy in a patient with cerebral amyloid angiopathy.

We describe a 69-year-old woman who developed subacute onset cognitive decline after hitting the left side of her head. Cerebral spinal fluid showed yellowish discoloration with highly elevated protein content. FLAIR MRI revealed diffuse high signal intensity in all cortical sulci, and leptomeningeal enhancement in the left cerebral hemisphere was seen in the T1 image after contrast administration. She was treated with a corticosteroid. Consciousness disturbance was temporarily relieved but again worsened, resulting in an apathetic state due to communicating hydrocephalus. A shunt tube was placed in her right lateral ventricle. A brain biopsy disclosed multiple cortical microbleeds and heavy deposition of Abeta-immuoreactive amyloid on vascular walls. Inflammatory mononuclear cells surrounded a few leptomeningeal vessels. After the operation her condition further deteriorated and she fell into a coma. MRI showed diffuse swelling of the right cerebral white matter. She again received high-dose corticosteroid and gradually recovered during the following 2 months. On MRI the vast majority of abnormal signals in the right cerebral white matter disappeared. An initial manifestation of this patient was possibly caused by multiple microhemorrhages from fragile cortical and subarachnoid vessels with Abeta-amyloid deposition, which was triggered by head trauma. CAA-related inflammation possibly worsened this condition. Additionally, surgical intervention for communicating hydrocephalus might have induced cerebral amyloid angiopathy (CAA)-related leukoencephalopathy in her right cerebral hemisphere. These CAA-derived manifestations are unusual and high-dose corticosteroids seems to be useful for vascular events in CAA patients.

Klüver-Bucy syndrome following status epilepticus associated with hepatic encephalopathy.

Described here is the case of a patient with liver cirrhosis who developed bilateral temporo-occipital lobe lesions on MRI and Klüver-Bucy syndrome following status epilepticus. Herpes encephalitis, paraneoplastic syndrome, Hashimoto's encephalopathy, reversible posterior leukoencephalopathy syndrome, mitochondrial encephalomyopathy, lactic acidosis, and strokelike episode syndrome were judged not to be involved on the basis of laboratory results. The possible cause of the temporo-occipital lesions on MRI in this patient was cortical damage related mainly to status epilepticus and partially to coexisting hepatic encephalopathy.

Herpes simplex virus type-1 meningoencephalitis showing disseminated cortical lesions.

We report a 41-year-old man with meningoencephalitis associated with herpes simplex virus type 1 (HSV-1). The patient developed fever, headache and dysuria followed by generalized convulsion and neck stiffness, and the CSF showed pleocytosis. The titers of enzyme-linked immunosorbent assay against HSV measured 6 days after onset showed a significant rise; IgG antibody 4.89 (<0.2) and IgM antibody 1.45 (<0.8) in CSF, IgG antibody 46.1 (<2.0) and IgM antibody 1.76 (<0.8) in the serum. The antibody index for IgG was 0.50, and that for IgM was 4.2. CFS neutralization test showed HSV-1 antibody of x16 and HSV-2 antibody of

[Cervical myelopathy in a patient with congenital cervico-cerebral vascular malformation].

We report a 50 year-old woman with cervical myelopathy. The patient, who had cutaneous angiomas in the right orbital area, became aware of left upper limb weakness when she woke up, followed by painful abnormal sensation in both axilla and arms. MRI revealed an intramedullar lesion mainly located in cervical cord at the level of C3-C4. Angiography showed that serpentine left vertebral artery entered the canalis vertebralis at C3 and fed the blood flow of bilateral middle cerebral arteries. In this case, the upper cervical spinal cord ischemia might be induced by hemodynamic insufficiency of the anterior spinal artery ascribed to congenital cervico-cerebral vascular malformation.

Severe generalized dystonia as a presentation of a patient with aprataxin gene mutation.

A 14-year-old girl, homozygous for an insertion mutation of aprataxin (APTX), 689 ins T, is described. She presented with severe generalized dystonia, ataxia, ocular motor apraxia, and areflexia. The dystonia of this patient suggests involvement of the basal ganglia or thalamus, along with clinical diversity in this disorder.