Case Report: Dexmedetomidine for Intractable Clusters of Myoclonic Jerks and Paroxysmal Sympathetic Hyperactivity in Progressive Encephalomyelitis With Rigidity and Myoclonus.

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a severe form of stiff-person spectrum disorder characterized by painful spasms, myoclonic jerks, hyperekplexia, brainstem dysfunction, and dysautonomia, which is sometimes resistant to γ-amino-butyric acid (GABA)-ergic agents. The response to immunotherapy varies depending on identified autoantibodies. We report a dramatic response to dexmedetomidine in a patient with glycine receptor (GlyR) antibody-positive PERM who developed intractable clusters of myoclonic jerks and paroxysmal sympathetic hyperactivity (PSH) that was highly refractory to conventional symptomatic treatment with GABAergic drugs and immunotherapy.

Subdural haematoma, the great imitator, mimicking acute spinal cord lesion.

Acute non-traumatic paraparesis is usually caused by vascular, inflammatory or neoplastic myelopathies; however, it is sometimes caused by non-myelopathic pathologies, including polyradiculoneuropathies, myopathies, psychogenic aetiologies or parasagittal cortical pathologies. A 73-year-old woman reported weakness of the bilateral lower limbs and urinary incontinence. Together with the sensory level at the left T6 dermatome, we initially considered thoracic myelopathy as the most likely diagnosis.

Temporal Changes in Brain Magnetic Resonance Imaging Findings over 16 Years in a Patient with Neuronal Intranuclear Inclusion Disease.

Leukoencephalopathy with high-intensity signals in the corticomedullary junction on diffusion-weighted imaging (DWI) is a diagnostic hallmark for neuronal intranuclear inclusion disease (NIID). We herein report a 65-year-old man who developed dementia and was diagnosed with NIID 2 years later. Of note, he had coincidentally undergone brain magnetic resonance imaging 14 and 10 years before the onset of dementia.

A 10-year longitudinal study of deep white matter lesions on magnetic resonance imaging.

Purpose: Deep white matter lesions (DWMLs), T2 high-intensity areas in the subcortical white matter on magnetic resonance imaging (MRI), are a clinical phenotype of cerebral small vessel disease. Factors such as age and hypertension have been reported to significantly contribute to the presence and severity of DWMLs in cross-sectional studies. We herein report a 10-year longitudinal study on DWMLs in elderly Japanese subjects to reveal the clinical variables contributing to the progression of DWMLs.

Experimental infection of pigs with different doses of the African swine fever virus Armenia 07 strain by intramuscular injection and direct contact.

We experimentally infected pigs with the African swine fever virus (ASFV) Armenia 07 strain (genotype II) to analyze the effect of different dose injections on clinical manifestations, virus-shedding patterns, histopathology, and transmission dynamics by direct contact. Each three pigs and four pigs were injected intramuscularly with 0.1 fifty percent hemadsorbing doses (HAD)/ml, 10 HAD/ml and 10 HAD/ml of ASFV Armenia 07 strain, respectively.

Cerebral Infarction with Pulmonary Thromboembolism Due to Immobilization.

Arterial and venous thrombi can coexist without preexisting conditions, such as malignant disease, thrombotic predisposition, or arteriovenous shunt. We herein report a case of acute cerebral infarction and pulmonary thromboembolism in the absence of underlying disease. A 71-year-old woman presented with left hemiplegia.

Unusual Clinical Sequelae of Kawasaki Disease-Symptomatic Extracranial Internal Carotid Stenosis in Young Adult.

Background: Kawasaki disease (KD) is an acute systemic vasculitis that primarily affects the coronary artery, but it does not commonly affect the carotid artery. Cerebral infarction (CI) with internal carotid artery stenosis (ICS) in patients with KD has not been reported until now. We report a patient with CI as a remote-phase complication of KD.

Association between α-Klotho and Deep White Matter Lesions in the Brain: A Pilot Case Control Study Using Brain MRI.

Background: The anti-aging protein, α-Klotho, may be involved in cognitive decline and has potential as a surrogate marker that reflects dementia. However, the role of α-Klotho in the brain has not been sufficiently investigated.

Objective: Here, we investigated the association between α-Klotho and cognitive decline that is associated with cerebral deep white matter lesions (DWMLs).

Oral Cnm-positive Streptococcus Mutans Expressing Collagen Binding Activity is a Risk Factor for Cerebral Microbleeds and Cognitive Impairment.

Cerebral microbleeds (CMBs) are an important risk factor for stroke and dementia. We have shown that the collagen binding surface Cnm protein expressed on cnm-positive Streptococcus mutans is involved in the development of CMBs. However, whether the collagen binding activity of cnm-positive S.

Serum albumin to globulin ratio is related to cognitive decline via reflection of homeostasis: a nested case-control study.

Background: Recent research suggests that several pathogenetic factors, including aging, genetics, inflammation, dyslipidemia, diabetes, and infectious diseases, influence cognitive decline (CD) risk. However, no definitive candidate causes have been identified. The present study evaluated whether certain serum parameters predict CD.

CD62-mediated activation of platelets in cerebral white matter lesions in patients with cognitive decline.

Background: Vascular dementia is related to intracranial arteriosclerosis associated with deep white matter lesions (DWMLs). DWMLs have been linked to thrombogenesis due to sustained platelet activation; therefore, an accurate hematological marker is needed. This study was done to evaluate the usefulness of a new method to examine the function of activated platelets in order to assess DWMLs associated with cognitive decline.

Ventricular Temperatures in Idiopathic Normal Pressure Hydrocephalus (iNPH) Measured with DWI-based MR Thermometry.

Purpose: The brain produces intense heat as a result of cerebral metabolism and cerebral blood flow, and the generated heat is removed mainly through circulation of the intracranial blood vessels and cerebrospinal fluid (CSF). Because magnetic resonance (MR) images are constructed from analysis of the spin of various molecules, the diffusion coefficient can be used as a parameter that reflects the temperature of water molecules. We used diffusion-weighted imaging (DWI)-based MR imaging to measure the temperature of the CSF around the lateral ventricles in patients with idiopathic normal pressure hydrocephalus (iNPH).

The relationship between neurological worsening and lesion patterns in patients with acute middle cerebral artery stenosis.

Background: Intracranial atherosclerotic disease is one of the most common causes of ischemic stroke especially in Asians, Hispanics and blacks. Although middle cerebral artery (MCA) stenosis is increasingly being recognized with the advent of magnetic resonance angiography (MRA) or transcranial Doppler ultrasonography, few studies have focused on acute neurological worsening (NW) in patients with MCA stenosis. We investigated the relationship between NW and lesion patterns detected by diffusion-weighted imaging (DWI).

Characteristics of intracranial branch atheromatous disease and its association with progressive motor deficits.

Background: Small deep brain infarcts are often caused by two different vascular pathologies: 1. atheromatous occlusion at the orifice of large caliber penetrating arteries termed branch atheromatous disease (BAD) and 2. lipohyalinotic degenerative changes termed lipohyalinotic degeneration (LD).

A combined treatment for acute larger lacunar-type infarction.

Larger lacunar-type infarcts (LLIs), presumably caused by occlusion at the orifices or proximal portions of larger-caliber penetrating arteries by atheromatous plaque, are frequently associated with progressive motor deficits (PMD) and lead to poor functional outcome. This study was conducted to examine the efficacy of a combined treatment to prevent PMD or improve the functional outcome in patients with LLI. A total of 218 consecutive patients with LLI and motor lacunar syndrome were enrolled, including 138 patients with infarcts in the territory of the lenticulostriate artery and anterior choroidal artery (supratentrial group) and 80 patients with infarcts in the territory of the anterior pontine artery (pontine group).

The infarct location predicts progressive motor deficits in patients with acute lacunar infarction in the lenticulostriate artery territory.

Background And Purpose: Patients with acute lacunar infarction in the lenticulostriate artery (LSA) territory often show progression of motor deficits (PMD) after admission. The purpose of our study is to identify predictors for PMD using the findings of diffusion-weighted imaging (DWI) on admission.

Methods: From January 2005 to December 2008, we studied 60 consecutive patients with acute lacunar infarction in the LSA territory within 24h after onset.

Predictive factors for progressive motor deficits in penetrating artery infarctions in two different arterial territories.

Background: Progressive motor deficits (PMD) are common in cerebral penetrating artery disease (PAD) during the acute stage and leads to severe disability. Reliable predictors and stroke mechanism for PMD in PAD have been yet to be elucidated. Moreover, difference of predictors between topographically classified PAD has not ever been systematically studied.

Cognitive impairment and cerebral hypoperfusion in a CADASIL patient improved during administration of lomerizine.

A 64-year-old woman was admitted to our hospital for recurrent stroke and cognitive impairment and was diagnosed with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Iodine-123 iodoamphetamine single photon emission computed tomography showed hypoperfusion in the whole brain, but cerebral blood flow increased dramatically after the administration of acetazolamide in the cerebral cortex. Lomerizine, a diphenylmethylpiperazine Ca2+ channel blocker, can selectively increase cerebral blood flow.