Calcified Cerebral Embolism Due to a Calcified Amorphous Tumor.

A 59-year-old man developed brain embolism in the frontal and parietal cortex. Brain CT showed a high-density spot in the upper branch of the left middle cerebral artery, indicating calcified cerebral embolism. Calcified amorphous tumor attached to the mitral valve was identified as the cause of embolism. After surgical resection, anticoagulation was started and recurrent stroke did not occur.

Gait disturbance due to foot drop is refractory to treatment in nonsystemic vasculitic neuropathy.

BACKGROUND: Nonsystemic vasculitic neuropathy (NSVN) is a vasculitis syndrome clinically restricted to the peripheral nervous system. Although treatment may improve prognosis, daily activities of such patients after treatment have not been well studied. METHODS: We evaluated clinical features, laboratory data, nerve conduction, and sural nerve biopsy findings for 16 unbiased consecutive patients with NSVN. RESULTS: Initial symptoms included neuropathic pain (31%) and lower limb sensory disturbance (19%). The mean duration between disease onset and initial treatment was 4.1 ± 4.8 months. Mean modified Rankin scale scores were 3.13 at hospital admission and 2.69 at final follow-up. The poor outcome group had significantly decreased compound muscle action potentials of peroneal nerves and significantly more patients presenting with foot drop compared with the good outcome group. No other significant differences were found. CONCLUSION: Pretreatment foot drop signaled poor outcome in daily activities of patients with NSVN, and earlier treatment may be critical for these patients.

Questionnaire survey on pharmacists' roles among non- and health care professionals in medium-sized cities in Japan.

Although the scope of pharmacists' work has expanded in Japan, people's perception of this is unclear. To contribute to medical care together with non- and health care professionals, clarifying the perceptions of these groups is important to best utilize pharmacist professionals. We conducted a cross-sectional questionnaire survey among non-health care professionals (n = 487) and nurses (n = 151), medical doctors (n = 133), and pharmacists (n = 204) regarding the work of pharmacists. The questionnaire comprised 56 items in four categories associated with the roles of pharmacists. For each questionnaire item, we performed logistic regression analysis to compare pharmacists' opinions with those of other professionals and non-health care professionals. Opinions were similar between pharmacists and nurses or medical doctors regarding "collecting patient information" and "providing drug information to patients." However, there were differences in perceptions regarding "medical collaboration" (nurses; 8/23 items, physicians; 11/23 items) and "community medicine" (nurses; 9/15 items, physicians; 11/15 items), and pharmacists themselves perceived greater roles related to health care collaboration and community health care. Perceptions of non-health care professionals were poorer than those of pharmacists in all categories (47/56 items). These results suggest that pharmacists must actively communicate to help others understand their specialty and build trusting relationships to improve patient care.

Long-term outcome of polymyositis treated with high single-dose alternate-day prednisolone therapy.

BACKGROUND: We previously reported no difference in the efficacies of high-dose alternate-day (ADT) and daily-dose (DDT) prednisolone therapies in myositis patients, but that the incidence of side effects was lower in the former. The aim of the present study was to compare the long-term outcomes of both treatments in polymyositis patients. METHODS: We compared clinical courses, efficacies, adverse reactions, and outcomes of 115 consecutive, biopsy-proven polymyositis patients treated between 1970 and 2008 with ADT (32 patients) or DDT (83 patients). RESULTS: Mean onset ages, disease severity, incidences of malignancy, and response rates did not differ between the ADT and DDT groups. Adverse reactions (incidence of diabetes) were significantly higher in the DDT group. In this group, the incidences of hyperlipidemia, infection, hypertension, and psychiatric symptoms were also slightly higher, but not significantly so. The 20-year survival rate of the ADT group (68%) was significantly higher (p = 0.0112) than that of the DDT group (37%). CONCLUSION: ADT might be useful as an initial treatment option for polymyositis.

Amyotrophic lateral sclerosis in a patient with Kartagener syndrome.

We present a case of a patient with clinically definite ALS, who had earlier suffered from Kartagener syndrome, which is characterized by the triad comprising chronic sinusitis, bronchiectasis, and situs inversus. Recent linkage and mutational analyses identified several genes that are responsible for Kartagener syndrome. Most of them encode subunits of axonemal dyneins, highlighting the importance of dynein motors to ciliary motility. Recent data indicate that defects in cytoplasmic dynein-mediated retrograde axonal transport are involved in the etiology of ALS. Genes encoding the dynein heavy chain of cytoplasmic and outer arm axonemal dyneins are reported to have similar sequences in their central and 3'-end regions. Although a causal link between ALS and Kartagener syndrome has not yet been definitely established, the precise relationship between disrupted axonemal dynein function in Kartagener syndrome and motor neuron death should be investigated.

[A case of dural arteriovenous fistula associated with bilateral thalamic lesions].

We report a 51-year-old man with a dural arteriovenous fistula (DAVF) associated with bilateral thalamic lesions. He was admitted to our hospital because of cognitive disorder. T2-weighted MRI and fluid-attenuated inversion recovery (FLAIR) sequence of the brain revealed symmetric hyperintense lesions of bilateral thalamus and abnormal flow void that represents the enlarged veins. Cerebral angiography demonstrated DAVF in the superior petrosal sinus (SPS). It was mainly supplied by the internal carotid arteries. The strait sinus was not revealed, and the venous drainage was retrograde into the internal cerebral vein. Therefore the mechanism of cognitive disorder in this case was considered to be vasogenic edema of the bilateral thalamus due to DAVF of SPS. We decided to treat the DAVF by embolization via the feeding arteries approach, because strait sinus was not revealed and venous approach was difficult. After embolization, the size of DAVF was remarkably reduced. His cognitive disorder was markedly improved and the hyperintense area on T2-weighted MRI and FLAIR sequence had disappeared. Cognitive disorder due to DAVF of SPS is very rare. It is also difficult to diagnose bilateral thalamic lesions as DAVF, but it may be reversible by DAVF treatment. Thus, early diagnosis and treatment is important. Like this case, abnormal flow void that represents the enlarged veins could help to diagnose bilateral thalamic lesions due to DAVF.

Perfusion abnormality on three-dimensional arterial spin labeling with a 3T MR system in pediatric and adolescent patients with migraine.

PURPOSE: Few studies have assessed the prevalence of perfusion abnormality with migraine. This study aimed to determine the prevalence and topography of perfusion abnormality on three-dimensional (3D) arterial spin labeling (ASL) and assess the correlation between perfusion abnormality and clinical data in pediatric and adolescent patients with migraine. METHODS: Forty-nine consecutive pediatric and adolescent patients with migraine were enrolled, and they underwent 3 T MRI, including 3D ASL. Perfusion abnormality on 3D ASL was qualitatively evaluated using a five-point grading system and was compared with non-ASL MR findings. In patients with perfusion abnormality, relative cerebral perfusion signal intensity (rCPS) was measured. Moreover, we compared clinical data and 3D ASL findings between patients with and those without perfusion abnormality. RESULTS: Of the 49 patients, 11 (22%) exhibited perfusion abnormality, and the occipital lobe was the most frequently involved (73%). One patient showed mild hyperperfusion (rCPS =2.474), and 10 showed hypoperfusion (mean rCPS = 0.405 ±â€¯0.134). There was no abnormality on non-ASL MRI, except in one case. We found statistically significant differences in the presence of aura (P < .001), motor disabilities (P = .019), confusion (P = .004), hospitalization (P = .004), between patients with and those without perfusion abnormality. CONCLUSION: In pediatric and adolescent patients with migraine, 3D ASL shows a high prevalence of perfusion abnormality, especially in the occipital lobe. Patients with perfusion abnormality tend to show the specific clinical symptoms at disease onset and need hospitalization.

Usefulness of intraventricular infusion of antifungal drugs through Ommaya reservoirs for cryptococcal meningitis treatment.

INTRODUCTION: Cryptococcal meningitis is a severe infection among immunosuppressed individuals, with a high mortality rate. Although amphotericin B is the first-choice drug for treatment, its use is restricted when adverse effects are clinically problematic. The usefulness of intraventricular infusion of antifungal drugs through Ommaya reservoirs for cryptococcal meningitis treatment has been unconfirmed. We evaluated the efficacy of intraventricular infusion of amphotericin B through Ommaya reservoirs. MATERIALS AND METHODS: We retrospectively analyzed 10 consecutive patients with cryptococcal meningitis who were refractory to systemic administration of antifungal drugs. RESULTS: Fever or nausea occurred in most patients. However, no patient complained of serious complications such as renal toxicity. Seven patients recovered completely or partially, whereas three patients died. CONCLUSIONS: To establish the efficacy of the intraventricular infusion of antifungal drugs through Ommaya reservoirs for cryptococcal meningitis, a prospective investigation should be designed to compare those treated according to the updated guidelines and those treated with antifungal drugs through the Ommaya reservoirs.

Clinical features of Japanese patients with inclusion body myositis.

BACKGROUND: The incidence of sporadic inclusion body myositis (sIBM) has been much lower in Japanese than in Western populations. Because of a few reports on Asian populations, it is unclear whether the clinical characteristics of sIBM are identical in Caucasian and Japanese patients. METHODS: We compared 18 patients with sIBM, divided into 3 groups by age-of-onset, with previous cohort studies. We calculated the ΔIBM functional rating scale/time duration (ΔIBMFRS/Δtime) as an index of functional disability progression. Patients' electrophysiology was analyzed in relation to their clinical characteristics. RESULTS: The cohort was 83.3% male and showed uniform initial muscle weakness in the lower and/or upper limbs. An older age-at-onset was associated with a more rapid progression, and patients with a longer duration frequently showed F-wave abnormalities and findings of chronic denervation. CONCLUSIONS: The clinical characteristics of sIBM were relatively homogeneous beyond the ethnic differences. Aging might be a synergistic factor for the progression of sIBM pathology.

Coexistence of Amyotrophic Lateral Sclerosis and Myasthenia Gravis.

The mechanisms by which amyotrophic lateral sclerosis (ALS) causes motor neuron degeneration remain unknown. We present the case of a 77-year-old Japanese female with clinically probable ALS, who developed ALS symptoms 41 years after onset of myasthenia gravis (MG). We concluded that neither the relapse of MG nor the adverse effects of anti-cholinesterase medication aggravated her symptoms. Although MG and ALS are extremely rare, we reviewed several case reports describing their coexistence. We suggest that clinicians should consider the possibility of ALS occurring with MG. Further investigations will improve our understanding of the pathogenic relationship between ALS and MG.

Drastic therapy for listerial brain abscess involving combined hyperbaric oxygen therapy and antimicrobial agents.

BACKGROUND: Listeria monocytogenes (L. monocytogenes) is a rare causative pathogen of brain abscess that is often found in immunocompromised patients. Although patients with supratentorial listerial abscesses showed a longer survival with surgical drainage, the standard therapy for patients with subtentorial lesions has not been established. CASE REPORT: We report herein a patient with supra- and subtentorial brain abscesses caused by L. monocytogenes infection. These abscesses did not respond to antibiotics, and his symptoms gradually worsened. Drainage was not indicated for subtentorial lesions, and the patient was additionally treated with hyperbaric oxygen therapy, which dramatically reduced the volume of abscesses and improved the symptoms. CONCLUSIONS: This is the first report of drastic therapy for a patient with listerial brain abscesses involving combined antibiotics and hyperbaric oxygen therapy. The findings suggest that hyperbaric oxygen therapy is a good option for treating patients with deep-seated listerial abscesses and for who surgical drainage is not indicated.

Bilateral basal ganglia lesions as initial manifestation of CNS invasion in adult T-cell leukemia.

We describe the case of a 67-year-old man who exhibited unsteadiness in walking, topographical disorientation, and urinary incontinence. Neurological examination revealed somnolence and mild weakness in the lower limbs with slight rigidity in the upper limbs. Cerebrospinal fluid examination showed pleocytosis with "flower cells" and an extremely high level of soluble interleukin-2 receptor. T2-weighted brain imaging revealed symmetrical high-intensity lesions in the bilateral caudate putamen. Positron emission tomography demonstrated intense uptake of 2-[fluorine-18]-fluoro-2-deoxy-d-glucose in the same region. He was diagnosed with central nervous system invasion by adult T-cell leukemia (ATL) and received chemotherapy. Interestingly, chemotherapy ameliorated the lesions and terminally caused the gray matter itself to atrophy in the bilateral caudate nuclei, which may be evidence for the direct infiltration of ATL tumors.

Muscle biopsy findings predictive of malignancy in rare infiltrative dermatomyositis.

OBJECTIVE: The characteristic pathological muscular findings of polymyositis (PM) and dermatomyositis (DM) have been shown to reflect their different pathogeneses. Here, we characterized the muscle biopsy findings of PM and DM patients with or without malignancy. METHODS: We evaluated the muscle biopsy findings of 215 consecutive PM and DM patients admitted to our hospital between 1970 and 2009. Pathology of the lesion biopsy sections was classified into 3 types: endomysial infiltration-type, perivascular infiltration-type, and rare-infiltrative-type. RESULTS: There was no difference between the muscle pathology of PM patients with and without malignancy. However, the incidence of rare-infiltrative type muscle pathology in DM patients with malignancy was significantly higher than in those without such tumors (p=0.0345). CONCLUSION: The incidence of rare-infiltrative type muscle pathology may be a predictive marker of DM with malignancy.

Fulminant myelopathy following neurogenic proximal weakness associated with human T-cell lymphotropic virus type I infection.

We report a patient with human T-cell lymphotropic virus type I (HTLV-I) infection, who presented with proximal extremity neurogenic muscular weakness followed by fulminant myelopathy, but with no upper motor symptoms. The symptoms were inconsistent with the World Health Organization or El Escorial criteria for HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) or amyotrophic lateral sclerosis (ALS). This case indicates that fulminant myelopathy without upper motor neuronal symptoms may occur long after the onset of HTLV-I-associated neurogenic proximal muscular weakness. Additionally, we report that treatment with high-dose steroid pulse therapy partially improves symptoms of lightning pain and sensory disturbance.

A novel GJB1 frameshift mutation produces a transient CNS symptom of X-linked Charcot-Marie-Tooth disease.

X-linked Charcot-Marie-Tooth disease (CMT1X) is the second most common variant of CMT and is caused by mutations in the GJB1 gene encoding connexin 32. Some CMT1X patients with GJB1 missense mutations have shown transient central nervous system (CNS) symptoms with abnormal brain magnetic resonance imaging (MRI). Herein we report the first case with a novel GJB1 frameshift mutation that associates with a transient CNS symptom. The patient noticed high-arched feet and limited ankle dorsiflexion in early childhood; he transiently developed numbness and paresis of left face and arm, and dysphagia, with abnormal brain MRI. Although the CNS symptoms recovered within several hours without treatment, intravenous immunoglobulin (IVIg) therapy ameliorated progressing symptoms such as those of toe extensor muscles. His mother had been diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP), and repetitive IVIg treatments had relieved the symptoms. Therefore, inflammation might be involved in the pathophysiology of CMT1X with the GJB1 mutation, while molecular analysis revealed that the mutant GJB1 was more rapidly degraded by the proteasome pathway known as endoplasmic reticulum (ER)-associated degradation.

A reversible lesion of the corpus callosum splenium with adult influenza-associated encephalitis/encephalopathy: a case report.

INTRODUCTION: Influenza virus-associated encephalitis/encephalopathy is a severe childhood illness with a poor prognosis. Adult case reports are rare and, to date, there have been no reports of adults with a mild subcortical encephalopathy with reversible lesions of the corpus callosum splenium. CASE PRESENTATION: A previously healthy 35-year-old man presented with acute progressive tetraplegia, transcortical motor aphasia and a mild decrease in his consciousness during his recovery after receiving oseltamivir phosphate treatment, and influenza type A antiviral medication. The initial magnetic resonance imaging study at day 1 showed symmetrical diffuse lesions in the white matter and a lesion on the central portion of the corpus callosum splenium. These findings had resolved on follow-up studies at day 8 and day 146. His neurological deficits mostly recovered within 12 hours following methylprednisolone pulse therapy. The levels of interleukin-6 and interleukin-10 in his blood and cerebrospinal fluid were initially elevated, but rapidly decreased to normal levels by day 8. CONCLUSION: It is important for clinicians to recognize that even in adulthood, the subcortical encephalopathy observed during the therapeutic treatment for influenza type A infection can occur in conjunction with a reversible lesion of the corpus callosum, which may recover quickly. In addition, the cytokine storm in the blood system and the corticospinal cavity may play an important role in the etiology of the disease process.