Analysis of Relationships between Spinal Deformity and Walking Ability in Parkinson's Disease Patients.

INTRODUCTION: This study aimed to determine impacts on walking ability of spinal deformity and imbalance as distinct from movement disorders in Parkinson's disease (PD). METHODS: Thirty-two patients (15 males, 17 females; mean age 72.5 years) were analyzed. Three, thirteen, eleven, and five were at Hoehn-Yahr stages I, II, III, and IV, respectively. In addition to various spinal imbalance and deformity classifications the following were assessed: Cobb angle (CA) for scoliosis, thoracic kyphosis (TK) at T2-12, thoracolumbar kyphosis(TLK) at T12-L2, lumbar lordosis(LL) at L1-S1, pelvic tilt(PT), pelvic incidence(PI), and sagittal vertical axis(SVA). The Timed Up and Go (TUG) test was used to measure walking ability. Patients were evaluated using the Unified Parkinson's Disease Rating Scale (UPDRS) part III, and bone mineral density (BMD) scans. RESULTS: Nineteen patients (59%) had spinal deformity and imbalance within the following classifications: thoracic scoliosis, 1; thoracic kyphosis, 2; lumbar scoliosis, 15; Pisa syndrome, 3; camptocormia, 2. Mean values were 20.0° CA for scoliosis, 42.3° TK, 14.8° TLK, 26.7° LL, 20.8° PT, 48.8° PI, and 66.4 mm SVA. The mean TUG score was 13.9s. The UPDRS III mean was 36.6±24.5 points. Mean BMD was 0.856 g/cm2 at lumbar L2-4 and 0.585 g/cm2 at the femoral neck. UPDRS part III (P<0.001), LL (P<0.05), and femoral neck BMD (P<0.05) significantly correlated to TUG test results. CONCLUSIONS: Distinct from the movement disorders of PD (UPDRS III), loss of normal LL and loss of BMD at the femoral neck were shown to be correlated with diminished walking ability (TUG test) in PD patients. When UPDRS improved in response to L-dopa, walking ability improved. In addition to any PD-specific interventions that contribute to the maintenance of ambulation, interventions specific to the restoration of LL, as well as early treatment for osteoporosis may positively affect HRQOL in PD.

A study of brain metabolism in fibromyalgia by positron emission tomography.

PURPOSE: The aim of the present study was to determine the brain regions with altered metabolism in patients with treatment-naïve fibromyalgia (FM). METHODS: We used [18F] fluoro-d-glucose positron emission tomography to examine a total of 18 treatment-naïve FM patients and 18 age- and sex-matched healthy controls not suffering from pain. A voxel-by-voxel group analysis was performed using statistical parametric mapping. RESULTS: No significant voxel (peak)-level results were detected in this study; however, some regions were detected as significant-size clusters. There were no significant differences in brain metabolism between FM patients and controls. However, the right thalamus and left lentiform nucleus were hypermetabolic areas in FM patients with poor prognosis compared to the healthy controls. In contrast, the left insula and left lentiform nucleus were hypometabolic areas in FM patients with good prognosis compared to the healthy controls. Compared to FM patients with good prognosis, FM patients with poor prognosis showed significant hypermetabolism in the left thalamus, bilateral lentiform nucleus, and right parahippocampal gyrus. CONCLUSION: The present findings suggest an association between the metabolism in the thalamus, lentiform nucleus, and parahippocampal gyrus and prognosis in FM patients. Further study with a larger number of patients is required to confirm this association.

Cytomegalovirus-associated encephalomyelitis in an immunocompetent adult: a two-stage attack of direct viral and delayed immune-mediated invasions. case report.

BACKGROUND: It is clinically rare to find cytomegalovirus (CMV)-associated encephalomyelitis in immunocompetent adults. Here, we present the case of an adult patient who developed acute transverse myelitis that was followed by immune-mediated disseminated encephalomyelitis. CASE PRESENTATION: A 38-year-old man developed acute paraplegia with paresthesia below the level of the T7-8 dermatome. Both brain and spinal cord MRIs performed at admission appeared normal. Corticosteroid therapy was initiated, with the later addition of high-dose intravenous immunoglobulins. After polymerase chain reaction analysis indicated the presence of CMV DNA in his cerebrospinal fluid (CSF), anti-viral therapy was added. Forty days after symptom onset, despite an initial positive response to this therapy, he developed dysarthria and truncal ataxia. Repeated magnetic resonance imaging scans demonstrated progressively expanding lesions involving not only the spinal cord but also the cerebral white matter, suggestive of extensive immune-mediated demyelination involving the central nervous system (CNS), as is observed in acute disseminated encephalomyelitis (ADEM). CONCLUSION: This case report underscores the importance of careful patient observation following the initial diagnosis of a CMV-associated CNS infection, such as transverse myelitis, on the possibility that post-infectious ADEM may appear.

Cerebrospinal fluid dissemination of anaplastic intraventricular meningioma: report of a case presenting with progressive brainstem dysfunction and multiple cranial nerve palsies.

BACKGROUND: It is extremely rare to see cerebrospinal fluid dissemination of intraventricular meningioma, particularly with the development of acute, progressive brainstem/cerebellar dysfunction with an absence of mass formation in the corresponding anatomical sites. CASE PRESENTATION: An 81-year-old man was admitted because of double vision, right facial nerve palsy and truncal ataxia. Brain magnetic resonance imaging showed normal findings except for a tumor mass in the left lateral ventricle, which had been noted over 6 months previously. The patient developed hiccups, hyperventilation, and drowsiness, which worsened progressively, and did not respond to corticosteroid or intraventricular immunoglobulin therapy. Cerebrospinal fluid study revealed a mild elevation of protein, and cytology was negative. The patient died and an autopsy was performed. Postmortem investigation disclosed a malignant transformation of benign fibroid meningioma with cerebrospinal fluid dissemination of the malignant cells, diversely involving the surface of brainstem, cerebellum, and spinal cords, secondarily resulting in extensive ischemia in the brain parenchyma by vessel occlusion. CONCLUSION: If a patient with an intraventricular tumor develops acute, progressive neurological symptoms, the possibility that it is be caused by cerebrospinal fluid dissemination of tumor cells, after malignant transformation, should be considered.

A randomized double-blind multi-center trial of hydrogen water for Parkinson's disease: protocol and baseline characteristics.

BACKGROUND: Our previous randomized double-blind study showed that drinking hydrogen (H2) water for 48 weeks significantly improved the total Unified Parkinson's Disease Rating Scale (UPDRS) score of Parkinson's disease (PD) patients treated with levodopa. We aim to confirm this result using a randomized double-blind placebo-controlled multi-center trial. METHODS: Changes in the total UPDRS scores from baseline to the 8(th), 24(th), 48(th), and 72(nd) weeks, and after the 8(th) week, will be evaluated. The primary endpoint of the efficacy of this treatment in PD is the change in the total UPDRS score from baseline to the 72(nd) week. The changes in UPDRS part II, UPDRS part III, each UPDRS score, PD Questionnaire-39 (PDQ-39), and the modified Hoehn and Yahr stage at these same time-points, as well as the duration until the protocol is finished because additional levodopa is required or until the disease progresses, will also be analyzed. Adverse events and screening laboratory studies will also be examined. Participants in the hydrogen water group will drink 1000 mL/day of H2 water, and those in the placebo water group will drink normal water. One-hundred-and-seventy-eight participants with PD (88 women, 90 men; mean age: 64.2 [SD 9.2] years, total UPDRS: 23.7 [11.8], with levodopa medication: 154 participants, without levodopa medication: 24 participants; daily levodopa dose: 344.1 [202.8] mg, total levodopa equivalent dose: 592.0 [317.6] mg) were enrolled in 14 hospitals and were randomized. DISCUSSION: This study will confirm whether H2 water can improve PD symptoms. TRIAL REGISTRATION: UMIN000010014 (February, 13, 2013).

Good's syndrome with opportunistic infection of the central nervous system: a case report.

BACKGROUND: Immunodeficiency with a thymoma (Good's syndrome) is a rare condition occurring in patients with adult-onset hypogammaglobulinemia that is progressive after the removal of thymoma. Recently, we encountered a patient with Good's syndrome who suddenly developed opportunistic encephalitis 4 years after the resection of thymoma without a history of infectious complications. CASE PRESENTATION: A 58-year-old man, who underwent surgery to remove a thymoma at the age of 54, was admitted because of speech difficulties. A brain MRI showed multiple lesions involving the frontal lobes, but the CSF finding was normal. Acyclovir was empirically administered, and fever as well as his neurological symptoms fully recovered within a few days. However, 1 week after admission, motor aphasia and mild right hemiparesis reappeared. MRI showed that the lesion involving the left cingulate gyrus expanded in size, and revealed an abnormal signal intensity lesion in the left corona radiata. Laboratory examination found increased CMV pp65 antigen-positive lymphocytes in serum. Antiviral therapy using ganciclovir and immunoglobulin replacement therapy was started. The patient has since been free from any neurological symptoms for 1 year, and lesions demonstrated by MRI are gradually improving. CONCLUSION: Early recognition of this rare condition and prompt initiation of therapy are crucially important. Awareness of immunodeficiency in a patient after removal of thymoma may help neurologists to consider the possibility that opportunistic infection may be the cause of cerebral lesions.

Palliation of malignant tracheal stenosis with a second implantation of an expandable metallic stent under endotracheal intubation.

A 74-year-old man was admitted with respiratory failure and treated for tracheobronchial stenosis due to metastasis of renal cell carcinoma. It improved after implantation of an expandable metallic stent (EMS). One year later, the metastatic tumor at the near distal side of the EMS increased; eventually serious respiratory failure occurred again. However, the delivery catheter of EMS could not be inserted by the usual procedure because there was a strong tracheobronchial curve. Finally it passed along the inside of the endotracheal intubation tube. The respiratory failure was improved by the second implantation of EMS with the method of stent in stent. EMS is often effective in a case with a strong curve and twist of the trachea/bronchi. It was also considered one way of letting the delivery catheter pass inside the endotracheal intubation tube if the patient's respiratory condition was maintained.

Zonisamide reduces cell death in SH-SY5Y cells via an anti-apoptotic effect and by upregulating MnSOD.

Zonisamide, originally known as an antiepileptic drug, has been approved in Japan as adjunctive therapy with levodopa for the treatment of Parkinson's disease (PD). Although zonisamide reduces neurotoxicity, the precise mechanism of this action is not known. Here, we show that zonisamide increases cell viability in SH-SY5Y cells via an anti-apoptotic effect and by upregulating levels of manganese superoxide dismutase (MnSOD). These results would give us novel evidences of PD treatment.

[Reactivation of Epstein-Barr virus is involved in the pathogenesis of lymphomatoid granulomatosis].

Recent studies point to a role of Epstein-Barr virus in the pathogenesis of lymphomatoid granulomatosis. Little is however known about the association of the pathogenesis of the disease with Epstein-Barr virus reactivation. A 46-year-old man presented with fever and general malaise. He had been admitted with fever of unknown origin 10 years previously. Chest radiographs and CT scans showed multiple nodular shadows in both lung fields. PET/CT showed strong accumulation in lung nodules, femoral muscle, lumbar subcutaneous nodule. He died of rapidly progressive neurological lesions. The definitive diagnosis of lymphomatoid granulomatosis was made from histological findings of the surgical biopsy specimen and at autopsy. Elevation of early antigen-IgG titer in serum was shown in this case. This result suggests that reactivation of Epstein-Barr virus is involved in the development of the disease.

Common anti-apoptotic roles of parkin and alpha-synuclein in human dopaminergic cells.

Parkin, a product of the gene responsible for autosomal recessive juvenile parkinsonism (AR-JP), is an important player in the pathogenic process of Parkinson's disease (PD). Despite numerous studies including search for the substrate of parkin as an E3 ubiquitin-protein ligase, the mechanism by which loss-of-function of parkin induces selective dopaminergic neuronal death remains unclear. Related to this issue, here we show that antisense knockdown of parkin causes apoptotic cell death of human dopaminergic SH-SY5Y cells associated with caspase activation and accompanied by accumulation of oxidative dopamine (DA) metabolites due to auto-oxidation of DOPA and DA. Forced expression of alpha-synuclein (alpha-SN), another familial PD gene product, prevented accumulation of oxidative DOPA/DA metabolites and cell death caused by parkin loss. Our findings indicate that both parkin and alpha-SN share a common pathway in DA metabolism whose abnormality leads to accumulation of oxidative DA metabolites and subsequent cell death.