[An autopsy case of polyarteritis nodosa accompanied with multiple immune-specific autoantibodies and rhabdomyolysis].

A 72-year-old male complained of fever lasting 1 month and developed muscle weakness and paresthesia in the legs. He presented with muscle weakness, grasping pain, decreased deep tendon reflexes in the extremities, and reduction of tactile sensation in the distal parts of the left leg muscles. Blood tests revealed leukocytosis and inflammatory reactions. Collagen-disease-specific autoantibodies including anti-double-stranded DNA and anti-Scl-70 antibodies were positive, but antineutrophil cytoplastic antibodies were negative. Nerve conduction studies revealed asymmetric axonal degeneration, indicating multiple mononeuropathy. We started intravenous methylprednisolone pulse and plasma exchange therapies. However, the patient developed intestinal necrosis and perforation, and he died 44 days after the onset of fever. An autopsy revealed vasculitis in small- to medium-sized vessels in multiple organs as well as myoglobin casts in the renal tubules, which were suggestive polyarteritis nodosa (PAN) accompanied with rhabdomyolysis. Positivity for collagen-disease-specific autoantibodies and accompanying rhabdomyolysis are atypical findings with PAN. This patient was not clinically diagnosed as PAN, and so promptly starting immunotherapies should be considered when a case presents with evidence of vasculitis.

Oxytocin treatment improves dexamethasone-induced depression-like symptoms associated with enhancement of hippocampal CREB-BDNF signaling in female mice.

AIMS: Chronic stress and glucocorticoid exposure are risk factors for depression. Oxytocin (OT) has been shown to have antistress and antidepressant-like effects in male rodents. However, depression is twice as common in women than in men, and it remains unclear whether OT exerts antidepressant-like effects in women with depression. Therefore, in this study, we investigated the therapeutic effect of chronic OT administration in a female mouse model of dexamethasone (DEX)-induced depression. METHODS: Female C57BL/6J mice were administered saline (vehicle, s.c.), DEX (s.c.), or OT (i.p.) + DEX (s.c.) daily for 8 weeks, and then assessed for anxiety- and depression-like behaviors. We also examined the hippocampal levels of phosphorylated cAMP response element-binding protein (p-CREB) and brain-derived neurotrophic factor (BDNF), which are important mediators of the response to antidepressants. RESULTS: Simultaneous OT treatment blocked the adverse effects of DEX on emotional behaviors. Furthermore, it upregulated p-CREB and BDNF in the hippocampus. CONCLUSION: OT may exert antidepressant-like effects by activating hippocampal CREB-BDNF signaling in a female mouse model of depression.

Voriconazole treatment of pulmonary mycosis caused by Chrysosporium zonatum after treatment for pulmonary tuberculosis.

Chrysosporium zonatum is a soil-dwelling fungus that rarely causes pulmonary infections, and a small number of cases have been reported to date. A 74-year-old man, who had previously been treated for tuberculosis, presented with symptoms of low-grade fever, anorexia, cough, and bloody sputum. Chest computed tomography (CT) showed a thick-walled cavitary lesion in the right upper lobe, in which there was a suspected mycotic mass. Initially, the patient was suspected to have chronic aspergillosis due to positive serum anti-Aspergillus antibodies. However, bronchoscopic culture revealed the growth of C. zonatum. Symptoms and imaging findings improved with administration of voriconazole for 18 months. Infection by C. zonatum is very rare and is difficult to differentiate from aspergillosis by clinical features. Clinicians should be aware of the possibility of coinfection with C. zonatum and Aspergillus sp. Voriconazole may be an effective treatment option.

[Hemichorea in a patient with acute cerebral infarction of the somatosensory cortex].

A 86-year-old woman with left hemiparesis was admitted to our hospital. When visiting to our hospital, hemichorea appeared on her left extremities in an ambulance. She also had mild disturbance of consciousness, spatial disorientation, and sensory disturbance. Blood biochemical studies revealed mild renal failure. DWI MRI showed hyperintensities in the postcentral gyrus and a posterior part of the insula in the right hemisphere, but no signal changes in FLAIR. No lesions were detected in the basal ganglia. The DWI-FLAIR mismatch suggested acute cerebral infarction, and we performed intravenous thrombolysis therapy. Her neurological symptoms including hemichorea gradually improved, and she was finally discharged on foot. Two conspicuous points of the present patient are the sensory cortical infarction and an association with renal failure. In this patient, the sensory cortical infarction must produce chorea even though sensory cortical lesions rarely caused chorea. The associated renal dysfunction may play some role in the production of chorea. The double-crash of cerebral infarction and metabolic abnormality (renal dysfunction) may cause hemichorea which is rarely seen in patients with cerebral infarction of the sensory cortex and insula with no metabolic abnormalities.

[Successful early treatment with acyclovir and corticosteroids for acute myelitis associated with zoster sine herpete: a case report].

A 79-year-old man presented with chest and back pain on the right side but with no cutaneous lesions. He had received oral corticosteroids and immunosuppressants for systemic lupus erythematosus. He had spastic paraplegia, sensory disturbance in the lower limbs, and dysfunction of the bladder and bowel. He showed mononuclear-dominant pleocytosis and elevated proteins in the cerebrospinal fluid (CSF), and a decreased CSF/blood glucose ratio. Although polymerase chain reaction techniques found no varicella-zoster virus (VZV) DNA, VZV IgG antibodies were elevated in both the serum and CSF, and the VZV IgG index was dramatically elevated. MRI revealed no lesions in the brain or spine. However, somatosensory evoked potentials in the tibial nerve showed abnormal prolongation of the central sensory conduction time. We diagnosed the patient with acute myelitis associated with zoster sine herpete (ZSH). He received acyclovir and intravenous methylprednisolone pulse therapy in the early stage, and his symptoms and CSF findings completely recovered. We conclude that acute myelitis associated with ZSH should be treated as soon as possible because VZV infection may induce necrotizing myelitis if the treatment is delayed.

A Single-institution Study on Predictors of Short-term Progression from Mild Cognitive Impairment in Parkinson's Disease to Parkinson's Disease with Dementia.

BACKGROUND: Patients with non-demented Parkinson's disease (PD) sometime have mild cognitive impairment (MCI), and mild cognitive impairment in Parkinson's disease (PD-MCI) may convert to Parkinson's disease with dementia (PDD) within several years. Cognitive impairment also occurs in the early stages of the disease, gradually progressing to lower quality of life and instrumental activities of daily living. It is important to elucidate the predictors of progression from PD-MCI to PDD via longitudinal studies. METHODS: This was a single center, case-control study. We analysed data from 49 patients with PD-MCI diagnosed as level I using the Movement Disorder Society PD-MCI criteria at baseline who had completed 1.5 years of follow-up. We defined patients who progressed to PDD as patients with progressive PD-MCI and patients who did not progress to PDD as patients with non-progressive PD-MCI. Depression, apathy, sleep disorders, constipation, light-headedness, hallucinations, impulse control disorders (ICDs) and impulsive-compulsive behaviors (ICBs) at baseline were statistically analysed as predictors of progression. RESULTS: Of the 49 PD-MCI patients, 33 did not convert to PDD (non-progressive PD-MCI), and 16 converted to PDD (progressive PD-MCI). The Mini-Mental State Examination (MMSE) score, light-headedness and ICDs were elucidated as predictors of progressive PD-MCI via a multivariate logistic regression model. The adjusted odds ratios (ORs) and 95% confidence intervals (CIs) for each item were MMSE score, OR 0.324, 95% CI 0.119-0.882, P = 0.027; light-headedness, OR 27.665, 95% CI 2.263-338.185, P= 0.009; and ICDs, OR 53.451, 95% CI 2.298-291.085, P = 0.010. CONCLUSION: Cognitive function, ICDs and light-headedness may be risk factors for the development of PDD in PD-MCI patients.

Longitudinal course of mild parkinsonian signs in elderly people: A population-based study in Japan.

We aimed to clarify the longitudinal course of mild parkinsonian signs (MPS) and their association with dementia and functional disability by conducting a comprehensive epidemiological study, including brain MRI, and assessments of cognition, depression, and sleep, in people aged ≥65years living in Ama-cho. We diagnosed MPS and parkinsonism (PS) using a modified Unified Parkinson's Disease Rating Scale. The phase I study was conducted between 2008 and 2010 (n=729) and the phase II between 2011 and 2013 (n=436). By phase II, 8.5% of the phase I participants without PS had developed PS. In addition to older age, a lower Mini-Mental State Examination (MMSE) score, and lower body mass index, the MPS rigidity subtype was a significant independent predictor of PS onset. By phase II, 10.1% of the participants without dementia or PS at phase I had developed dementia. Older age, lower MMSE score, and the axial dysfunction and tremor MPS subtypes were significant independent predictors of dementia development. By phase II, 38.8% of participants with MPS at phase I showed no motor symptoms. Younger age and adequate sleep were significant predictors for this reversion. Periventricular and deep white matter hyperintensity Fazekas scores increased with the evolution of parkinsonian signs. MPS is therefore critically, although sometimes reversibly, associated with PS and dementia development in elderly people.

Screening tools for clinical characteristics of probable REM sleep behavior disorder in patients with Parkinson's disease.

INTRODUCTION: The REM sleep behavior disorder (RBD) screening questionnaire (RBDSQ) has been used as a screening tool for RBD. We investigated the clinical characteristics of probable RBD (pRBD) using the RBDSQ in patients with Parkinson's disease (PD). METHODS: Seventy patients with PD (age: 69.2 ± 8.9 years old, 31 males and 49 females, length of PD morbidity: 7.4 ± 6.4 years, Hoehn and Yahr: 2.7 ± 0.8) underwent examination including the RBDSQ and Mini-Mental State Examination (MMSE) in both 2011 and 2013. We assessed the changes and characteristics of pRBD associated with PD during the two year interval. RESULTS: Nineteen patients (27.1%) in 2011 and 27 patients (38.6%) in 2013 were diagnosed as having pRBD because they scored 6 or higher on the RBDSQ. During the 2 year interval, twelve patients showed persistent pRBD, 15 developed pRBD, and 7 showed improved pRBD. In 2013, PD patients with pRBD took higher amounts of levodopa equivalents and scored lower on the MMSE than those without pRBD. Also, more PD patients with pRBD had dementia than those without pRBD. Similarly, more patients with persistent and developed pRBD had dementia than those without. CONCLUSION: We found that RBD symptoms might fluctuate during the clinical course of PD, and RBD symptoms might temporarily affect cognitive impairment.

Clinical evaluation of fatigue in Japanese patients with Parkinson's disease.

BACKGROUND: Fatigue is a common nonmotor symptom of Parkinson's disease (PD). Although the causes of fatigue were estimated in the previous reports, fatigue is not fully understood. To determine the frequency of and factors related to fatigue in patients with PD, we carried out clinical assessments in our university hospital. METHODS: We used the Japanese version of the Parkinson Fatigue Scale (J-PFS). The J-PFS was administered to 110 patients with PD, and a cutoff point of 3.3 was used for the diagnosis of fatigue. Subsequently, demographic characteristics, clinical features, and medications utilized were evaluated to elucidate the factors related to fatigue. In particular, we focused on the relationship between fatigue and gait disorder assessed via the portable gait rhythmogram. RESULTS: The frequency of fatigue in patients with PD was 52.7%. Univariate analysis revealed that factors significantly associated with fatigue were many motor symptoms and nonmotor symptoms. In addition, multivariate analysis revealed that gait disorder and constipation were independent factors related to fatigue. Furthermore, short-step walking and bradykinesia in gait disorder had especially a relationship with fatigue. CONCLUSIONS: More than half of our patients were judged having fatigue. Several factors, including motor and nonmotor symptoms, might be related to fatigue in patients with PD.