Very-Late-Onset Neuromyelitis Optica Spectrum Disorder in a Patient with Breast Cancer and Parkinson Disease.

Anti-aquaporin-4 (anti-AQP-4) antibody-positive neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder resulting in severe, recurrent optic neuritis, transverse myelitis, brain stem syndrome, and other types of neurological involvement. Its median age of onset has been reported to be around 40 years. We report herein a case of very-late-onset NMOSD (76 years of age) and try to promote its awareness as a type of neurological deterioration in elder patients. A 76-year-old woman suffering from Parkinson disease was admitted to our hospital because of consciousness disturbance. Cranial magnetic resonance imaging revealed the presence of fluid-attenuated inversion recovery high-signal-intensity lesions in the right peri- and intralateral ventricle. Part of this lesion and the meninges showed gadolinium enhancement. Physical examination revealed the presence of a tumor in the right breast, which was later diagnosed as invasive ductal carcinoma. In addition, laboratory examinations led to the detection of anti-AQP-4 antibodies in her serum; consequently, the patient was diagnosed as having NMOSD. She received initial pulsed steroid therapy, followed by right mastectomy. Although the patient's consciousness improved significantly, she developed abrupt-onset bilateral leg weakness and multiple longitudinal spinal cord lesions. Additional steroid therapy ameliorated the patient's leg weakness and reduced the swelling of the spinal cord.

Relations of clinical symptoms with dopamine transporter imaging in drug-naïve Parkinson's disease.

OBJECTIVES: The aim of the present study was to determine the relations of clinical symptoms with nigrostriatal neuron loss in drug-naïve patients with Parkinson's disease (PD). We examined the severity of motor symptoms and freezing of gait (FOG), falls and overactive bladder (OAB) in PD patients and their relations with striatal dopamine transporter (DAT) binding. PATIENTS AND METHODS: Thirty-two untreated PD patients (14 men and 18 women with a mean age of 71.4 ±â€¯7.2 years) were included in this study. Clinical assessments were performed by using Unified Parkinson's Disease Rating Scale (UPDRS) and overactive bladder symptom score (OABSS), and striatal dopamine transporter (DAT) binding was measured by123I-FP-CIT SPECT. RESULTS: The results showed that striatal DAT availability was significantly lower in the high UPDRS motor score group, high akinetic-rigid score group, FOG group, and OAB group than in the low UPDRS motor score group, low akinetic-rigid score group, non-FOG group, and non-OAB group. However, the results also showed that there was no significant difference in striatal DAT availability between the high tremor score group and low tremor score group or between the faller group and non-faller group. CONCLUSIONS: The severity of bradykinesia and axial symptoms and the existence of FOG and OAB in untreated PD patients are related to a decrease in striatal DAT availability. Severity of tremors and occurrence of falls are not related to a decrease in striatal DAT availability. The mechanisms underlying the clinical symptoms of PD involve not only dopaminergic pathways but also non-dopaminergic pathways.

Relationships of drooling with motor symptoms and dopamine transporter imaging in drug-naïve Parkinson's disease.

OBJECTIVES: The aim of the present study was to determine the relationships of drooling with motor symptoms and nigrostriatal neuron loss in drug-naïve patients with Parkinson's disease (PD). We therefore examined the relationships of drooling with motor symptoms and striatal dopamine transporter (DAT) binding measured by [123-Iodine]-fluoropropyl-2beta-carbomethoxy-3beta-(4-iodophenylnortropane) dopamine transporter single-photon emission computed tomography(123I-FP-CIT SPECT). PATIENTS AND METHODS: Thirty-five untreated PD patients (14 men and 21 women with a mean age of 71.9 ±â€¯7.2 years) were included in this study. The patients were divided into a drooler group and non-drooler group. They underwent clinical assessments and 123I-FP-CIT SPECT imaging. Motor symptoms were assessed using Unified Parkinson's Disease Rating Scale (UPDRS). RESULTS: The results showed that UPDRS motor score (p = 0.002) and akinetic-rigid score (p = 0.008) were higher and that striatal DAT availability (p = 0.03) was lower in the drooler group than in the non-drooler group. However, tremor score, age, and duration of PD showed no significant differences between the drooler group and non-drooler group. CONCLUSIONS: Drooling in untreated PD is related to an increase in motor symptoms (especially bradykinesia and axial symptoms) and to reduction of striatal DAT availability.

Incidence of anti-NMDAR encephalitis in patients undergoing resection of ovarian teratoma in a single institution.

OBJECTIVE: The objective of this study was to determine the incidence of anti-NMDAR encephalitis in patients in whom a teratoma was removed. As far as we know, there has been no report on the incidence of anti-NMDAR encephalitis in patients in whom a teratoma was removed. METHODS: This study was a single-institutional observational study. We enrolled patients who were diagnosed with teratoma in the Department of Pathology, Sapporo City General Hospital during a nine-year period between January 2008 and December 2016. RESULTS: In Sapporo City General Hospital, 6 NMDAR encephalitis cases were detected during the 9-year period. In the same 9-year period, a pathological diagnosis of teratoma was made in 343 cases in the hospital. Anti-NMDAR encephalitis patients with a teratoma accounted for only 1.17% of all teratoma patients. Three of the 4 anti-NMDAR encephalitis patients with a teratoma underwent second removal of a teratoma, and no nervous tissue was detected pathologically. CONCLUSIONS: In this study, we determined the association between teratoma with anti-NMDAR encephalitis and teratoma without anti-NMDAR encephalitis in cases in a single institution. As far as we know, this report is the first report on the incidence of anti-NMDAR encephalitis in teratoma patients.

Sub-classification of apraxia of speech in patients with cerebrovascular and neurodegenerative diseases.

Some studies have hypothesized that primary progressive apraxia of speech (ppAOS) consists of heterogeneous symptoms that can be sub-classified; however, no study has classified stroke-induced AOS (sAOS) and ppAOS according to common criteria. The purpose of this study was to elucidate the symptoms and relevant brain regions associated with sAOS and ppAOS for sub-classification. Participants included 8 patients with sAOS following lesions in the left precentral gyrus and/or underlying white matter, and 3 patients with ppAOS. All patients with sAOS could be classified into three subtypes: type I, with prominent distorted articulation; type II, with prominent prosodic abnormalities or type III, with similarly distorted articulation and prosodic abnormalities. This sub-classification was consistent with the subtypes of ppAOS proposed in previous reports. All patients with ppAOS were classified as type III, and exhibited three characteristics distinguishable from those of sAOS. First, they showed prominent lengthened syllables compared with the segmentation of syllables. Second, they could not always complete the production of multi-syllabic single words in one breath. Finally, they showed dysfunctional lesions in the bilateral supplementary motor area. We conclude that sAOS and ppAOS can be sub-classified and are universal symptoms that are common between the English and Japanese populations.

Non-motor Comorbidity of Myasthenia Gravis: Myasthenia Gravis as a Systemic Immunological Disorder Involving Non-motor Systems.

To explore non-motor comorbidities of myasthenia gravis (MG), we present two cases of thymoma-associated MG patients. Alopecia, pure red cell aplasia, and thymoma- associated multiorgan autoimmunity were observed in Case 1, and alopecia, thrombocytopenia, hypogammaglobulinemia and nephrotic syndrome were observed in Case 2. In both cases, autoreactive T lymphocytes inappropriately stimulated by thymus tissue may have played key roles in generating the various autoimmune-associated symptoms. Consequently, systemic immunological involvement due to the thymoma-associated breakdown of immunoregulations in both motor and non-motor systems should be considered in MG patients.

Possible Combined Central and Peripheral Demyelination Presenting as Optic Neuritis, Cervical Myelitis, and Demyelinating Polyneuropathy with Marked Nerve Hypertrophy.

A 27-year-old woman with optic neuritis and cervical myelitis developed hypertrophic demyelinating polyneuropathy. It was hypothesized that the diagnosis was combined central and peripheral demyelination. A hypertrophic nerve was observed subcutaneously, and magnetic resonance imaging demonstrated marked hypertrophy of the nerve roots. The patient was negative for anti-aquaporin 4 antibodies. Her anti-neurofascin 155 antibody levels was slightly elevated, but it was not definitely positive. Pulsed steroid therapy and the administration of immunoglobulin ameliorated her symptoms. Molecules in both the peripheral and central nervous systems might be target antigens, but further investigations will be needed to clarify the precise pathogenic mechanisms.

Fatal Meningoencephalomyelitis due to the Tick-borne Encephalitis Virus: The First Detailed Neurological Observation in a Japanese Patient from the Central Part of Hokkaido Island.

To date, the only instance of tick-borne encephalitis (TBE) in Japan was reported from the southern part of Hokkaido Island in 1993; no other cases have been reported since then. We herein report the first case of TBE reported in the central part of Hokkaido Island, and describe the fatal clinical course of a patient who presented with meningoencephalomyelitis, which partly involved the nerve root. Magnetic resonance imaging (MRI) of the patient's cranium and spine revealed characteristic central nervous system involvement. Our case report is extremely relevant to efforts to protect public health and for precautions against TBE pandemics.

Relation of overactive bladder with motor symptoms and dopamine transporter imaging in drug-naïve Parkinson's disease.

OBJECTIVES: The aim of the present study was to determine the relation of urinary dysfunction with motor symptoms and nigrostriatal neuron loss in drug-naïve patients with Parkinson's disease (PD). We therefore examined the relation of overactive bladder (OAB) symptoms with motor symptoms and striatal dopamine transporter (DAT) binding measured by [123-Iodine]-fluoropropyl-2beta-carbomethoxy-3beta-(4-iodophenylnortropane) dopamine transporter single-photon emission computed tomography (123I-FP-CIT SPECT). PATIENTS AND METHODS: Thirty-one untreated PD patients (12 men and 19 women with a mean age of 71.2 ±â€¯6.7 years) were included in this study. Patients were evaluated with overactive bladder symptom score (OABSS) and divided into an OAB group and Non-OAB group. They underwent clinical assessments and 123I-FP-CIT SPECT imaging. Motor symptoms were assessed using Unified Parkinson's Disease Rating Scale (UPDRS). RESULTS: The results showed that UPDRS motor score (p = 0.01) and akinetic-rigid score (p = 0.002) were higher and that striatal DAT availability (p = 0.01) was lower in the OAB group than in the Non-OAB group. However, tremor score, age, and duration of PD showed no significant differences between the OAB group and Non-OAB group. CONCLUSIONS: Urinary dysfunction in untreated PD is related with increase in motor symptoms (especially bradykinesia and axial symptoms) and reduction of striatal DAT availability.

Neuromyelitis Optica Spectrum Disorder with Recurrent Intracranial Hemorrhage.

The patient was a woman without hypertension who had previously experienced intracranial hemorrhage twice at 48 and 56 years of age. At 59 years of age, she was diagnosed with neuromyelitis optica spectrum disorder (NMOSD) based on the presence of a brain stem lesion and the detection of anti-aquaporin 4 (AQP4) antibodies. After 5 months of continuous treatment with prednisolone (15 mg/day), she presented with optic neuritis and intracranial bleeding. A recurrent attack of NMOSD and intracranial hemorrhage were concurrently diagnosed. We herein report a case of NMOSD with recurrent intracranial hemorrhage, which indicates an association between NMOSD and cerebellar vascular dysfunction.

Two Cases of Human T-Lymphotropic Virus Type I-Associated Myelopathy/Tropical Spastic Paraparesis Caused by Living-Donor Renal Transplantation.

In rare instances, recipients of organ transplants from human T-lymphotropic virus type I- (HTLV-I-) positive donors reportedly developed neurologic symptoms due to HTLV-I-associated myelopathy (HAM). We present herein two cases of HAM associated with renal transplantation from HTLV-I seropositive living-donors. The first patient was a 42-year-old woman with chronic renal failure for twelve years and seronegative for HTLV-I. She underwent renal transplantation with her HTLV-I seropositive mother as the donor, and she developed HAM three years after the transplantation. The second patient was a 65-year-old man who had been suffering from diabetic nephropathy. He was seronegative for HTLV-I and underwent renal transplantation one year previously, with his HTLV-I seropositive wife as the donor. He developed HAM eight months after renal transplantation. Both cases showed neurological improvements after the immunomodulating therapies. We tried to shed some light on the understanding of immunological mechanisms of transplantation-associated HAM, focusing on therapeutic strategies based on the immunopathogenesis of the condition.

Urinary dysfunction and motor symptoms in untreated Parkinson's disease.

OBJECTIVES: The aim of the present study was to determine the associations of motor symptoms in untreated Parkinson's disease (PD) with urinary dysfunction. We therefore examined the association between the overactive bladder symptom score (OABSS) and Unified Parkinson's Disease Rating Scale (UPDRS). PATIENTS AND METHODS: Thirty-one untreated PD patients without dementia (12 men and 19 women with a mean age of 72.0±6.7years) were included in this study. Their urinary tract dysfunctions were rated using the OABSS. The motor symptoms of all patients were also assessed using UPDRS. RESULTS: OABSS had significant correlations with UPDRS motor score (Spearman's rho=0.39, p=0.03) and akinetic-rigid score (Spearman's rho=0.47, p=0.01). However, OABSS showed no significant correlation with tremor score, age, or duration of PD. CONCLUSIONS: Higher OABSS was consistently associated with increase in severity of motor disorders, especially akinetic-rigid score, in the study participants.

Creutzfeldt-Jakob disease with a codon 210 mutation: first pathological observation in a Japanese patient.

We herein report a case of Creutzfeldt-Jakob disease (CJD) with a V210I mutation and discuss the pathological findings. The patient's clinical course was quite similar to that of patients with sporadic CJD. Diffusion-weighted magnetic resonance imaging (MRI) disclosed a high signal intensity in the basal ganglia and cerebral cortices. Pathologically, spongiform degeneration of neurons and their processes with reactive astrocytosis was observed. Prion protein immunostaining revealed diffuse positive and plaque-type patterns. Only one Japanese case of CJD with this type of mutation has been reported to date, but without any pathological examination results. Therefore, this report is considered to be highly significant for understanding CJD.

Unusual basal ganglia lesions in a diabetic uraemic patient proven to be demyelination: first pathological observation.

A 64-year-old man suffering from diabetes mellitus and chronic renal failure was admitted to our hospital because of consciousness disturbance and parkinsonism. Cranial MRI showed very characteristic features involving the bilateral basal ganglia. Subsequent postmortem examinations demonstrated demyelination in the affected areas. These myelin destruction patterns were quite similar to those of central pontine myelinolysis. However, rapid correction of hyponatraemia was ruled out in this patient. Therefore, a new demyelinating brain disease associated with diabetes mellitus and chronic renal failure was suggested.