Characteristics of Early-Onset Dementia in Chiba Prefecture, Japan: A Multicenter Survey.

INTRODUCTION: Early-onset dementia (EOD), defined as dementia onset before the age of 65 years, is relatively rare, but its social impacts are significant. This study aimed to characterize the diagnosis and clinical and social status of EOD subjects in the 11 dementia centers in Chiba Prefecture, Japan. METHODS: A retrospective 1-year survey was conducted. Collected data included clinical diagnosis, age at onset, age at survey, neuropsychological test, family history, employment, and living status. RESULTS: We identified 208 EOD subjects, including 123 (59.4%), 24 (11.6%), 21 (10.1%), 17 (8.2%), and 10 (4.8%) with Alzheimer's disease (AD), vascular dementia, frontotemporal lobar degeneration (FTLD), dementia with Lewy bodies/Parkinson's disease dementia, and alcohol-related dementia, respectively. The Mini-Mental State Examination (MMSE) score <24 was observed in 50-75% of patients and was not correlated with disease duration. Twenty-four (16.4%) subjects had positive family history of EOD. EOD subjects were at risk of early retirement, and 133 subjects lived with their family, in whom 64 (30.8%) lived with their child. CONCLUSION: In dementia centers, AD, FTLD, and Lewy body dementia had relatively large proportion. Employment, economy, and social supports are urgently needed for EOD subjects and their family.

[A Case of Bone Marrow Metastasis of Oligodendroglioma with IDH Mutation and 1p/19q Codeletion].

A 71-year-old woman was transferred to our hospital, complaining of a seizure for the first time. A tumor was detected in the right frontal lobe, and a craniotomy was performed with a partial tumor resection. The pathological diagnosis was oligodendroglioma with IDH mutation and 1p/19q codeletion, and irradiation therapy was performed. Six months later, the patient's lactate dehydrogenase(LDH)level elevated remarkably, and the fluoro-deoxyglucose-positron emission tomography/computed tomography showed abnormal uptake in multiple bone marrow locations. Bone marrow aspiration was performed, and the pathological diagnosis was oligodendroglioma metastasis. The patient was given two cycles of chemotherapy with temozolomide(TMZ), and her LDH level reduced to normal. After a few months, the LDH level elevated again, so we gave her two more cycles of TMZ;however, her LDH level did not change. Thereafter, the patient was hospitalized because of paraplegia, which started a few days prior, and right lower jaw swelling. Her CT and magnetic resonance imaging showed metastasis to the thoracic vertebrae and right mandibular bone. Irradiation therapy was performed to these locations, and the patient was given chemotherapy using nimustine(ACNU), procarbazine, and vincristine(PAV). Her LDH levels reduced temporarily, but elevated again. The patient deteriorated slowly and died 20 months after she presented with a seizure. Oligodendroglioma with extracranial metastasis is extremely rare, and this case report is the 68th report. The chemotherapy approach with TMZ or PAV/PCV may be effective against oligodendroglioma metastasis to the bone marrow.

[A Ruptured Middle Cerebral Artery Dissecting Aneurysm Operation in a 2-Year-Old Boy].

We report the case of a ruptured dissecting aneurysm in an infant. A 2-year-old boy suffered sudden headache and nausea. CT scan revealed a subarachnoid hemorrhage, and digital subtraction angiography(DSA)revealed a dissecting aneurysm(3.9mm)at a branch of the right middle cerebral artery(MCA). Eight days after onset, carotid angiography revealed enlargement of the aneurysm(up to 6.8 mm). Right frontotemporal craniotomy and aneurysm trapping with STA-MCA bypass was performed. Two years and two months later, the aneurysm recurred as assessed by magnetic resonance angiography(MRA). DSA revealed the recurrence of the aneurysm(7.8mm)proximal to the previous aneurysm and displayed development of collateral flow through leptomeningeal anastomosis between the branches of the MCA. During the second operation, the aneurysm extended to the artery branch proximal to the previous trapping. Parent artery occlusion without bypass was performed. Postoperative DSA revealed collateral flows through leptomeningeal anastomosis between the branch of the MCA and the branch of anterior cerebral artery. At the 3-year follow-up period, abnormal findings on MRI did not appear and he was healthy without any neurologic abnormalities.

Secondary Central Nerve System Lymphoma With Intratumoral Hemorrhage Suggested as Intravascular Lymphoma by Autopsy: A Case Report.

Intravascular large B-cell lymphoma (IVL) is a rare type of extranodal diffuse large B-cell lymphoma (DLBCL), which often infiltrates the central nervous system (CNS) during the clinical course. Cerebral hemorrhage in patients with CNS lymphoma at presentation is rare. Herein, we describe a case of secondary CNS lymphoma with intratumoral hemorrhage, which was suggested as IVL from autopsy findings. A 76-year-old Japanese man with a history of treatment for B-cell non-Hodgkin's lymphoma was transferred to our hospital in an ambulance for generalized convulsions. Brain CT scan revealed a high-density tumor with edema and intratumoral hemorrhage in the left temporal lobes. He died in a rapid course, and autopsy revealed a focal hemorrhage with diffuse infiltration of lymphoma cells in the left temporal lobe and findings suggestive of IVL. Furthermore, the autopsy revealed a discrepancy in the CD20 immunostaining of lymphoma cells between the brain and other organs. Clinicians should not eliminate CNS lymphoma from the differential diagnosis of intracranial tumor with hemorrhage. Although many patients with IVL have rapidly progressive courses, it is very important to diagnose IVL at the initial onset, even in serious situations, to consider CNS prophylaxis.

Development of Epstein-Barr Virus-related Primary Diffuse Large B-cell Lymphoma of the Central Nervous System in a Patient with Peripheral T-cell Lymphoma, Not Otherwise Specified after Mogamulizumab Treatment.

Mogamulizumab is a defucosylated humanized anti-CC chemokine receptor type 4 (CCR4) antibody that exerts an anti-tumor immune effect against various tumors through a suppressive effect on regulatory T-cells. We herein report a patient with peripheral T-cell lymphoma who developed Epstein-Barr virus (EBV)-related primary diffuse large B-cell lymphoma of the central nervous system (CNS DLBCL) after mogamulizumab therapy. Our experience should alert physicians to the possibility of the development of EBV-related CNS DLBCL in patients treated for primary lymphoma and suggests that the anti-tumor immune effect of mogamulizumab is ineffective for the prophylaxis of EBV-related lymphomas.

[Hydrocephalus Mimicking Idiopathic Normal Pressure Hydrocephalus as the First Manifestation of Neurosarcoidosis].

A 61-year-old woman presented with a 10-month history of gait disturbance and a 7-month history of urinary incontinence. The Hasegawa dementia scale-revised score indicated cognitive impairment. Brain magnetic resonance imaging (MRI) indicated hydrocephalus with disproportionately enlarged subarachnoid space. This is usually considered a characteristic finding in idiopathic normal pressure hydrocephalus (iNPH). Ventriculo-peritoneal shunting improved the patient's symptoms. Neurosarcoidosis was suspected as a cause of the hydrocephalus because of the abnormalities in the cerebrospinal fluid and the abnormal enhancement of the cauda equina, the leptomeninges of the brainstem, and the spinal cord, as seen on MRI with gadolinium enhancement. A biopsy from the mediastinum lymph nodes confirmed the histological diagnosis of sarcoidosis. Physicians should consider the possibility of neurosarcoidosis in patients presenting with hydrocephalus, even in cases where clinical and radiological data are characteristic of iNPH. (Received April 13, 2016; Accepted July 5, 2016; Published December 1, 2016).