[Autonomic Disorders in Trigeminal Autonomic Cephalalgias: An Update].

The term trigeminal autonomic cephalalgias (TACs) was introduced in the 2 edition of the International Classification of Headache Disorders, and has been retained in the 3 edition. TACs include cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks, hemicrania continua, and probable trigeminal autonomic cephalalgia. Headaches classified as TACs share clinical features with unilateral headache and usually present with prominent lateralized and ipsilateral cranial parasympathetic autonomic features, including the following: (1) conjunctival injection and/or lacrimation, (2) nasal congestion and/or rhinorrhea, (3) eyelid edema, (4) forehead and facial sweating, (5) miosis and/or ptosis.

Impact of the COVID-19 pandemic on migraine in Japan: a multicentre cross-sectional study.

Objectives: To assess the impacts of social situation changes due to the coronavirus disease 2019 (COVID-19) pandemic on headache-related disability and other symptoms in patients with migraine in Japan.

Methods: We conducted a multicentre, cross-sectional study including 659 outpatients with migraine diagnosed by headache specialists. The participants were asked about the impacts of the first wave of the COVID-19 pandemic on headache-related disability, headache days, headache intensity, stress, physical activity, hospital access and their work and home lives.

[Gepants and Ditans].

The calcitonin gene-related peptide (CGRP) has been shown to play a major role in the pathophysiology of migraine in recent years. Studies have suggested that blocking CGRP signaling is an effective preventive and therapeutic strategy in patients with migraine. Triptans, considered the mainstay of antimigraine treatment cause vasoconstriction; however, gepants and ditans (two novel classes of therapeutic agents) inhibit CGRP release but do not show a vasoconstrictor effect.

Cowden Syndrome with a Novel PTEN Mutation Presenting with Partial Epilepsy Related to Focal Cortical Dysplasia.

Cowden syndrome is a rare autosomal dominant disorder characterized by multiple hamartomas of the ectoderm and brain. A 36-year-old Japanese man presented with right facial seizure during sleep and was admitted to our hospital. He showed cobblestoning over the tongue and palmar pitting but no neurological abnormalities while he was not having a seizure.

Association between Serum Uric Acid Level and Activity of Daily Living in Japanese Patients with Ischemic Stroke.

Background: An association between serum uric acid and outcomes of ischemic stroke has been reported, but the results are controversial. The aim of this study is to clarify how uric acid may affect activities of daily living after acute ischemic stroke.

Methods: Consecutive Japanese patients with acute ischemic stroke were analyzed.

Proximal Bright Vessel Sign on Arterial Spin Labeling Magnetic Resonance Imaging in Acute Cardioembolic Cerebral Infarction.

Background: The congestion of spin-labeled blood at large-vessel occlusion can present as hyperintense signals on perfusion magnetic resonance imaging with 3-dimensional pseudo-continuous arterial spin labeling (proximal bright vessel sign). The purpose of this study was to clarify the difference between proximal bright vessel sign and susceptibility vessel sign in acute cardioembolic cerebral infarction.

Methods: Forty-two patients with cardioembolic cerebral infarction in the anterior circulation territory underwent magnetic resonance imaging including diffusion-weighted imaging, 3-dimensional pseudo-continuous arterial spin labeling perfusion magnetic resonance imaging, T2*-weighted imaging, and 3-dimensional time-of-flight magnetic resonance angiography using a 3-T magnetic resonance scanner.

Prevalence of progressive supranuclear palsy in Yonago: change throughout a decade.

Background: Progressive supranuclear palsy (PSP) is a neurodegenerative disorder that is sometimes confused with Parkinson's disease, multiple system atrophy, and other disorders. The typical clinical features are categorized as Richardson's syndrome (RS), but other clinical subtypes include PSP-parkinsonism (PSP-P) and PSP-pure akinesia with gait freezing (PSP-PAGF). In this study, we determined the prevalence of PSP in a Japanese rural area compared to our previous 1999 report.

Atypical Arteritis in Internal Carotid Arteries: A Novel Concept of Isolated Internal Carotid Arteritis.

We presented a 38-year-old woman suffering from acute cerebral infarction due to arteritis limited to bilateral internal carotid arteries without a condition of giant cell arteritis or granulomatosis with polyangitis. Our case is unprecedented and characterized by a young woman with wall enhancement in the internal carotid arteries on contrast-enhanced magnetic resonance imaging (MRI), therapeutic effects of steroids, and positive status for human leucocyte antigen-B39, -B51 and -DR4. These disease characteristics were not in accordance with existing diagnostic criteria of vasculitis, such as Takayasu's arteritis, giant cell arteritis, granulomatosis with polyangiitis, and Behcet's disease.

Association between body mass index and outcome in Japanese ischemic stroke patients.

Aim: An association between body mass index (BMI) and stroke outcome have been reported, but the results are controversial. The aim of the present study was to evaluate whether BMI is associated with ischemic stroke outcome.

Methods: Consecutive Japanese acute ischemic stroke patients were analyzed.

Plasma Brain Natriuretic Peptide is a Marker of Prognostic Functional Outcome in Non-Cardioembolic Infarction.

Background: High plasma levels of brain natriuretic peptide (BNP) may also be observed in patients with non-cardioembolic infarction (CEI). We aimed to evaluate the relation between plasma BNP level, clinical parameters, and functional outcome in patients with and without CEI.

Method: This study analyzed consecutive Japanese patients with acute ischemic stroke.

[A case of late-onset cobalamin C disease (methylmalonic aciduria and homocystinuria, cobalamin C type)].

We report the case of an 18-year-old Japanese woman with cobalamin (cbl) C disease. She was born between non- consanguineous parents, and had easy fatigability from a childhood. At 14 years old, she developed renal failure, and had repeated psychosis during 2 years.

[Cortical spreading depression and pain: a missing link in the pathophysiology of migraine?].

It is generally believed that cortical spreading depression (CSD) demonstrated by Leao underlie migraine aura and migraine headache depends on the activation of the trigeminovascular pain pathway proposed by Moskowitz. The onset of migraine attack and the association between CSD and the trigeminovascular pain pathway have remained largely unknown. Recent animal studies indicate that CSD can activate trigeminal nociception and thus trigger headache mechanism.

[Current status of migraine chronification in Japan].

Chronification of migraine headache is one of the major issues to resolve urgently. The prevalence of chronic migraine is close to 2％, and the chronification occurs in approximately 2.5％ of episodic cases annually.

[Cortical spreading depression and molecular genetics in migraine].

Cortical spreading depression (CSD) is a slowly propagated wave of depolarization of neurons and glial cells, followed by a subsequent sustained suppression of spontaneous neuronal activity. Functional imaging studies of migraine patients have shown dramatic changes in blood flow and brain activity whose characteristics are similar to those of CSD, implicated in migraine visual aura. Although the trigeminal nerve innervates the meninges and participates in the genesis of migraine headaches, triggering mechanisms remain controversial and poorly understood.

[Clinical course and natural history in migraine].

Migraine is one of the common diseases, suffering 8.4 million patients in Japan. Recently conditions associated with chronic migraine were considered to be worse than episodic migraine in socio-economic status, health-related quality of life, and headache-related burden.

Relationship between three-year survival and functional outcome at discharge from acute-care hospitals in each subtype of first-ever ischemic stroke patients.

Background: We studied the relationship between survival prognosis and the functional outcome at discharge from acute-care hospitals in each subtype of ischemic stroke patients.

Methods: A total of 853 consecutive patients with first-ever ischemic stroke, recruited from December 1999 to November 2002, were followed for 725.8 ± 430.

[A case of acute cerebellar ataxia associated with serum anti-NH2 terminal of alpha-enolase (NAE) antibody].

We reported a 61-year-old man who had developed acute cerebellar ataxia in the trunk and the lower limbs. His chemical blood analysis showed very mild hypothyroidism and the presence of serum anti-thyroid peroxidase (TPO) antibody and anti-NH2 terminal of alpha-enolase (NAE) antibody. While cerebellar atrophy was not evident on magnetic resonance imaging (MRI) of the brain, 99mTc-ECD SPECT using the easy Z-score imaging system (eZIS) showed decreased regional cerebral blood flow (rCBF) in the vermis of cerebellum.

Long-term prognosis of patients with large subcortical infarctions.

Aim: We assessed the long-term prognosis of patients with large subcortical infarctions (LSCI).

Methods: We defined LSCI as lesions > or =15 mm confined to deep penetrating arteries without a cardioembolic or atherothrombotic source. Patients with acute ischemic strokes were consecutively registered and followed for 751 +/- 441 days.

Changes in prevalence and incidence of Parkinson's disease in Japan during a quarter of a century.

Background/aim: To determine the prevalence and incidence of Parkinson's disease (PD) and compare them with results from our previous studies.

Methods: We examined epidemiological characteristics of PD patients using a service-based study in Yonago City, and a door-to-door study in Daisen Town. The prevalence days were April 1, 2004 in Yonago, and April 1, 2003 in Daisen.

The effect of donepezil on increased regional cerebral blood flow in the posterior cingulate cortex of a patient with Parkinson's disease dementia.

It has been reported that the cholinesterase inhibitor, donepezil, improves cognitive decline in patients with Parkinson's disease dementia (PDD). However, this improvement was dominant for frontal lobe dysfunction, and the increase in the Mini-Mental State Examination (MMSE) score was minimal. We report a PDD patient with a decline of regional cerebral blood flow (rCBF) in the posterior cingulate cortex, precunei, and bilateral parietotemporal association cortex, as determined by single-photon emission computed tomography (SPECT) using the easy Z-scores imaging system (e-ZIS).

[Omohyoid muscle syndrome in a patient with Parkinson's disease].

A 49-year-old man noticed a resting tremor in his right hand and was diagnosed with Parkinson's disease (PD) at the age of 43. Soon thereafter, he noticed a right neck mass that only appeared when he swallowed and was accompanied by an uncomfortable sensation. At the age of 49, he was admitted to our hospital to adjust his medication for PD and to examine the neck mass.

Serum heart-fatty acid binding protein levels in patients with Lewy body disease.

Objective: In order to examine a possible role of serum heart-fatty acid binding protein (H-FABP) in patients with Lewy body disease, we measured serum levels of H-FABP in patients with dementia with Lewy bodies (DLB), Parkinson's disease (PD), and Alzheimer's disease (AD).

Methods: Serum levels of H-FABP were measured using a solid-phase enzyme-linked immunoassay. Iodine-123 metaiodobenzylguanidine ((123)I-MIBG) cardiac scintigraphy was performed on each patient and the heart to mediastinum (H/M) ratio was calculated.