Investigating the relationships between the burden of multiple sensory hypersensitivity symptoms and headache-related disability in patents with migraine.

OBJECTIVE: Sensory hypersensitivities such as photophobia, phonophobia, and osmophobia are common in patients with migraine. We investigated the burden of these multiple sensory hypersensitivities in migraine. METHODS: In this cross-sectional study, 187 consecutive patients with migraine (26 men/161 women; age, 45.9 ± 13.2 years) were included. Sensory hypersensitivity symptoms such as photo-/phono-/osmophobia and accompanying symptoms were determined by neurologists in interviews. The Migraine Disability Assessment (MIDAS) was used to assess headache-related disability. The Kessler Psychological Distress Scale (K6) was also administered. RESULTS: Photophobia, phonophobia and osmophobia were observed in 75.4%, 76.5% and 55.1% of the patients with migraine, respectively. A significant overlap in sensory hypersensitivities (photo-/phono-/osmophobia) was found; the proportions of patients with 2 and 3 coexisting sensory hypersensitivities were 33.2% and 41.7%, respectively. The MIDAS score was higher in those with 3 sensory hypersensitivity symptoms than in those with 0 to 2 sensory hypersensitivity symptoms. A generalized linear model with ordinal logistic regression analysis revealed that multiple sensory hypersensitivities, younger age, more migraine days per month, and a higher K6 score were significantly related to the higher MIDAS score. CONCLUSION: Our study showed that sensory hypersensitivities commonly occur and overlap in patients with migraine and that multiple sensory hypersensitivity symptoms have a significant impact on headache-related disability.

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. For headache-related disability, the total Migraine Disability Assessment (MIDAS) score and part A and B scores were analysed. Multivariate stepwise linear regression analysis was performed to identify the clinical predictors of changes in the total MIDAS score before and during the COVID-19 pandemic. Logistic regression analysis was performed to determine the factors related to new-onset headache during the COVID-19 pandemic. RESULTS: Finally, 606 migraine patients (73 M/533 F; age, 45.2 ± 12.0 years) were included in the study, excluding those with incomplete data. Increased stress, substantial concern about COVID-19 and negative impacts of the first wave of the COVID-19 pandemic on daily life were reported in 56.8 %, 55.1 and 45.0 % of the participants, respectively. The total MIDAS and A and B scores did not significantly change after the first wave of the COVID-19 pandemic. New-onset headache, which was observed in 95 patients (15.7 %), was associated with younger age and worsened mood and sleep in the logistic regression analysis. The multivariate stepwise linear regression analysis of changes in the total MIDAS score before and during the first wave of COVID-19 pandemic identified worsened sleep, increased acute medication use, increased stress, medication shortages, comorbidities, the absence of an aura and new-onset headache were determinants of an increased total MIDAS score during the first wave of the COVID-19 pandemic. CONCLUSIONS: In this multicentre study, clinical factors relevant to headache-related disability, such as new-onset headache, stress and sleep disturbances, were identified, highlighting the importance of symptom management in migraine patients during the first wave of the COVID-19 pandemic.

Sudden onset of sleep caused by hypothalamic infarction: a case report.

BACKGROUND: Hypothalamic lesions, such as tumors and demyelinating diseases, reportedly cause abnormal sleepiness. However, stroke involving the hypothalamus has rarely been described. Here, we report a patient with infarction restricted to the hypothalamus who presented with sudden onset of sleep. CASE PRESENTATION: A 42-year-old woman with a history of migraine without aura presented with irresistible sleepiness and developed several episodes of sudden onset of sleep. Neurological examinations were unremarkable except for partial left Horner syndrome. Brain magnetic resonance imaging (MRI) revealed a high-intensity lesion restricted to the left hypothalamus on diffusion-weighted and fluid-attenuated inversion recovery MRI images. Cerebrospinal fluid (CSF) orexin-A levels obtained on hospital day 3 after her sleepiness had resolved were normal (337 pg/mL; normal > 200 pg/mL). Serum anti-nuclear and anti-aquaporin 4 (AQP4) antibodies and CSF myelin basic protein and oligoclonal band were negative. A small hypothalamic infarction was suspected, and the patient was treated with intravenous edaravone and argatroban, as well as oral clopidogrel. Three months later, there had been no clinical relapse, and the hypothalamic lesion had almost disappeared on follow-up MRI. No new lesion suggestive of demyelinating disease or tumor was observed. CONCLUSION: Hypothalamic stroke should be considered a cause of sudden onset of sleep.

Restless legs syndrome and its variants in acute ischemic stroke.

BACKGROUND: The clinical-radiological correlation between restless legs syndrome (RLS) or its variants and acute ischemic stroke remains unclear. METHODS: This study prospectively included 104 consecutive patients with acute ischemic stroke, confirmed by diffusion-weighted imaging. The frequency and clinical characteristics of RLS or RLS variants were evaluated according to the International RLS Study Group criteria, as was the topography of the associated lesions. RESULTS: Among 104 patients with acute ischemic stroke, 6 (5.8%) and 2 patients (1.9%) had RLS and RLS variants, respectively, for a total of 8 patients (7.7%). Three (3.3%) had poststroke RLS/RLS variants: 2 (66.7%) had bilateral symptoms and 1 (33.3%) had unilateral symptoms contralateral to the lesion. RLS symptoms developed within 2 days after the onset of stroke. Forty percent of prestroke RLS/RLS variant patients experienced exacerbation of their symptoms after stroke onset, and two-thirds of poststroke RLS/RLS variant patients required treatment for their RLS/RLS variants. Patients positive for RLS/RLS variants tended to have difficulty falling asleep, but there was no difference in daytime sleepiness, sleep quality, depressive symptoms, stroke subtypes, comorbid diseases, laboratory data, or modified Rankin Scale scores at admission or discharge between patients with and without RLS/RLS variants. RLS/RLS variants were most frequently observed to accompany lesions in the medulla (25%), followed by the pons (15.4%), the corona radiata (14.8%), the basal ganglia (3.8%), and the cortex (3.8%). CONCLUSION: RLS/RLS variants were found in 8% of acute ischemic stroke patients. Adequate screening and management are needed to improve patients' quality of life.

Serum insulin-like growth factor-1 levels in neurodegenerative diseases.

BACKGROUND: We investigated serum insulin-like growth factor (IGF)-1 levels in patients with neurodegenerative diseases and correlated these levels with clinical parameters. METHODS: One hundred and fifty-six patients with neurodegenerative diseases were included in this study, and serum IGF-1 levels were determined. RESULTS: Serum IGF-1 levels (mean ± standard error) were not significantly different among the patients with different neurodegenerative diseases: Parkinson's disease (PD; n = 73), 112.1 ± 5.1 ng/mL; progressive supranuclear palsy (n = 15), 102.9 ± 8.3 ng/mL; multiple system atrophy (n = 22), 103.1 ± 37.6 ng/mL; Alzheimer's disease (AD; n = 18), 102.2 ± 9.4 ng/mL; amyotrophic lateral sclerosis (n = 6), 105.5 ± 27.4 ng/mL; dementia with Lewy bodies (n = 14), 82.4 ± 7.4 ng/mL; frontotemporal dementia (n = 6), 90.0 ± 17.0 ng/mL; and corticobasal syndrome (n = 2), 118.0 ± 14.0 ng/mL. In patients with PD, serum IGF-1 levels were negatively correlated with age and modified Rankin scale (mRS) scores and positively correlated with the striatal dopamine transporter-specific binding ratio and the frontal assessment battery score. In patients with AD, serum IGF-1 levels were negatively correlated with age, disease duration, and mRS scores. CONCLUSION: We found correlations of serum IGF-1 levels with frontal lobe and striatal dopaminergic function and disability in PD patients and with disability in AD patients. The usefulness of measuring serum IGF-1 levels for monitoring disease progression in neurodegenerative diseases requires further studies.

Co-occurrence of multiple cerebral infarctions due to hypercoagulability associated with malignancy and meningeal carcinomatosis as the initial manifestation of gastric cancer.

BACKGROUND: Meningeal carcinomatosis and hypercoagulability associated with malignancy are typical late stage complications in cancer patients. The co-occurrence of meningeal carcinomatosis and cerebral infarction related to hypercoagulability associated with malignancy in an individual as the initial manifestation of malignancy has not been previously reported. CASE PRESENTATION: Herein, we report the case of an 80-year-old patient who presented with meningeal carcinomatosis and hypercoagulability related to malignancy as the initial manifestation of occult gastric cancer. The patient displayed consciousness disturbance, mild left facial paralysis, and bilateral positive Babinski's sign. Using brain magnetic resonance imaging, the patient was diagnosed as having acute multiple cerebral infarctions. Cerebrospinal fluid (CSF) cytology showed adenocarcinoma and upper gastrointestinal endoscopy disclosed scirrhous gastric cancer. The patient presented with headache, fever, and meningeal irritation with a subacute course. Tuberculous or fungal meningitis was initially suspected; however, cytological evidence of adenocarcinoma in the CSF led to the diagnosis of meningeal carcinomatosis. CONCLUSION: The comorbidity of hypercoagulability associated with malignancy and meningeal carcinomatosis should be considered in a patient presenting with multiple cerebral infarctions, progressive disturbance of consciousness, fever, and meningeal irritation.

Acceleration time ratio for the assessment of extracranial internal carotid artery stenosis.

PURPOSE: To apply the acceleration time (AcT) ratio as an additional marker for diagnosing internal carotid artery (ICA) stenosis. METHODS: Carotid artery sonography was performed in 140 patients, and the AcT ratio was calculated as the AcT of the ICA divided by the AcT of the ipsilateral common carotid artery, and compared with diameter stenosis. RESULTS: There was a significant correlation between diameter stenosis and the AcT ratio. The receiver operating characteristic curve revealed a cutoff level of 1.5, with 90.0 % sensitivity and 93.5 % specificity for stenosis >65 %. CONCLUSION: Our results indicate that applying the AcT ratio can help in the diagnosis of ICA stenosis.

Isolated shoulder palsy due to cortical infarction: a case report and literature review of clinicoradiological correlations.

Clinicoradiological correlations observed in patients with small cortical infarctions have supported somatotopic representation of different parts of body areas in primary motor cortex. However, isolated shoulder weakness because of infarction in precentral gyrus has rarely been described. We report an 80-year-old woman with isolated shoulder palsy because of cortical ischemic infarction in the base of the left precentral gyrus as confirmed by brain magnetic resonance imaging. In our patient, cardiogenic embolism or Trousseau syndrome associated with lung cancer was considered the cause of ischemic infarction. Physicians should consider small cortical infarction, when a patient complains of sudden onset of shoulder weakness without pain. In line with the previous reports, a responsible cortical lesion in our patient corresponded to motor shoulder area in the motor homunculus reported to be located more medially to the hand area.

Isolated body lateropulsion in a patient with pontine infarction.

A 72-year-old man with diabetes mellitus and hypertension was admitted to our hospital with lightheadedness. The patient showed lateropulsion to the right side, but his neurological findings were otherwise normal. Brain magnetic resonance images showed a fresh ischemic infarct in the left dorsal part of the lower pons. Body lateropulsion is characterized by an irresistible falling to one side and has been reported in lesions in several brain regions. However, it has rarely been reported in pontine lesions. We suggest that physicians should be aware that pontine lesions can cause isolated body lateropulsion without other neurological deficits.

Exploring the contributing factors to multiple chemical sensitivity in patients with migraine.

OBJECTIVE: Multiple chemical sensitivity (MCS) is a form of chemical intolerance in which various systemic symptoms are triggered by exposure to a variety of chemical substances. Although migraine has been associated with central sensitivity syndrome, the relationship between MCS and migraine has not been studied. We assessed the frequency of MCS and its related factors in patients with migraine. METHODS: We performed a cross-sectional study that included 95 patients (14 M/81 F; age, 45.4 ± 12.4 years) out of 100 consecutive patients with migraine from our outpatient headache clinic. MCS was defined as having a combination of Q1 ≥ 30, Q3 ≥ 13, and Q5 ≥ 17 on the quick environment exposure sensitivity inventory (QEESI; Japanese version). Central sensitization inventory-A scores >40 were considered an indication of central sensitization. Headache-related disability and psychological distress were evaluated with the Migraine Disability Assessment score (MIDAS) and Kessler Psychological Distress Scale (K6), respectively. RESULTS: MCS was identified in 20% of patients with migraine; however, none had previously been diagnosed with MCS. The MCS-positive group had higher rates of photophobia, osmophobia, visual aura, sensory aura, and central sensitization and higher MIDAS and K6 scores than the MCS-negative group. A logistic regression analysis showed that osmophobia, sensory aura, and central sensitization were significant contributors to MCS. CONCLUSION: We showed that MCS was observed in 20% of patients with migraine, and our study results may indicate a possible association of MCS with central sensitization and hypersensitivity-related symptoms in patients with migraine.

Impact of the COVID-19 Pandemic on the Quality of Life of Patients with Parkinson's Disease and Their Caregivers: A Single-Center Survey in Tochigi Prefecture.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic has negatively affected the mental health of the general population. OBJECTIVE: We investigated the determinants of quality of life (QOL) in Parkinson's disease (PD) patients during the COVID-19 pandemic. METHODS: Impacts of lifestyle changes due to the COVID-19 pandemic on 100 patients with PD and their caregivers/spouses were assessed. The Hospital Anxiety and Depression Scale was used to assess anxiety and depression. The physical component summary (PCS) and mental component summary (MCS) scores of the short form (SF)-8 were used to evaluate health-related QOL. RESULTS: Regarding health-related QOL, physical function, role physical, general health, vitality and the PCS score were significantly worse in PD patients than in caregivers. Worsening of PD-related symptoms, increased stress, and decreased physical activity were observed in 29.0%, 37.0% and 44.0% of PD patients, respectively. Sixteen patients (16.0%) experienced problems with hospital access, but none reported medication shortages. Strong concerns about COVID-19 were reported by 47.0% of caregivers and 50.0% of PD patients. In PD patients, increased gait disturbance and rigidity, disease severity, smoking, the levodopa equivalent dose and decreased body weight predicted a worse PCS score; anxiety, depression, female sex, stress and long disease duration predicted a worse MCS score. In caregivers, age and smoking contributed to a worse PCS score; depression, stress and worsening patient mood contributed to a worse MCS score. CONCLUSION: We report the negative impacts of the COVID-19 pandemic on health-related QOL and its determinants in PD patients and their caregivers.

Central sensitization in migraine is related to restless legs syndrome.

OBJECTIVE: We hypothesized that, in migraine patients, central sensitization (CS) could be associated with comorbid restless legs syndrome (RLS). METHODS: We conducted a case-control study including 186 migraine patients and 186 age- and sex-matched healthy controls. Symptoms related to CS syndrome were assessed by the Central Sensitization Inventory (CSI). Individuals with CSI Part A (CSI-A) scores ≥ 40 were defined as having CS. For patients with migraine, the Brief Pain Inventory (BPI) and Patient Health Questionnaire (PHQ)-9 were administered. In the patient group, RLS and migraine were diagnosed through face-to-face interviews. RESULTS: Among migraine patients, 26 (14.0%) suffered from chronic migraine. The mean disease duration was 23.7 ± 11.8 years. Migraine patients showed a higher rate of CS (21.0% vs. 8.6%) than healthy controls, with an adjusted odds ratio (AOR) of 3.039 (95% confidence interval (CI) 1.560-5.992; p = 0.001). Migraine patients in the CS group had higher rates of smoking, chronic migraine and RLS and higher BPI and PHQ-9 scores than migraine patients in the non-CS group. The use of acute and preventive treatment for migraine did not significantly differ between the CS and non-CS groups. Multivariable analysis identified the presence of RLS (AOR, 28.471; 95% CI 6.438-125.918; p < 0.001) and the BPI pain interference score (AOR, 1.398; 95% CI 1.061-1.843; p = 0.017) as the significant determinants of CS among migraine patients. CONCLUSION: Migraine patients were 3 times more likely to have CS than healthy controls. Our study results showed an association between RLS and CS in migraine patients.

[A patient with neurosarcoidosis presenting with easy falling and dysphagia].

A 68-year-old woman was referred to our hospital for progressive dizziness, gait disturbances and weight loss for 18 months. The patient was alert and showed dysphagia and a marked tendency to fall backward. Electronystagmography showed bilateral vestibular dysfunction and audiometry showed right sensorineural hearing disturbance. Cerebrospinal fluid exam showed mononuclear pleocytosis and elevated protein levels. On 18F-FDG PET/CT, abnormal uptake was observed in the mediastinal lymph nodes, from which biopsy specimens were obtained. Histological findings showed non-caseous granuloma and a diagnosis of bilateral vestibulocochlear, glossopharyngeal and vagal nerve palsies due to neurosarcoidosis was made. Steroid therapy resulted in improvement in her clinical symptoms. Neurosarcoidosis should be included in the differential diagnosis of patients showing progressive easy falling and dysphagia.

[Bilateral Medial Medulla Infarction Mimicking Guillain-Barré Syndrome and its Variants].

A 70-year-old man presented with dizziness and unsteadiness when standing and was hospitalized in another hospital. Magnetic resonance imaging (MRI) of the brain on Day 1 showed no abnormalities. The patient developed respiratory failure on Day 1and flaccid tetraplegia on Day 3, and was transferred to our hospital. Progressive upper and lower limb weakness and bulbar symptoms suggested Guillain-Barré syndrome or its variant. Diffusion-weighted MRI on Day 6 disclosed high signal intensities in the bilateral medial portion of the medulla, and the patient was diagnosed with bilateral medial medulla infarction. Bilateral medial medulla infarction should be considered when a patient shows progressive tetraplegia, and bulbar palsy and follow-up MRI is important to confirm the diagnosis. (Received January 23, 2020; Accepted April 21, 2020; Published August 1, 2020).

Plasma prostaglandin D2 synthase levels in sleep and neurological diseases.

BACKGROUND: Prostaglandin D2 (PGD2) induces sleep and may play a role in sleep and neurological disorders. We investigated PGD synthase (PGDS) levels in various sleep and neurological disorders. METHODS: Sixty-three patients with neurological or sleep disorders (Parkinson's disease with excessive daytime sleepiness (PDS), n = 19; PD without sleepiness (PDWS), n = 14; Alzheimer's disease (AD), n = 10; narcolepsy (NA), n = 10; sleep apnea syndrome (SAS), n = 10) and 21 healthy controls were included in this study. Plasma lipocalin-type PGDS (L-PGDS) and glutathione-dependent hematopoietic PGDS (H-PGDS) levels were assessed using an enzyme-linked immunosorbent assay. RESULTS: H-PGDS levels were not significantly different among the groups. Compared with healthy controls, the PDWS, PDS and AD groups had higher levels of L-PGDS. Neither H-PGDS nor L-PGDS levels correlated with scores on the Epworth Sleepiness Scale or Pittsburgh Sleep Quality Index in any group. CONCLUSION: We found higher levels of L-PGDS in patients with neurodegenerative diseases such as PD and AD. Whether increased L-PGDS levels reflect underlying sleepiness or the pathophysiology of neurodegenerative diseases needs further study.

Conjugate eye deviation due to pontine infarction: Report of 2 cases.

•We report 2 patients with pontine infarcts showing transient conjugate eye deviation.•Conjugate eye deviation resolved within a few days in both patients.•Small restricted dorsomedial pontine lesions can produce conjugate eye deviation.

Usefulness of acceleration time ratio in diagnosis of internal carotid artery origin stenosis.

PURPOSE: The acceleration time (AcT) ratio of the internal carotid artery (ICA) is increased in ICA stenosis. However, there are few reports that have directly compared the AcT ratio to digital subtraction angiography (DSA) findings. METHODS: We evaluated 177 vessels with DSA and carotid artery ultrasonography. The AcT ratio was calculated as AcT of the ICA (ICA-AcT)/AcT of the ipsilateral common carotid artery (CCA). We evaluated the correlation of DSA-NASCET stenosis with the origin of the ICA or the peak systolic velocity (ICApsv) in the stenotic region, ICApsv/peak systolic velocity of the CCA (CCApsv), ICA-AcT, and AcT ratio. Sensitivity and specificity for stenosis ≥ 70% were calculated based on the ICApsv, ICApsv/CCApsv, ICA-AcT, and AcT ratio. RESULTS: Using NASCET criteria, 34 vessels had 70% or greater stenosis. DSA-NASCET showed a significant positive correlation with ICApsv, ICApsv/CCApsv, ICA-AcT, and AcT ratio (p < 0.0001). When the cut-off value for ICApsv was set at 176 cm/s, ICApsv/CCApsv at 2.42, ICA-AcT at 0.095 s, and the AcT ratio at 1.35, the sensitivity was 97.1, 97.1, 82.4, and 97.1%, and the specificity was 94.4, 91.0, 83.2, and 83.2%, for DSA-NASCET ≥ 70%, respectively. CONCLUSION: The AcT ratio is a beneficial parameter for evaluating ICA stenosis as well as ICApsv and ICApsv/CCApsv.

Human herpesvirus 6 encephalitis followed by acute disseminated encephalomyelitis in an immunocompetent adult.

A 26-year-old, otherwise healthy man presented with visual abnormality followed by loss of consciousness and convulsion. The patient then developed headache and fever 14 days later. Brain MRI showed hyperintensities in the left cingulate cortex. The cerrebrospinal fluid examinations showed mononuclear pleocytosis and positive PCR results for human herpesvirus 6 (HHV-6). A diagnosis of HHV-6 encephalitis and symptomatic epilepsy was made. The patient's clinical symptoms improved promptly following acyclovir treatment. However, 3 months later the patient noticed dysesthesia in the trunk, the left upper limb and the right lower limb. Brain and spine MRI showed multiple brain white matter lesions, the middle cerebellar peduncle and cervical spinal lesions. The symptoms resolved following methylprednisolone pulse therapy only. We report an adult patient with HHV-6 encephalitis followed by acute disseminated encephalomyelitis whose initial presentation was epilepsy. HHV-6 encephalitis should be included in the differential diagnosis of encephalitis of unknown etiology in an immunocompetent adult.

Increased maximum common carotid intima-media thickness is associated with smoking and hypertension in Tochigi Prefecture residents.

OBJECTIVE: We investigated maximum intima-media thickness of the common carotid artery (IMT-Cmax) in residents of Tochigi Prefecture, who have been reported to have high stroke mortality. METHOD: Our study included 840 individuals. All participants underwent carotid ultrasonography and answered a questionnaire during participation in a health festival in Tochigi Prefecture. The questionnaire was designed to collect information on age, gender, and risk factors for stroke. IMT-Cmax was measured. Statistical analyses were performed to identify factors contributing to IMT-Cmax values ≥1.1 mm. RESULTS: In total, 117 subjects had an IMT-Cmax value ≥1.1 mm. IMT-Cmax correlated significantly with age, current smoking, hypertension, diabetes mellitus, heart disease, and previous symptomatic stroke (p < 0.05) in univariate analysis. Current smoking (p < 0.001, odds ratio 3.88) and hypertension (p = 0.0070, odds ratio 1.83) were seen as significant contributing factors to IMT-Cmax ≥1.1 mm in logistic regression analysis adjusted by age, gender, and previous symptomatic stroke. CONCLUSION: We identified current smoking and hypertension as the most significant contributing factors to increased IMT-Cmax in residents of Tochigi Prefecture, emphasizing the importance of routine blood pressure monitoring and anti-smoking education in this population.