Migraine With Aura Accompanied by Myoclonus: A Case Report.

Migraine is a condition characterized by pulsating headaches, often accompanied by photophobia, phonophobia, and/or gastrointestinal symptoms such as nausea and vomiting. Approximately 15% to one-third of migraine patients experience an aura either before or during the headache. To the best of our knowledge, the occurrence of migraine with myoclonus is extremely rare. This report describes a rare case of migraine with aura accompanied by myoclonus. The patient is a 46-year-old man who developed a visual aura followed by vomiting and a throbbing headache on the right side. As the headache intensified, involuntary movements of the left lower extremity appeared. Brain magnetic resonance imaging (MRI) revealed no structural abnormalities or stroke lesions; however, arterial spin labeling MRI showed hypoperfusion in the right cerebral hemisphere. An ophthalmological evaluation was unremarkable. He was diagnosed with migraine with myoclonus, and the intravenous administration of diazepam and sumatriptan resulted in the cessation of the myoclonus and mild relief of the headache. By the day after admission, the myoclonus and visual symptoms had completely disappeared. The headache resolved by the third day of admission.

Rumination Swallowing in Limb-Girdle Muscular Dystrophy.

Patients with limb-girdle muscular dystrophy (LGMD) may develop swallowing disorders as the disease progresses. We detected a novel swallowing pattern in patients with LGMD, characterized by unique findings such as a rumination-like behavior, where food reaches the vallecula and piriform sinuses and then is regurgitated back into the oral cavity, re-chewed, and swallowed again. We termed this swallowing pattern "rumination swallowing." In this report, we describe a case of rumination swallowing, as observed through a videofluoroscopic (VF) examination, in a 56-year-old patient with LGMD.

Elbow Joint Hybrid Assistive Limb Treatment Improves Upper Limb Dysfunction in Guillain-Barré Syndrome: A Case Report.

Robot-assisted rehabilitation is becoming an important option in rehabilitation medicine. We utilized one such device, the elbow joint hybrid assistive limb (HAL), for a patient with Guillain-Barré syndrome (GBS). The patient was a 64-year-old man, 16 months post-onset of GBS. Due to severe neuropathy, he was completely dependent on others and unable to eat independently. After approximately two weeks of intensive rehabilitation and self-training following his hospital discharge, he regained the ability to feed himself. This report highlights the effectiveness of HAL in the chronic phase of GBS.

Verbal-Cue Rehabilitation Exercises Are Effective for the Mixed Callosal-Frontal Variant of Alien Hand Syndrome Following Stroke.

Alien hand syndrome (AHS) is a rare condition in which a brain-injured patient develops involuntary movements, particularly in the upper limbs, along with difficulty in controlling them and a loss of ownership of the affected hand. AHS is traditionally classified into frontal, callosal, and occipital types. Recently, mixed types have also been reported. In this report, we describe a case of mixed callosal-frontal type AHS following stroke, in which symptoms improved with a verbal-cue rehabilitation exercise. The patient was a 62-year-old woman diagnosed with cerebral infarction in the right frontal lobe and corpus callosum due to disruption of the right anterior cerebral artery. She exhibited left hemiplegia, left deep tendon hyperreflexia, and AHS in the left upper extremity. She received verbal-cue rehabilitation exercises for AHS in addition to regular rehabilitation therapy and medications. After treatment, AHS persisted but was remarkably attenuated.

Supernumerary Phantom Limb After Stroke in the Left Hemisphere: A Case Report.

Supernumerary phantom limb (SPL) is a rare sensation of additional limbs that are perceived to exist alongside existing limbs. SPL can occur in various neural regions, but it is rare for SPL to be caused by left hemisphere cerebral infarction. In this report, we describe a case of a 64-year-old woman with SPL after a stroke. She had a history of handedness change. A neurological examination revealed that she had right hemiplegia, unilateral spatial neglect (USN), anosognosia, and pan-sensory loss on the right side of her body. Brain magnetic resonance (MR) imaging disclosed cerebral infarction in the left corona radiata region. She suffered from SPL in the right upper limb. Although SPL was prolonged, the recovery of USN was noted four months after onset, followed by the improvement of SPL.

Fission yeast Bgs1 glucan synthase participates in the control of growth polarity and membrane traffic.

Rod-shaped fission yeast grows through cell wall expansion at poles and septum, synthesized by essential glucan synthases. Bgs1 synthesizes the linear ß(1,3)glucan of primary septum at cytokinesis. Linear ß(1,3)glucan is also present in the wall poles, suggesting additional Bgs1 roles in growth polarity. Our study reveals an essential collaboration between Bgs1 and Tea1-Tea4, but not other polarity factors, in controlling growth polarity. Simultaneous absence of Bgs1 function and Tea1-Tea4 causes complete loss of growth polarity, spread of other glucan synthases, and spherical cell formation, indicating this defect is specifically due to linear ß(1,3)glucan absence. Furthermore, linear ß(1,3)glucan absence induces actin patches delocalization and sterols spread, which are ultimately responsible for the growth polarity loss without Tea1-Tea4. This suggests strong similarities in Bgs1 functions controlling actin structures during cytokinesis and polarized growth. Collectively, our findings unveil that cell wall ß(1,3)glucan regulates polarized growth, like the equivalent extracellular matrix in neuronal cells.

Characteristics of Visual Cognition in Patients with Anoxic Encephalopathy: An Eye-tracking Study.

Objectives: : Despite the frequent occurrence of visual cognitive impairment after anoxic encephalopathy, only a few studies have analyzed gaze movements following encephalopathy. This study determined the visual cognitive characteristics of patients with anoxic encephalopathy using an eye-tracking system. Methods: : This study included ten patients with anoxic encephalopathy and ten age/sex-matched controls. Factors for anoxic encephalopathy onset and brain imaging findings were extracted from medical records. An eye-tracking system was used to track eye movements during three visual search tasks (pop-out, serial search, and saliency) in patient and healthy control groups. The average target search time, number of saccades, and number of fixations to salient stimuli were compared. Results: : Stagnant blood flow, observed in six of ten patients, was the most common cause of disease onset, four of whom exhibited hypoperfusion in bilateral occipital or parietal lobes on single-photon emission computed tomography. The patient group required longer search times during all visual search tasks and a higher number of saccades during pop-out and serial search tasks. However, no significant difference was observed between the two groups for the number of fixations to salient stimuli during the saliency task. Conclusions: : Following anoxic encephalopathy, bottom-up (pop-out task) and top-down (serial search task) gaze control were considered impaired because of extensive parieto-occipital lobe damage after blood flow stagnation. Patients exhibited reduced top-down function for finding targets (serial search task) but relatively retain inhibitory function for salient stimuli (saliency task). Gaze analysis can be used to reveal the clinical characteristics of anoxic encephalopathy.

A Case of Mirror-Image Crossed Thalamic Aphasia With Jargon Agraphia.

In right-handed individuals, aphasia resulting from right hemisphere damage is termed crossed aphasia and has a very low occurrence rate. Additionally, aphasia due to thalamic lesions often involves hemorrhage, with infarction cases less frequently reported. We present the case of an 81-year-old right-handed female who developed aphasia due to a right thalamic infarction. She exhibited characteristics typical of thalamic aphasia observed in left thalamic lesions. Furthermore, jargon agraphia manifested during writing tasks. This may suggest disinhibition of the left hemisphere writing motor memory by the right hemisphere language function.

Double Skull Sign After Cranioplasty: A Case Report.

The double skull sign (DSS) is a unique image on the outside of the brain that looks like two skulls. Whereas congenital and acquired types of DSS have been reported, the etiology of both of them is calcified hematomas. We encountered a case of a 46-year-old woman with a history of subarachnoid hemorrhage followed by cranioplasty at 43 years old. She developed right hemiparalysis and motor aphasia suddenly. Brain computed tomography and magnetic resonance imaging revealed not only cerebral infarction but also DSS incidentally. After detailed analysis, we concluded that the DSS in this case was not due to calcification of the hematoma but was related to the cranioplasty. In this report, we present an interesting case and discuss etiologies of the development of DSS after cranioplasty.

Efficacy of Trihexyphenidyl on Apraxia of Eyelid Opening in Parkinsonism: A Case Report.

Apraxia of eyelid opening (AEO) is occasionally seen in Parkinson's disease (PD) or related diseases. However, many clinicians have trouble with the management of AEO by Parkinsonism. In this report, we describe a case of AEO in Parkinsonism improved by trihexyphenidyl (THP). The patient was a 64-year-old woman, who was previously healthy but developed bradykinesia. She was clinically diagnosed as PD due to an L-dopa challenge test, but no other detailed tests were performed. She started antiparkinsonian medications and her symptoms were improved at an early phase. However, her motor symptoms were gradually exacerbated over time, and antiparkinsonian medications were dosed up. At 69 years old, blepharospasm and AEO developed. Although other antiparkinsonian medications did not improve her AEO, THP cured AEO dramatically at 73 years old. In this report, we discuss a mechanism of AEO by Parkinsonism and the pathway of THP for the improvement of AEO.

Miller-Fisher Syndrome Following Influenza A Infection.

Miller-Fisher syndrome (MFS), characterized by ophthalmoplegia, ataxia, and areflexia, is a Guillain-Barré syndrome (GBS) variant. It is well-known that the causative antibody for MFS is anti-GQ1b antibody. This report describes a rare case of MFS with not only anti-GQ1b antibodies but also anti-GT1a antibodies following Influenza A infection. The patient, a 47-year-old woman, contracted Influenza A three weeks before admission. She complained of double vision followed by areflexia, ataxia in the four extremities, and complete gaze palsy. She was treated with intravenous methylprednisolone pulse and intravenous immunoglobulin therapies. Her neurological symptoms were recovered after these immunotherapies.

Treatment of Subcortical Aphasia Due to Putaminal Hemorrhage With the Japanese Version of Melodic Intonation Therapy (MIT-J).

Melodic intonation therapy (MIT) is one of the rehabilitation methods for patients with non-fluent or dysfluent aphasia, mainly caused by stroke or brain injury. Although MIT is conducted in various languages, reports on the Japanese version of MIT (MIT-J) are limited. In this report, we describe a case about the efficacy of MIT-J in the subacute phase after stroke on subcortical aphasia. Our case was a 60-year-old right-handed woman who suffered from left putaminal hemorrhage. She was treated with acute therapy, including medications and rehabilitation, but non-fluent aphasia was preserved. Regardless of general speech therapies, her aphasia was not improved. In the subacute phase, we started MIT-J (protocol: 20 minutes per day, five days per week for two weeks). The effect of MIT-J was remarkable and in particular, speech intelligibility was improved. It is required to accumulate more cases to reveal the effect of MIT-J.

Pure Motor Isolated Finger Palsy by Cerebral Infarction: Tips for Diagnosis by Neurological Examination.

There are various causes of unilateral finger palsy. Its potential etiologies include peripheral neuropathy, carpal tunnel syndrome, and nerve root disorder due to myelopathy. In addition to them, similar paralysis has been reported in localized lesions of the cerebrum, classically referred to as pseudoperipheral palsy. In this report, we describe a case of an 80-year-old man who developed sudden clumsiness of the right fingers. Neurological examination showed muscle weakness mainly in the 1st and 2nd fingers (Medical Research Council grade 1-4) and normal reflexes in the extremities. The affected muscles were innervated by the median nerve, ulnar nerve, and radial nerve, and their nerve root levels ranged from C6 to T1. All the Phalen's, Tinel's, and flick signs were negative. Diffusion-weighted brain magnetic resonance imaging showed hyperintensity limited in the precentral knob on the left precentral gyrus. The etiology was diagnosed as cardiogenic embolism due to atrial fibrillation. In this report, we provide key findings for diagnosing pure motor isolated finger palsy by cerebral infarction through neurological examination.

Dural Arteriovenous Fistula in Neuro-Behçet's Disease: Association or Chance?

Behçet's disease (BD) is a multisystemic vasculitis disorder. Neuro-Behçet's disease (NBD) is a set of neurologic symptoms imputable to an underlying Behçet vasculitis. Among the wide range of vascular abnormalities secondary to BD, a dural arteriovenous fistula (dAVF) is not classically described. Whether a dAVF is associated with BD or dAVF is a chance occurrence is still a matter of debate. Herein, we describe an NBD case of a 48-year-old male, presenting with headache and fever, where a dAVF was seen on imaging. He was treated with prednisolone and colchicine, followed by the surgical resection for dAVF. Then, we discuss the possible association between BD and dAVF based on the latest literature.

Hashimoto Encephalopathy and Thyroid Storm by Diabetic Ketoacidosis and Acute Pancreatitis: A Case Report.

Hashimoto encephalopathy (HE) is a rare condition related to autoimmune origin characterized by high titers of antithyroid antibodies. Steroids are effective for treatment of HE, suggesting the autoimmunity as an underlying mechanism. In addition, anti-NH2-terminal of α-enolase antibodies (anti-NAE antibodies) is useful for diagnosis of HE. This report describes a 69-year-old woman developing both HE and thyroid storm (TS), following diabetic ketoacidosis (DKA) and acute pancreatitis. She had a history of Basedow's disease and uncontrolled type 2 diabetes mellitus, and her serum hemoglobin A1c was 10%. She complained of nausea and visited our hospital. She was diagnosed with DKA and acute pancreatitis. After admission, she went into cardiopulmonary arrest and she was diagnosed with TS after resuscitation. In addition, blood test collected during acute phase of TS revealed positive for not only anti-thyroid peroxidase (TPO) antibodies, thyroid stimulating hormone receptor antibodies and thyroid stimulating antibodies, but also anti-NAE antibodies. She was treated with intravenous steroids, potassium iodide and thiamazole under respirator and recovered sufficiently to do daily activities of life. We should keep in mind that there might be cases of HE in cases of TS presenting with central nervous system symptoms.

Lateral Medullary Infarction With Atonic Bladder and Lateropulsion.

Lateral medullary infarction (LMI), or Wallenberg syndrome, can develop various symptoms, but it is rare that ipsilateral axial lateropulsion (or body lateropulsion, BL) or atonic bladder (AB) are caused by LMI. This report describes a case of LMI with both BL and AB. A 77-year-old man, with a history of hypertension and diabetes, developed acute left BL and anuresis. A neurological exam showed right gaze nystagmus, slight dysarthria and dysphagia, right dysesthesia in the trunk, and ataxia in the left limbs and trunk. Horner's syndrome and paralysis were unremarkable. Brain magnetic resonance imaging revealed hyperintensity in the lateral medulla oblongata. Cystometry revealed AB, although the patient had the urge to urinate. Owing to acute therapy, although trunk ataxia was presented for several months, BL and anuresis were recovered on day 15 and day 35, respectively. Here, we describe the potential mechanisms of BL and AB caused by LMI.

Normal-Pressure Hydrocephalus-Like Appearance in Myotonic Dystrophy Type 1.

Myotonic dystrophy type 1 (DM1) is one of the monogenic neurological diseases that neurologists most often experience. DM1 can develop several symptoms, including muscle weakness, gait disturbance, urinary incontinence, and cognitive decline. Other hand, normal pressure hydrocephalus (NPH) is more frequent in the elderly population and is characterized by a triad of symptoms, gait disturbance, urinary urge incontinence, and cognitive decline. Therefore, some symptoms overlap between DM1 and NPH. In this report, we described a case of DM1 that presented with a triad of NPH, and NPH-like changes in brain images. A 54-year-old man with DM1 visited our hospital for rehabilitation. He had a history of dyslipidemia, diabetes, and cataracts. He developed muscle weakness, blepharoptosis, and dysarthria at 43 years. Neuro-exam revealed percussion and grip myotonia, distal muscle weakness and atrophy, broad-based gait, and urinary incontinence. The mini-mental state examination score was 18. Brain magnetic resonance imaging revealed enlarged lateral and third ventricles and Evans index was 0.38 (NPH criterion; >0.3), which was mimicking for NPH. Tap test (TT) was evaluated twice. First TT improved clinical symptoms slightly, but second was unremarkable. Based on the second TT result, we could not diagnose with NPH and could prevent unnecessary surgical shunting. Brain imaging of DM1 can show an NPH-like appearance in patients older than 50. Although TT is the gold standard for diagnosing NPH, its sensitivity and specificity vary among reports. TT results should be interpreted with caution before performing a surgical shunt. If necessary, multiple TTs should be considered in DM1 patients.

A Case of Autoimmune Polyendocrine Syndrome Type 3B and Peripheral Neuropathy Due to Thiamine Deficiency.

Autoimmune polyendocrine syndrome (APS) type 3B is characterized by presence of autoimmune thyroid disease, chronic atrophic gastritis and pernicious anemia. In this report, we present a rare case of APS type 3B with neuropathy by thiamine deficiency. A 65-year-old man had a history with hypothyroidism, gastritis, gastrectomy for gastric cancer and subacute combined degeneration of the spinal cord. Patient developed polyneuropathy with not mecobalamin but thiamine deficiency. Serum anti-thyroglobin (TG), anti-thyroid peroxidase (TPO), and anti-gastric parietal cell antibodies were positive. He was treated with thiamine supplementation and improved muscle weakness, sensory impairment and gait disturbance. Classically, it is reported gastric cancer related to hypothyroidism. Additionally, thiamine deficiency can be caused by gastrectomy. Here, his thiamine deficiency was related to APS type 3B, leading to polyneuropathy.

Effect of radical trachelectomy on ovarian reserve: A single-institute prospective study.

AIM: Reduced responses to controlled ovarian stimulation (COS) after radical trachelectomy (RT) have been previously reported. We aimed to assess the effect of RT on ovarian reserve by measuring anti-Müllerian hormone (AMH) levels before and after the procedure in this prospective study. METHODS: We included 12 patients who underwent RT between September 2019 and December 2021 in this study. Serum AMH levels were measured preoperatively, 1 month postoperatively, and 6 months postoperatively. Differences in the AMH levels were assessed using a paired t-test. RESULTS: The median age of the patients was 30.6 years, and the median follow-up time was 30.1 months. AMH levels at 1 and 6 months postoperatively did not show a consistent trend. At 1 month postoperatively, the average AMH level decreased insignificantly but returned to preoperative levels at 6 months. The differences in AMH levels before and after RT were insignificant. CONCLUSION: Our findings indicate that RT did not affect ovarian reserve as measured by AMH levels. However, the relationship between unchanged ovarian reserve and reduced response to COS remains unclear. Further research with larger sample sizes and additional measures of ovarian function is needed to corroborate these results and investigate the long-term effects of RT on ovarian reserve. Understanding these mechanisms will help guide surgical practices and provide patients with valuable information about their reproductive outcomes after RT.

Risk Factors for Alcohol Consumption after Starting Assisted Reproductive Technology Treatment among Japanese Women: Japan-Female Employment and Mental Health in Assisted Reproductive Technology (J-FEMA) Study.

AIMS: To determine the association between drinking habits and social factors among women undergoing assisted reproductive technology (ART) treatment in Japan. METHODS: The study participants who provided answers for the questionnaire concerning alcohol consumption were 1017 female patients undergoing ART treatment were enrolled in the Japan-Female Employment and Mental Health in assisted reproductive technology (J-FEMA) study between August and December 2018. Patient characteristics, including demographic, clinical, and socioeconomic status, were assessed using a self-administered questionnaire which was distributed only once during the period, regardless of their first or follow-up examination. We defined current drinkers who drank ≥46 g of ethanol per week as the habitual drinking group. The risk factors for habitual drinking were analyzed using multivariable-adjusted logistic regression. RESULTS: The proportion of habitual drinkers was 15.5% in this study population. The multivariable-adjusted odds ratios (95% confidence interval) for habitual drinking were 2.27 (0.99-5.21) for women aged ≥35 years versus those <35 years, 4.26 (1.98-9.16) for women having partners who currently drink compared to those with partners without current drinking, 1.84 (1.08-3.12) for women without a history of childbirth versus those with, and 1.77 (1.00-3.14) for working women compared with those not working. CONCLUSIONS: In our study, habitual drinking among women undergoing ART treatment was significantly associated with older age, no history of childbirth, partner's current drinking status, and working.