Publications by authors named "Hitoshi Aizawa"
Objectives: This study aimed to clarify the relationship between 43-kDa TAR DNA-binding protein (TDP-43) pathology and spinal cord anterior horn motor neuron (AHMN) atrophy in sporadic amyotrophic lateral sclerosis (SALS).
Methods: Eight patients with SALS and 12 controls were included in this study. Formalin-fixed specimens of lumbar spinal cord samples were paraffin-embedded and sectioned at the level of the fourth lumbar spinal cord with a 4 μm thickness.
Objective: It is generally believed that the decremental response in repetitive nerve stimulation (RNS) stabilizes at the fourth or fifth response. We have a preliminary impression that the decremental response approaches a plateau earlier in proximal muscles than in distal muscles. We investigated the speed of the completion of the decremental response in different muscles.
View Article and Find Full Text PDFPatients with amyotrophic lateral sclerosis (ALS) do not develop oculomotor disturbances and vesicorectal dysfunction until end-stage disease owing to the survival of certain motor neurons (MNs), including oculomotor neurons and MNs within Onuf's nucleus. In sporadic ALS, adenosine deaminase acting on RNA 2 (ADAR2)-mediated editing of GluA2 mRNA at the Q/R site is compromised in lower MNs. We previously developed genetically modified mice with a conditional knockout of ADAR2 in cholinergic neurons (ADAR2/VAChT-Cre, Fast; AR2).
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- A 73-year-old man experienced a non-traumatic rectus sheath hematoma (RSH) while on the direct oral anticoagulant, apixaban, after suffering a severe cough, leading to sudden right abdominal pain.
- RSH occurs due to bleeding in the rectus sheath, typically from ruptured blood vessels and muscle tears, and can complicate anticoagulation therapy.
- The patient was diagnosed through a CT scan, anticoagulant therapy was temporarily halted, and RSH was resolved after four weeks, emphasizing the need to consider RSH in patients on anticoagulants presenting with acute abdominal pain.
High-risk patent foramen ovale and elderly in cryptogenic stroke.
J Stroke Cerebrovasc Dis
November 2023
Background: High-risk patent foramen ovale (PFO) could be pathological in cryptogenic stroke (CS), but its clinical characteristics have not been fully studied, especially in elderly patients.
Methods: Patients with CS were enrolled in the CHALLENGE ESUS/CS registry, a multicenter registry of CS patients undergoing transesophageal echocardiography. Clinical characteristics were compared among three groups: high-risk PFO group, large shunt PFO (≥25 microbubbles) or PFO with atrial septal aneurysm (ASA); right-to-left shunt (RLS) group, RLS including PFO with <25 microbubbles or without ASA; and no-RLS group.
Background: Dropped head syndrome (DHS) is a rare specific abnormal posture known to develop in Parkinson's disease (PD). This case series study aimed to characterize DHS by analyzing the characteristics of sagittal spinopelvic alignment in patients with PD/DHS.
Methods: The study included eight patients with PD/DHS (men = 3, women = 5; mean age, 68.
Article Synopsis
- The study aimed to investigate how perampanel (PER) affects the progression of sporadic amyotrophic lateral sclerosis (sALS) by analyzing the relationship between PER serum levels and ALSFRS-R scores in patients.* -
- A total of 12 sALS patients participated, and based on their serum PER concentrations, they were divided into two groups: a high concentration group (H group) and a low concentration group (L group), with a control group taking a placebo.* -
- Results showed no significant differences in ALSFRS-R scores at 48 weeks, but the H group showed a significantly worse bulbar subscore at 36 weeks compared to the control group, suggesting that higher PER levels may worsen certain symptoms
Article Synopsis
- Amyotrophic lateral sclerosis (ALS) leads to the degeneration of motor neurons, primarily due to a lack of a specific RNA editing enzyme, ADAR2, which affects a key mRNA.
- In genetically altered mice that lack ADAR2 in cholinergic neurons, there is a notable loss of lower motor neurons and increased TDP-43 abnormalities, especially in older mice.
- The study found that in 12-month-old ADAR2-deficient mice, there was a significant reduction in motor neurons and an increase in reactive astrocytes in the lateral areas of the anterior horns of the lumbar spinal cord compared to normal mice.
The patient was a 30-year-old man who developed muscle weakness in both lower extremities, sensory deficits below the fourth thoracic spinal cord level, and bladder rectal dysfunction owing to cytomegalovirus (CMV) associated myelitis. His blood tests showed mononucleosis, hepatic dysfunction, and the presence of serum CMV-IgM antibodies, and T-weighted imaging on MRI displayed a continuous high signal on the ventral side of the spinal cord. Although his medical history and laboratory tests did not indicate that he was immunocompromised, we speculated he had CMV-associated myelitis.
View Article and Find Full Text PDFHigh frequency of AND mutations in Japanese patients with adult-onset cerebral small vessel disease.
J Neurol Neurosurg Psychiatry
January 2023
Background: This study aimed to clarify the frequency and clinical features of monogenic cerebral small vessel disease (mgCSVD) among patients with adult-onset severe CSVD in Japan.
Methods: This study included patients with adult-onset severe CSVD with an age of onset ≤55 years (group 1) or >55 years and with a positive family history (group 2). After conducting conventional genetic tests for and , whole-exome sequencing was performed on undiagnosed patients.
Article Synopsis
- Freezing of gait (FOG) is a major issue for Parkinson's disease (PD) patients affecting daily life, but its underlying causes are not well understood.
- *The study examined 53 newly diagnosed, untreated PD patients and classified them into "freezers" and "nonfreezers" based on their FOG symptoms, looking at various clinical factors.
- *Results indicated that FOG was present in 15% of participants, with freezers showing more severe apathy and motor symptoms, but there were no significant differences in cognitive abilities or depressive symptoms between the groups.*
Background And Purpose: Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease. Selective deficiency of edited adenosine deaminase acting on RNA 2 (ADAR2), a key molecule in the acquisition of Ca resistance in motor neurons, has been reported in sporadic ALS (sALS) spinal motor neurons. Since ADAR2 activity is positively regulated by prolyl isomerase Protein never in mitosis gene A interacting-1 (Pin1), a known phosphorylation-dependent peptidyl-prolyl cis/trans isomerase, we investigated Pin1 expression in spinal motor neurons in sALS.
View Article and Find Full Text PDFBackground: Autoimmune cerebellar ataxia (AICA) is a general term for diseases in which the cerebellum is damaged by an autoimmune mechanism. For the diagnosis of the AICA, anti-thyroid antibodies (anti-thyroid peroxidase antibody and anti-thyroglobulin antibody), anti-glutamic acid decarboxylase (GAD) antibodies, and anti-gliadin antibodies are measured. Immunotherapy is known to be effective for AICA, but some patients with effective immunotherapy lack autoantibodies associated with cerebellar ataxia.
View Article and Find Full Text PDFBackground Cerebrovascular diseases are common comorbidities in patients with cancer. Although active cancer causes ischemic stroke by multiple pathological conditions, including thromboembolism attributable to Trousseau syndrome, the relationship between stroke and inactive cancer is poorly known. The aim of this study was to elucidate the different underlying pathogeneses of cryptogenic stroke in active and inactive patients with cancer, with detailed investigation by transesophageal echocardiography.
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- - The study aimed to analyze how the alignment of the spine and pelvis affects motor symptoms in patients with Parkinson's disease (PD), particularly focusing on differences between male and female patients
- - Researchers evaluated 79 patients with idiopathic PD, using spine imaging and the MDS-UPDRS to assess motor symptoms, finding significant correlations between these motor symptoms and various spine alignment parameters based on sex
- - Results indicated notable differences: in males, motor symptoms correlated with cervical alignment, while in females, correlations were found with thoracic kyphosis and other pelvic measures, highlighting the need for sex-specific considerations in treatment
Article Synopsis
- - An 84-year-old woman experienced persistent left hemichorea due to stenosis in the right middle cerebral artery (MCA), before a cerebral infarction occurred.
- - She presented to the hospital 9 days after the onset of hemichorea, which lasted for 19 days with no initial signs of cerebral infarction on MRI.
- - On day 21, she developed left hemiplegia and subsequent MRI revealed a cerebral infarction in the right putamen, indicating that MCA stenosis can cause hemichorea and may predict future infarctions.
Article Synopsis
- The study investigates how functional imaging findings, specifically I-FP-CIT-SPECT, relate to motor symptoms in early-stage Parkinson's disease (PD) patients.
- It analyzed 46 drug-naive patients, measuring specific binding ratios (SBRs) in brain regions related to motor function and evaluating symptoms with established scales and wearable sensors.
- Results showed that lower SBRs in the striatum and anterior putamen were linked to worse motor symptoms and gait disturbance, indicating a significant relationship between imaging findings and clinical features of PD.
Article Synopsis
- * After two days of treatment, she experienced decreased alertness and low sodium levels (hyponatremia), and a brain MRI revealed temporary swelling in the occipital lobes.
- * The findings suggested posterior reversible encephalopathy syndrome (PRES), with the onset likely linked to the IVIg therapy and the resulting hyponatremia.
In the pharmacotherapy of patients with Parkinson's disease (PD), entacapone reduces the peripheral metabolism of L-dopa to 3-O-methyldopa (3-OMD), thereby prolonging the half-life (t) of L-dopa and increasing the area under the concentration curve (AUC). The effect of entacapone on the pharmacokinetics of L-dopa differs between patients with high-activity (H/H) and low-activity (L/L) catechol-O-methyltransferase (COMT) Val158Met polymorphisms, but the effects are unclear in heterozygous (H/L) patients. 3-OMD has a detrimental effect and results in a poor response to L-dopa treatment in patients with PD; however, the influence of this polymorphism on the production of 3-OMD remains unknown.
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- * A 75-year-old man experienced right-sided hemiplegia (weakness) due to a stroke, accompanied by muscle pain and fever, leading to the EGPA diagnosis despite no history of asthma or allergies.
- * After starting corticosteroid treatment, the man's hemiplegia improved quickly, indicating that EGPA should be considered in similar cases of stroke combined with muscle pain and high eosinophil levels.
Article Synopsis
- The study examined the prevalence of PINK1 gene variants in 1,700 Parkinson's disease patients to understand their impact on the disease.
- Researchers found that patients with homozygous PINK1 variants had an earlier onset of Parkinson's, indicating a correlation between variant type and age of onset.
- Additionally, about half of the patients with heterozygous PINK1 variants showed reduced heart function, highlighting the clinical significance of these genetic variations in Parkinson's disease.