Clinical Neurology最新文献

We conducted an additional analysis using the data from the post-marketing surveillance of Alglucosidase alfa for Pompe disease. We aimed to investigate the changes in the percentage of predicted forced vital capacity (%FVC) and the changes in the distance of the 6-min walk test (6MWT) by overall improvement and to investigate the %FVC change by the duration from symptom onset to survey registration (shorter/longer groups) using a linear mixed model. Thirty-seven and eighteen survey participants had %FVC and 6MWT data available, respectively; of the patients whose overall improvement was rated as "relatively improved," %FVC and 6MWT worsened in 71.4% and 66.7%, respectively. The %FVC at the survey registration estimated using a linear mixed model was significantly higher in the shorter group than in the longer group (P = 0.0413). The estimated slope of %FVC was significantly lower in the shorter group than in the longer group (P = 0.0051). These results suggest the importance of early treatment initiation and quantitative evaluation of each symptom.

This study aims to quantify the economic burden of Parkinson's disease (PD) in Japan. We conducted a targeted literature review and extracted data on the direct medical costs and long-term care costs of PD patients based on their severity, as categorized by the Hoehn and Yahr (HY) disease severity scale. These costs were estimated using information from literature sources and governmental statistics. The mean annual long-term care costs for HY1 to 5 were estimated: JPY 333 433, 527 194, 982 578, 2 023 735, and 3 080 743, respectively. These estimates were based on a cross table of HY severity and designated grade of long-term care, which was extracted from a survey of PD patients in Tokyo conducted in 2017 and national statistics on long-term care expenditure per person in June 2023. Assuming that the number of PD patients in Japan is 200,000, the annual costs for direct medical, long-term care, and the total are estimated approximately JPY 380, 270, and 650 billion, respectively. These results suggest that PD requires a significant amount of long-term care resources.

We retrospectively investigated mechanical thrombectomy (MT) in nonagenarians and older with cerebral infarction. Analysis of 21 consecutive patients showed favorable outcomes (improved modified Rankin scale [mRS] 0-2 or returned prestroke mRS) in 33.3% and poor outcome (mRS 5 or 6) in 52.4%. Factors related to favorable outcomes remain unknown, but the overall results of MT were good. MT is effective for the treatment of very elderly patients and further research is required to predict prognosis.

The Japanese Society of Neurology's Special Committee on Measures for Transition from Pediatric to Adult Health Care held a workshop to discuss the activities of the transitional care support centers (TCSCs). The following points were addressed: (1) from Kanagawa Prefecture, the activities of the TCSC, which is set up alongside the Intractable Disease Consultation Support Center and the Intractable Disease Information Coordination Center, separated from medical institutions, and the efforts addressing cases of difficult transitions and consultations where patients cannot transition from specific pediatric chronic diseases to designated intractable diseases; (2) from Nagano Prefecture, the supporting the health care transition undertaken by the neurologist as intractable disease medical coordinator, and (3) the efforts of the transitional health care support coordinator at the TCSC established at the university hospital in collaboration with the Nagano Children's Hospital and the government. For the creation of a seamless support system, we hope that the pioneering activities reported at this time will spread nationwide.

A patient with behavioral variant frontotemporal dementia presented with stereotypic speech. The 85-year-old right-handed man had progressive language disorder over 2 years, with changes in eating behavior. His verbal output consisted mainly of the stereotypic speech; his articulation was mildly distorted, but his speech was intelligible. Spontaneous speech was rare, but there was no evidence of difficulty in initiating speech or effort. MRI of the head showed atrophy of the left frontal lobe, mainly in the superior, middle, and inferior frontal gyrus. Cerebral blood flow SPECT showed decreased cerebral blood flow in the same areas and in the left basal ganglia. We diagnosed behavioral variant frontotemporal dementia based on the symptoms, progression, and lesions. The stereotypic speech was judged to be non-meaningful recurrent utterance.

Posterior spinal artery (PSA) infarctions are rare and challenging to diagnose in the acute phase. Herein, we report two cases of PSA infarctions diagnosed using spinal diffusion-weighted imaging-MRI (DWI-MRI). Case 1 involved a 74-year-old male patient presenting to our hospital with right leg numbness and unsteadiness while walking. Neurological examination revealed muscle weakness in the right lower limb and decreased vibration sensation in the dermadrome below the right Th8 level. Spinal DWI-MRI showed a high-intensity signal in the posterior right Th8, leading to the diagnosis of thoracic spinal cord infarction in the PSA region. In Case 2, a 70-year-old woman visited our hospital complaining of numbness of the left hand. Neurological examination revealed left-sided paresthesia exhibiting a positive Romberg's sign. Spinal DWI-MRI showed a high-intensity signal in the right C2 level posterior region, confirming the diagnosis of cervical spinal cord infarction in the PSA region. A neurological examination for PSA infarction and highlights the usefulness of a spinal cord DWI for auxiliary diagnosis.

A 65-year-old man presented to the emergency department with the complaints of left anterior chest and back pain, numbness in the left medial arm, and weakness in the left hand grip. Myocardial infarction was suspected, but later ruled out, and the patient was subsequently referred to our department. Among the intrinsic muscles, the left abductor pollicis brevis was the most severely weakened, and there was a sensory disturbance in the left T1 region and left Horner's sign. An MRI T₂-weighted image of the cervical spine showed a herniated disc on the left lateral side at the T1/2 level, suggesting compression of the T1 nerve root. Cervical angina is a rare, angina-like anterior chest pain due to cervical spine diseases. It is often reported in patients with C6 and C7 radiculopathy. The most severe weakness in the abductor pollicis brevis muscle in T1 radiculopathy is important to distinguish it from C8 radiculopathy.

A 67-year-old woman with past medical history of chronic myelomonocytic leukemia (CMML) presented with a chief complaint of headache, diplopia, and hearing impairment in the right ear. Examination revealed impaired ocular movement in the left eye and sensorineural hearing loss in the right ear. Cerebrospinal fluid analysis showed increased cell count and protein, and MRI showed contrast enhancement of hypertrophic dura mater. Since there were no other abnormalities which would have been a cause of hypertrophic pachymeningitis, it was considered as systemic autoimmune/inflammatory disorder (SAID) associated with CMML. Treatment with steroid, cyclophosphamide, and methotrexate led to improvement of the symptoms. SAIDs develop in up to 25% of patients with myelodysplastic syndromes (MDS) or CMML, which may be the only symptoms of MDS/CMML. As a phenotype of SAIDs, systemic vasculitis, connective tissue diseases, and neutrophilic diseases are frequently reported; however, isolated involvement of central nerve system is rarely reported. To our knowledge, this is the first report of hypertrophic pachymeningitis as SAID associated with CMML. To clarify the pathogenesis of neurologic involvement of SAIDs, accumulation of cases is necessary.