Clinical Neurology最新文献

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Syphilitic gumma is a rare manifestation of neurosyphilis that can cause mass lesions in the central nervous system. We present an atypical case of a 53-year-old man presenting with syphilitic gummas affecting both the brain and spinal cord. The patient presented with right facial numbness, worsening back pain, gait disturbances, and lower-limb weakness. Serological tests were positive for syphilis, and cerebrospinal fluid analysis showed elevated cell count, protein concentration, and positive syphilis tests. Brain and spinal cord MRI revealed dural-based enhancing mass lesions in the right middle cerebellar peduncle and conus medullaris. The patient underwent posterior decompression and biopsy of the conus medullaris. Histopathological findings excluded malignancy and were consistent with syphilitic gumma. The patient received intravenous benzylpenicillin, followed by oral amoxicillin, resulting in partial improvement of neurological symptoms and gradual regression of the lesions on follow-up MRI. This case highlights the importance of considering syphilitic gumma in the differential diagnosis of intracranial and spinal cord lesions in patients with syphilis. Prompt antibiotic treatment and serial MRI imaging are crucial for managing this condition.

Objective: Although population-based studies of hemiplegic migraine (HM) exist, large-scale clinic-based studies focusing on the detailed clinical characteristics of HM have not been reported. This study aims to define the clinical characteristics of HM in a tertiary care headache centre.

Methods: A retrospective analysis was conducted based on the medical records of HM patients.

Patients: This study included 163 consecutive HM patients who visited the National Hospital for Neurology and Neurosurgery between 2006 and 2013.

Results: According to the diagnostic criteria of International Classification of Headache Disorders (ICHD-3β), 142 patients were diagnosed with HM. Although 21 patients did not satisfy the diagnostic criteria, migrainous headaches with repetitive hemiparesis were reported and other disorders were excluded, hence these patients were clinically diagnosed with HM. The temporal progression of aura symptoms was atypical in 40 patients. The median duration of hemiparesis was 24 hours (interquartile range: 3-60 hours) which was far longer than that of previous population-based studies. The lifetime experience of an episode of motor aura exceeding 72 hours was reported in 51.6%. Hemiparesis was observed without full recovery in 9 patients (5.5%).

Conclusions: In many HM patients, the temporal progression of aura symptoms was diverse compared to typical descriptions in the literature, and the aura symptoms sustained longer than reported in the population-based study.

We present the case of a 69-year-old woman who underwent tracheostomy for advanced amyotrophic lateral sclerosis. The patient was treated with furosemide for leg edema. Body mass index was stable at 21.5 ‍kg/m². The patient was admitted to our hospital after vomiting because of biliary infection. Fluid therapy with 286 ‍kcal/day of glucose was administered, followed by acute deterioration, including tachycardia (120 bpm), glucose intolerance, abdominal pain, hypophosphatemia (required intravenous phosphate supply; 60 ‍mmol/day), and hypokalemia (required intravenous potassium supply; 60 mEq/day). Refeeding syndrome was suspected, and the patient recovered with adjustments in serum electrolyte levels. We demonstrated that glucose infusion can cause refeeding syndrome in patients with advanced amyotrophic lateral sclerosis without low nutritional intake.

In multiple sclerosis and neuromyelitis optica spectrum disorders, the risk of relapse can no longer be reduced to nearly zero. Consequently, the possibility of misdiagnosis must be considered when a relapse occurs. Nevertheless, managing these conditions requires efforts to minimize the risk of infection and avoid overtreatment. Looking ahead, future treatments may focus not only on enhancing myelin regeneration but also on addressing neuronal and axonal degeneration. Additionally, immune reconstitution therapy-designed to reset and rebuild the immune system's memory-may become a viable option. As such, it will be increasingly important to avoid excessive dosages and to tailor personalized therapies to meet the specific needs of each patient.

Fatigue is one of the most frequent non-motor symptoms associated with people with Parkinson's disease (PwPD). In this study, we investigated the relationship between fatigue and patients' background characteristics, disease severity, motor and non-motor symptoms, and cognitive and psychological assessments in PwPD. A total of 80 PwPD were included in this study, 40% of whom experienced fatigue. PwPD with fatigue were associated with being female, dyskinesia, and higher levels of levodopa equivalent daily dose, as well as increased severity of depression, apathy and sleep disturbances, in addition to lower quality of life. Fatigue in PwPD is a symptom that warrants clinical attention, as it is linked to multiple risk factors, indicating the need for further intervention studies that incorporate the perspective of personalized medicine.

The patient is a 45-year-old male. He has psoriasis vulgaris and thromboangiitis obliterans (TAO) as a comorbidity and was transferred to our hospital with dysarthria and right hemiparesis. On arrival, he presented with right hemispatial neglect, hemiparesis, and sensory disturbance. Head MRI showed scattered infarctions at the left middle cerebral artery territory, and 3D TOF MRA showed left middle cerebral artery occlusion. We performed mechanical thrombectomy with effective recanalization despite remained residual stenosis. Dual antiplatelet therapy was initiated, and neurological findings gradually improved. No apparent embolic source was identified including antibodies for vasculitis. He was diagnosed with large artery atherosclerosis with psoriasis and TAO as a vascular risk factor. Psoriasis is known to cause atherosclerosis and inflammatory disease by increasing cardiovascular risk. Contrast-enhanced MRI after three months of treatment for psoriasis showed decreased contrast signal at the stenotic lesion.

We report a case of Erdheim-Chester disease (ECD) presenting with recurrent mass lesions that posed significant diagnostic challenges. The patient, a 66-year-old man, had multiple recurrent intracranial lesions with contrast enhancement and edema. Given his history of international travel and partial responsiveness to antibiotics, imported infectious diseases were initially suspected. However, infectious etiology was ruled out based on histopathological examination, leading to a diagnosis of ECD. This case was atypical for ECD due to the imaging findings and recurrent nature of the lesions. When granulomatous lesions with histiocytic infiltration are non-infectious, histiocytic disorders should be considered, and immunohistochemical staining is recommended.

This manuscript complements the clinical course of the first case of neurosyphilis in our previous report (Kotani. et al. Clin Nuc Med 2024) which highlighted the utility of ¹⁸F-THK5351 positron emission tomography (PET), a marker of astrogliosis, to visualize neuroinflammation. The patient was a right-handed man in his early 60s who presented with a three-month history of forgetfulness and subsequent right hemiparesis. Neurological and neuropsychological examinations revealed the right pyramidal signs and impairments in attention, memory, executive function, visuospatial cognition, and verbal fluency. The patient was diagnosed with neurosyphilis based on positive tests for syphilis antibodies in the serum and cerebrospinal fluid (CSF) and elevated CSF cell and protein levels. MRI revealed multiple infarcted lesions that explained the pyramidal signs; however, the lesions responsible for cognitive impairment were not visualized. Two months after penicillin G treatment, the patient exhibited partial improvements in cognitive function, without obvious changes in MRI. To investigate the underlying neuroinflammation associated with astrogliosis, we performed PET imaging after treatment. ¹⁸F-THK5351 PET revealed increased uptake and ¹⁸F-fluorodeoxyglucose (FDG) PET showed decreased uptake in the left deep frontal white matter and thalamus. We believed that the right pyramidal signs were associated with infarctions contributed by meningovascular syphilis in addition to the arteriosclerosis, whereas cognitive impairment was associated with neuroinflammation due to parenchymal syphilis. Furthermore, the impairment of thalamocortical circuits may have compromised the widespread cortical excitability underlying cognitive impairments. This report highlights the utility of ¹⁸F-THK5351 PET imaging in understanding the pathogenesis of neurosyphilis, including cognitive impairment. Further longitudinal studies are required to elucidate the relationship between neuroinflammation and the clinical presentation of neurosyphilis.