The number of Parkinson’s disease (PD) patients has been increasing year by year in Japan. However, there are few reports that comprehensively evaluate the symptoms and treatment details of PD patients. We collected and analyzed information on PD patients regularly visiting the Department of Neurology at Saiseikai Matsuyama Hospital as of the end of October 2020. We included 187 patients (83 males and 104 females) with a mean age of 73.6 years and a mean disease duration of 8.9 years. The disease duration was positively correlated with Hoehn & Yahr (HY) stage and the number of antiparkinsonian drugs. The L-dopa equivalent dose decreased after 20 years of disease duration or HY 5. Wearing-off phenomenon and L-dopa-induced dyskinesia were more common in patients with longer duration of disease and higher daily dose of L-dopa. This study provides an overview of the clinical picture of PD patients in a regional core hospital.
We conducted the multicenter questionnaire survey targeting patients with Parkinson’s disease (PD) in order to investigate the impacts on their daily lives and their requests to hospitals in the pandemic of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Mainly using open-ended questionnaire, we asked their anxiety, troubles they are facing, and requests toward hospitals in the pandemic of SARS-CoV-2. Two hundred fifth-eight PD patients answered the questionnaire. There were various opinions about anxiety such as “PD patients are susceptible and vulnerable to SARS-CoV-2” (36.8%). Concerning the troubles in the pandemic, the most frequent answer was that they couldn’t participate in the rehabilitation and elderly day care (38.4%). Relatively many PD patients requested telemedicine (29.5%), whereas some people hoped face-to-face medical care (8.1%). There were demands about the delivery of medications (50.0%), the establishment of telephone consultations (43.8%), resources for rehabilitation at home (43.8%). The medical care adapted to the anxiety, trouble and requests of PD patients will be required in the era when we have to live with SARS-CoV-2.
Pain is one of the most frequent non-motor symptoms associated with Parkinson’s disease (PD) and it has a great impact on patient’s quality of life. Thus, its quantitative evaluation is critical in establishing therapeutic evidence. The King’s Parkinson’s Disease Pain Scale (KPPS) was introduced as a scale of pain specific to PD in 2015. As a follow-up to the evaluator-based KPPS, the patient-based questionnaire, the King’s Parkinson’s Disease Pain Questionnaire (KPPQ), was introduced in 2018. We developed a linguistically validated Japanese version of the KPPS and KPPQ, and the process of its construction is reported in this study.
A 73-year-old woman was admitted to our hospital owing to abnormal diurnal behavior, sudden brief episodes of impaired awareness, and loud nocturnal sleep talking. Her symptoms had developed gradually over several months and had been treated as dementia with Lewy bodies (DLB) at another clinic. Video-polysomnography revealed brief sleep talking and gross movements associated with REM sleep without atonia. 18F-FDG PET revealed increased glucose metabolism in both medial temporal lobes. These findings led to a diagnosis of limbic encephalitis (LE) comorbid with REM sleep behavior disorder (RBD). After two courses of intravenous methylprednisolone pulse therapy, her symptoms gradually improved. Her illness was later confirmed as anti-voltage-gated potassium channel (VGKC) complex/leucine-rich glioma-inactivated protein 1 (LGI1) antibody-associated LE using serum analyses. Clinical features of anti-VGKC complex/LGI1 antibody-associated LE can mimic those of DLB, particularly when comorbid with RBD.
A 65-year-old woman with a six-year history of paroxysmal nocturnal hemoglobinuria (PNH) was admitted due to weakness in the right leg following a seven-day history of fever and upper respiratory infection. MRI revealed several high-intensity areas in bilateral frontal lobe cortices and the left cerebellum on diffusion-weighted imaging, and signal hypointensity along the course of the cortical vein in the left frontal lobe on T2*-weighted imaging. We diagnosed cerebral venous thrombosis and brain infarction, and commenced heparin infusion. She developed right-sided dens hemiparesis on hospital day 6, when brain CT showed subcortical hemorrhage in the left frontal lobe. Despite eculizumab administration and decompressive craniectomy for hematoma, she died on hospital day 26. Thrombosis in PNH has been recognized as a life-threating complication, and intensive treatment including emergent administration of eculizumab is warranted if this situation arises.
A 54-year-old woman was diagnosed with acute adult T-cell leukemia (ATL) in November 2015 and underwent allogeneic hematopoietic stem cell transplantation in March 2016. Cognitive impairment appeared suddenly around May 2019, and MRI of the brain showed cerebral white matter lesions. Cerebrospinal fluid examination showed no significant findings other than elevated protein. Brain biopsy showed inflammatory cells, (mainly CD8-positive T lymphocytes), infiltrating the white matter. Based on the pathological findings and the history of chronic graft versus host disease (GVHD) in the lungs and intestines, we diagnosed central nervous system involvement of GVHD (CNS-GVHD). Immunotherapy with steroids and mycophenolate mofetil resulted in improvement of the cognitive dysfunction and inflammatory findings in the spinal fluid. This case is the first report of CNS-GVHD in ATL, suggesting the importance of diagnosis by brain biopsy and the efficacy of immunotherapy.
A 64-year-old woman experienced a broad brain infarction in the right middle cerebral artery territory with left hemiparesis and left unilateral spatial neglect. She was treated by endovascular thrombectomy and decompressive craniectomy. During the subacute phase of the brain infarction, she became alert and insisted, “My eldest daughter is at my feet and I can touch her body with my feet.” The patient’s abnormal experience was accompanied by visual information, such as her daughter’s physical characteristics and clothing, which could not be directly confirmed, and a false ideation that she had two eldest daughters. Although the patient was aware that her experience was abnormal, she was unable to deny her strong beliefs regarding the experience. We concluded that a series of the patient’s abnormal experiences consisted of both tactile hallucinations and delusions. In this case, the subjects of the delusions associated with the patient’s tactile hallucinations were limited to close relatives. Finally, it was clinically important that introspection by the patient revealed details of the nature of her hallucinations.
A 60-year-old woman, who had a kidney transplant 16 years ago, was admitted to our hospital owing to cognitive decline and gait disturbances. She developed ataxia, consciousness disturbances, and myoclonus, and died two years after the onset of symptoms. No specific findings were observed on MRI or in the cerebrospinal fluid and blood analyses. The patient was diagnosed with post-transplant lymphoproliferative disorder (PTLD) based on the results of the autopsy. Pathological findings revealed proliferating monoclonal B cells in the perivascular space that was confined to the central nervous system. PTLD is a serious complication of transplantation. Furthermore, PTLD of the central nervous system usually presents as nodular lesions on MRI. When neurological symptoms appear after transplantation, it is necessary to consider PTLD as a differential diagnosis even if abnormal findings cannot be pointed out on MRI.
A 75-year-old man with a history of temporal lobe epilepsy (treated with levetiracetam) was transferred to our hospital because of loss of consciousness. At admission, he was drowsy and exhibited myoclonus on the left side of face. We established a diagnosis of status epilepticus and started treatment with levetiracetam, fosphenytoin, and midazolam. FLAIR and DWI showed hyperintensity in the right cerebral cortex. Electroencephalography (EEG) showed lateralized periodic discharges (LPDs) at the right hemisphere, indicative of non-convulsive status epilepticus (NCSE). He regained consciousness after treatment with anti-epileptic drugs but showed persistent LPDs in EEG. MRI arterial spin labeling (ASL) showed normal perfusion in the right hemisphere; therefore, he was deemed to have recovered from status epilepticus and transferred to the rehabilitation hospital. MRI ASL is useful for diagnosing recovery from NCSE irrespective of sustained periodic discharges on EEG.