Transient phonemic paraphasia by bilateral hippocampus lesion in a case of limbic encephalitis

Although the hippocampus has not typically been identified as part of the language and aphasia circuit, recent evidence suggests that the hippocampus is closely related to naming, word priming, and anomic aphasia. A 59-year old woman with limbic encephalitis of possible autoimmune etiology, after recovery of consciousness, presented with severe memory impairment in both anterograde and retrograde modalities, episodes of fear, hallucination and convulsion, and transient fluent, phonemic paraphasia, together with small sharp waves diffusely by EEG. Brain MRI revealed bilateral symmetric, discrete lesions in the body to the infundibulum of the hippocampus

Isolated Hemiataxia and Cerebellar Diaschisis after a Small Dorsolateral Medullary Infarct

Isolated hemiataxia after a medullary infarct is rare. We describe a case of isolated hemiataxia after a small infarct localized at the ipsilateral dorsolateral medulla. An 83-year-old man developed acute onset of ataxia in the left arm and in both legs. Speech and extraocular movement were normal, and he did not have any other neurological manifestations. Brain MRI showed a small infarct localized at the left dorsolateral medulla, which involved the inferior cerebellar peduncle. 123ECD-SPECT showed hypoperfusion in the left cerebellar hemisphere without clear vascular territory. Neuroimaging findings for our patient suggested the involvement of the inferior cerebellar peduncle that projects to the cerebellum in our patient

Cerebellar Ataxia and Overactive Bladder after Encephalitis Affecting the Cerebellum

The cerebellum is one of the regions that contribute to urinary dysfunction in humans. A 43-year-old woman at age 35 had an acute onset of encephalitis that led to fever, generalized convulsion and coma. Six months after the disease onset, she regained consciousness and developed generalized myoclonus, cerebellar ataxia and overactive bladder, e.g., urinary urgency, daytime urinary frequency, and urinary incontinence. Eight years after the disease onset, she was revealed to have cerebellar atrophy on MRI, cerebellar hypoperfusion on SPECT, and detrusor overactivity on urodynamic study. Selective inflammation in the cerebellum seemed to produce cerebellar ataxia and overactive bladder in our case

Young-Onset Dementia with Lewy Bodies

Young-onset (< 65 years) dementia is a challenging clinical problem. A 61-year-old man visited our clinic because of a 2-year history of mild cognitive impairment of the executive disorder type. He was initially suspected of having young-onset Alzheimer’s disease due to the lack of motor signs or hippocampal atrophy by conventional brain MRI. However, he proved to have anosmia, erectile dysfunction, hypersexuality, constipation, REM sleep behavior disorder, and emotional lability; imaging findings included positive brain perfusion SPECT, nigrosome MRI, DAT scan, and MIBG myocardial scintigraphy. All these clinical imaging features led to the correct diagnosis of young-onset dementia with Lewy bodies (YOD-DLB). It is hoped that this case report will help facilitate a future prospective study to diagnose and follow YOD-DLB patients with the aim of determining appropriate management and care

Amantadine can Ameliorate Lower Urinary Tract Dysfunction and Nocturnal Polyuria in Patients with Parkinson Disease and Vascular Parkinsonism

Background:Amantadine is a drug used for patients with Parkinson\u27s disease (PD) and vascular parkinsonism (VP). These patients often have lower urinary tract symptoms (LUTS) and nocturnal polyuria (NP). Thus, we investigated the effect of amantadine on these in parkinsonian patients.Methods:Twenty-two patients with LUTS, including 13 with PD and nine with VP, were recruited. We performed a urinary questionnaire, frequency-volume chart, and residual urine (RU) measurement before and after daily administration of 150 mg and 300 mg amantadine.Results:Before amantadine administration, mean daytime urinary frequency was 9.07(standard error [SE], 0.64), nighttime urinary frequency 2.89 (0.24), urinary urgency per week 24.2 (6.69), urge incontinence per month 15.1( 9.94), urine volume per void 145.6( 12.6) mL, and residual urine volume 12.5( 6.30) mL. After daily 150 mg amantadine administration, mean daytime and nighttime urinary frequency, urinary urgency, and urge incontinence decreased to 6.9( 0.42), 1.97( 0.21), 13.0( 3.58), and 14.2( 10.2), respectively, and urine volume per void increased to 174.1( 11.3) mL. NP( N=8) was ameliorated in six patients. No patient had side effects. After daily 300 mg amantadine administration( N=8), mean daytime and nighttimeurinary frequency, urinary urgency, and urge incontinence decreased to 6.90 (0.33), 1.69 (0.10), 5.88 (1.61), and 2.31 (0.61), respectively, and urine volume per void increased to180.2 (15.0) mL. NP (N=4) was ameliorated in two patients. One patient developed hallucination, and two patients developed flashing sensation.Conclusion:Amantadine has beneficial effects on LUTS and NP in patients with VP and PD

DOCK2 is involved in the host genetics and biology of severe COVID-19

「コロナ制圧タスクフォース」COVID-19疾患感受性遺伝子DOCK2の重症化機序を解明 --アジア最大のバイオレポジトリーでCOVID-19の治療標的を発見--. 京都大学プレスリリース. 2022-08-10.Identifying the host genetic factors underlying severe COVID-19 is an emerging challenge. Here we conducted a genome-wide association study (GWAS) involving 2, 393 cases of COVID-19 in a cohort of Japanese individuals collected during the initial waves of the pandemic, with 3, 289 unaffected controls. We identified a variant on chromosome 5 at 5q35 (rs60200309-A), close to the dedicator of cytokinesis 2 gene (DOCK2), which was associated with severe COVID-19 in patients less than 65 years of age. This risk allele was prevalent in East Asian individuals but rare in Europeans, highlighting the value of genome-wide association studies in non-European populations. RNA-sequencing analysis of 473 bulk peripheral blood samples identified decreased expression of DOCK2 associated with the risk allele in these younger patients. DOCK2 expression was suppressed in patients with severe cases of COVID-19. Single-cell RNA-sequencing analysis (n = 61 individuals) identified cell-type-specific downregulation of DOCK2 and a COVID-19-specific decreasing effect of the risk allele on DOCK2 expression in non-classical monocytes. Immunohistochemistry of lung specimens from patients with severe COVID-19 pneumonia showed suppressed DOCK2 expression. Moreover, inhibition of DOCK2 function with CPYPP increased the severity of pneumonia in a Syrian hamster model of SARS-CoV-2 infection, characterized by weight loss, lung oedema, enhanced viral loads, impaired macrophage recruitment and dysregulated type I interferon responses. We conclude that DOCK2 has an important role in the host immune response to SARS-CoV-2 infection and the development of severe COVID-19, and could be further explored as a potential biomarker and/or therapeutic target