Neurocase最新文献

We report a case of posterior reversible encephalopathy syndrome (PRES) during treatment for alcohol withdrawal syndrome with gabapentin and clonidine. The patient developed severe hypertension, confusion and tremor, culminating in bilateral vision loss and a seizure. Imaging revealed posterior cerebral edema. Treatment with benzodiazepines, antihypertensives, and anti-seizure medications led to resolution. One year later, imaging showed resolution of the findings. We review the associated literature and propose the recognition of a PRES sub-entity, Alcohol-Related PRES (ARPRES), which can appear in the setting of alcohol withdrawal syndrome, chronic alcohol use, and acute alcohol intoxication, with or without hypertension.

In this study, Event-Related Potential (ERP) analyzes were performed to detect cognitive impairments in PD with Deep Brain Stimulation (DBS). A total of 85 volunteers underwent ERP analysis and neuropsychological testing (NPT) to determine cognitive level. In ERP analyses, prolonged latencies were observed in PD groups. However, patients implanted with DBS showed a decrease in latencies, a decrease in symptoms and statistical improvements in both cognitive and attention skills. Considering all these data, ERP results are promising as a noninvasive method that can be used in both disease status and diagnosis of PD.

Cataplexy is the sudden loss of muscle tone often provoked by emotion such as laughter or excitement. Cataplexy is one of the essential diagnostic features of Narcolepsy type 1 (NT1). We describe two cases of isolated cataplexy with different outcomes, highlighting the diagnostic and prognostic challenges. There is conflicting literature as to whether it is a legitimate standalone diagnosis or an early warning sign of narcolepsy. Our cases do not fit with current diagnostic criteria for narcolepsy, yet still share some clinical or laboratory features. These ambiguous cases question what the mechanistic relationship between narcolepsy and cataplexy may be.

Alzheimer's Disease (AD) is the most common cause of dementia, although multiple pathologies are found in nearly half of the cases with clinically diagnosed AD. Prion diseases, such as Creutzfeldt-Jakob disease (CJD), are rare causes of dementia and typically manifest as a rapidly progressive dementia, where symptom onset to dementia most often occurs over the course of months. In this brief report, we describe a patient's typically progressive dementia with a precipitous decline at the end of their life who, on neuropathological evaluation, was found to have multiple neurodegenerative proteinopathies as well as spongiform encephalopathy due to CJD. This case of unsuspected CJD highlights a rare, but epidemiologically important, cause of sudden decline in well-established neurodegenerative dementias.

Alice in Wonderland syndrome (AIWS) is a rare perceptual disorder characterized mainly by perceptual distortions of visual objects and one's own body. While there are many case reports of visual and somatosensory distortions associated with AIWS, little is known about auditory distortion. Therefore, we present the case of a 22-year-old right-handed woman who described having auditory as well as visual and somatosensory distortion experiences and a family history of AIWS. The subject reported experiencing multisensory perceptual distortions, where she sees other people's faces as larger and hears their voices as louder at the same time. This particular case suggests that auditory distortion - which contributes to constructing the perception of the surrounding space and the body - may also be characterized as a perceptual symptom of AIWS.

Prognosis after carbon monoxide (CO) poisoning is difficult to assess using structural images. Functional connectivity provided by functional magnetic resonance imaging (fMRI) may explain the mechanism of differential prognosis. We report here two cases of carbon monoxide poisoning with simultaneous coma. They were nearly normal on days 7-8, but diagnosed with delayed neurological sequelae (DNS) with cognitive and motor impairments on days 22-29. Similar Methylprednisolone pulse therapy and hyperbaric oxygen therapy were given to them. The movement disorder of case 1 improved slightly during the recovery stage, while the movement disorder of case 2 worsened significantly. In case 1, the function of supplementary motor area decreased first and then increased, and the function of pallidum increased first and then decreased. Case 2 showed a reduction in the supplementary motor area and small changes in the pallidum after DNS, but both were reduced during recovery stage. The cognitive ability of case 1 remained poor, while that of case 2 improved during the recovery stage. FMRI showed damage to the right and bilateral hippocampus in case 1 and partial damage to the left hippocampus in case 2. Taken together, fMRI can be a useful method to study functional connectivity abnormalities corresponding to different prognoses.

Most individuals recover quickly from a concussion; however, youth who sustain multiple concussions may be at risk for long-term cognitive impairments. This case study examines the neuropsychological performance of a 13-year-old malewith five head injuries. After his first concussion during study participation (fourth injury overall), several improvements were observed, likely due to practice effects, yet after sustaining another concussion <2 years later,declines were observed in visuoconstruction, verbal memory, and intellectual functioning. Across serial re-evaluation, his vocabulary knowledge declined, and fewer improvements were observed than anticipated when accounting for serial practice effects, highlighting the possible cumulative impact of multiple concussions.

Erotomania (de Clérambault's syndrome) refers to the delusional belief that another person, usually socially unreachable, is in love with the holder of the delusion. The occurrence of erotomania in Frontotemporal Dementia has rarely been reported. We present the unique case of a 59-year-old woman with a strong family history of early-onset dementia in whom erotomania was the initial manifestation that led to a diagnosis of definite Behavioral Variant of Frontotemporal Dementia with a pathogenic missense mutation in the MAPT gene. Based on this case, we propose a hypothetical model for developing erotomania in patients with FTD.

A 69-year-old Japanese male presented with acute dystextia and dystypia, defined as texting and typing impairments, respectively. His text input speed decreased due to a phonologically incorrect kana flick input on his smartphone. Additionally, dystypia occurred due to phonemic paragraphia of Romaji. Brain MRI revealed a new left lenticulostriate infarction sparing the cerebral cortex. SPECT showed reduced cerebral blood flow in the left inferior precentral frontal gyrus as well as in the infarction area. It was concluded that his abnormal phonological processes resulted from hypoperfusion in the left inferior precentral gyrus that is assumed to be an endpoint of the arcuate fasciculus.

The piriform cortex (PC) is part of the olfactory system, principally receiving input from the lateral olfactory tract and projecting to downstream components of the olfactory network, including the amygdala. Based on preclinical studies, PC is vulnerable to injury and can be easily kindled as an onset site for seizures. While the role of PC in human epilepsy has been studied indirectly and the subject of speculation, cases of demonstrated PC seizure onset from direct intracranial recording are rare. We present a pediatric patient with drug-resistant focal reflex epilepsy and right mesial temporal sclerosis with habitual seizures triggered by coconut aroma. The patient underwent stereoelectroencephalography with implantation of olfactory cortices including PC, through which we identified PC seizure onset, mapped high-frequency activity associated with presentation of olfactory stimuli and performance on cognitive tasks, and reproduced habitual seizures via cortical stimulation of PC. Coconut odor did not trigger seizures in our work with the patient. Surgical workup resulted in resection of the patient's right amygdala, PC, and mesial temporal pole, following which she has been seizure free for 20 months without functional decline in cognition or smell. Histological findings from resected tissue showed astrogliosis and subpial gliosis.