Neurocase最新文献

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.

To study a case of a middle-aged male with a non-tumor-associated Epstein-Barr virus (EBV) infection associated with Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE), to explore the role of EBV in the pathogenesis of anti-NMDARE. The patient was diagnosed with "Anti-NMDARE, EBV infection" by using Cerebrospinal fluid (CSF) autoimmune encephalitis profile, and Metagenomics Next-Generation Sequencing (mNGS) pathogenic microbial assays, we discuss the relationship between EBV and NMDARE by reviewed literature. EBV infection may trigger and enhance anti-NMDARE, and the higher the titer of NMDAR antibody, the more severe the clinical presentation.

A 23-year-old man presented with behavioral disinhibition, stereotypies, motor apathy, flattened affect, and inappropriate laughter. CT demonstrated generalized cerebral atrophy. He was admitted with a diagnosis of unspecified psychosis and discharged on antipsychotic medication. He was readmitted 3 months later, was diagnosed with schizophrenia, and antipsychotic medication was continued. Owing to symptom progression and aggressive behavior, he was readmitted 2 months later. CT again demonstrated moderate central and cortical cerebral atrophy. MRI showed severe, stable atrophy with frontotemporal predominance, and he was diagnosed with probable behavioral variant frontotemporal dementia (bvFTD).  Over the next year he rapidly deteriorated, with loss of cognitive abilities. Genetic testing revealed several variants, none of which are clearly disease-causing.

Introduction: We report on a musician who acquired synesthesia, enhanced sensory experience, and improved creativity following traumatic brain injury (TBI).

Background: Creativity and synesthesia can be acquired from an injury, though both simultaneously has not been frequently documented.

Narrative: This case report details heightened creativity and developing synesthesia in a 66-year-old right-handed man following TBI. He developed a "compulsion" to write music. Synesthesia included "seeing" the notation and being able to name chord structures of music he heard, both of which were novel experiences. The Synesthesia Battery revealed a vision-sound synesthesia with higher than average level of Vividness of Visual Imagery (VVIQ-2), and "Absolute Pitch/Perfect Pitch."

Patient experience: The patient experienced an approximate four-month history of these changes, including musical compositions, developing perfect pitch, and enhanced sensory experience of typical phenomena.

Discussion: Both creativity and synesthesia depend on novel connections in the brain, and both have been reported following insults to the brain, including in degenerative conditions. However, the development of both simultaneously is not frequently reported. Evidence for the etiology of one prompting the other has not been described. Brain injury may result in increased creativity and synesthesia. Our fields would benefit from increased awareness of this possible relationship.