After experiencing two ischemic brain attacks, a 40-year-old, right-handed, female amateur cello player developed apraxia of speech (AOS) and amusia, especially on the aspect of the beat. Her tempo became gradually faster, but while playing, she could not recognize that. When she listened to her own recorded performances, she could identify the differences immediately and precisely. Brain MRI showed lesions in the right superior temporal lobe, temporal plate, and right and left precentral gyri. Neuromusicological assessments revealed severe beat impairment and moderate pitch impairment. We diagnosed her with beat deafness of music, possibly caused by the bilateral precentral gyri lesions.

We report a case of a 39-year-old woman with persistent vegetative state (PVS) following cardiac arrest and a right lower extremity fracture. Despite comprehensive rehabilitation for over 4 months, there was no improvement. Neuroelectrophysiological and neuroimaging assessments showed poor brain function, and the coma recovery scale-revised scale results suggested low arousal probability. Furthermore, patients with PVS exhibit autonomic reactivity and physiological responses to external stimuli. It is important to note that while these reactions may manifest as responsiveness to external stimuli, they should be interpreted as automatic physiological responses rather than indicative of consciousness.

Niemann-Pick Type-C (NPC) disease, an autosomal recessive lysosomal storage disorder, is associated with a spectrum of neuropsychiatric manifestations, including catatonia, which may not respond to conventional treatment. Here we report the case of a patient with adult-type NPC disease who developed catatonia and experienced recurrent catatonic episodes after the administration of antipsychotics required to treat active psychotic symptoms. Despite unsuccessful attempts with lorazepam, clozapine, and memantine to treat the catatonic symptoms, the patient showed improvement with electroconvulsive therapy (ECT). Catatonia recurred shortly after ECT discontinuation and improved with resuming ECT and adding lorazepam. However, catatonia recurred when the ECT frequency was reduced. We were able to resolve the catatonia with a combination of ECT and lorazepam. However, when we reduced the frequency of ECT sessions, the catatonia recurred despite continued lorazepam treatment. Remarkably, the addition of lamotrigine to the patient's treatment resulted in complete remission with no further recurrence of catatonia for 8 months. Our case highlights lamotrigine's mood-stabilizing effect and possible anti-NMDA effect in treating and preventing recurrent catatonia.

Psychotic symptoms, characterized by hallucinations, delusions, and cognitive disturbances, are associated with various psychiatric and neurological disorders. This manuscript explores two cases of acute psychotic episodes triggered by the regular consumption of herbal products. The cases highlight the need for increased awareness of the potential toxic side effects of herbal products. The impact of herbal ingredients like maca and matcha on neurotransmitter activity is explored, shedding light on the underlying mechanisms leading to psychosis. The manuscript highlights the need to report both the benefits and risks of herbal products, challenging the misconception that they are inherently safe.

Executive function (EF) deficits are common in epilepsy and impact quality of life. This study compares EF in idiopathic generalized epilepsy (IGE) and temporal lobe epilepsy (TLE) patients to healthy controls. Fifty-six IGE patients, 56 TLE patients, and 60 controls (matched by age) completed cognitive tests assessing attention, memory, learning, and verbal fluency. Both epilepsy groups performed worse than controls, with TLE patients showing significantly poorer verbal learning and memory compared to IGE patients. These findings suggest that TLE patients have more severe EF impairments, likely due to focal temporal lobe dysfunction and disruptions in EF networks.

Colpocephaly, a cause of hydrocephalus, is characterized by disproportionately enlarged occipital horns of the lateral ventricles. It is rarely seen in the older adults. Few cases were reported as colpocephaly in elderly individuals in the literature. However, it has not been reported as idiopathic normal pressure hydrocephalus-like clinical presentation in older adults. To the best of our knowledge, the co-existence of colpocephaly and Alzheimer's disease has not been reported previously. In these respects, it is the first case in the literature.

The case study explores bilingualism and neurodegenerative disorders, specifically progressive supranuclear palsy (PSP) with speech and language disorder (PSP-SL). It features a 78-year-old Mexican American woman who exhibits echolalia only in response to Spanish. This selective impairment suggests unevenly affected language control mechanisms despite her proficiency in both languages. Cognitive function is evaluated with neuropsychological tests; she's diagnosed with PSP-SL, depression, and anxiety. Echolalia in response to one language implies complex phonological retrieval mechanisms. Such observations prompt further inquiry into bilingual language control and processing mechanisms. The case supports evidence that bilingualism may attenuate neurodegeneration effects, suggesting better inhibitory control over disinhibited speech through enhanced executive functioning benefits.

Herein, we report a 62-year-old female patient with Multiple system atrophy (MSA) at whom the sympathetic skin responses (SSRs) were absent at initial investigations. However, the levodopa therapy provided normalization of SSRs and moderately improvement in orthostatic hypotension-related symptoms. Based on this rare illustration, we discuss the possible mechanisms underlying the pathophysiology of autonomic dysfunction in MSA. We remark on the need for future clinical and experimental studies in this field.

Moral cognition has largely been studied via dilemmas in which making a utilitarian choice causes instrumental harm (negative dimension). Studies of utilitarianism link this behavior with socioemotional unresponsiveness. However, there is a positive dimension of utilitarianism in which one sacrifices the good of oneself or close others for the overall welfare. We measured utilitarian choices multidimensionally in a patient with behavioral variant frontotemporal dementia (bvFTD), incorporating dilemmas accounting for negative and positive dimensions. Despite socioemotional deficits our patient was highly utilitarian in the positive, dimension of utilitarianism. This case study challenges the tendency to automatically associate bvFTD with antisocial tendencies.

Although handwriting impairment is a frequent sign of Parkinson's disease (PD), its significance in the evaluation processes of these patients may be overlooked among physicians. Therefore, we would like to report an illustrative patient who presented with isolated micrographia initially; but received the diagnosis of PD in the follow-up.

Cenobamate (CNB) is one of the newer antiseizure medications for the treatment of focal-onset seizures. The cognitive profile of CNB is not yet known in detail. Here we present the case of an 18-year-old male high school student with epilepsy who received adjunctive CNB. Under 400 mg/d of CNB in combination with lamotrigine, a neuropsychological reassessment revealed a severe deterioration of the formerly normal episodic memory functions, while executive functions remained unaffected. The de novo memory deficit had already led to a collapse in school performance and he unexpectedly failed to obtain the general qualification for university entrance. Given the beneficial effect of CNB on seizure control, a dose reduction of CNB to 200 mg/d and introduction of valproic acid was performed. This led to a full recovery of objective memory performance. To our knowledge this is the very first report of a dose-dependent, selective and severe decline in episodic memory performance under CNB, potentially impeding academic achievement. The findings call for a cognitive monitoring of CNB which also addresses episodic memory in addition to executive functions. Systematic studies on episodic memory upon CNB treatment would help to appreciate the scope of this apparently reversible adverse effect.

Neurodegeneration with brain iron accumulation (NBIA) is a genetic disorder characterized by iron accumulation in the basal ganglia. Patients may develop behavioral abnormalities, dementia, movement disorders, and neuropsychiatric symptoms such as emotional lability, depression, anxiety, hallucinations, impulsivity, obsessions, and hyperactivity. In this case, a 46-year-old male patient with a C19orf12 mutation experienced depressive complaints before movement disorders, followed by cognitive deficits and psychotic symptoms as the disease progressed. The patient's response to quetiapine treatment is crucial for managing neuropsychiatric symptoms. This case could contribute to the literature on presentation, differential diagnosis, and management of neuropsychiatric symptoms in rare NBIA patients.

This study explores Vincent van Gogh's struggles with executive functions and his experiences with episodes of depression. In the final years of his life, Van Gogh encountered visual and auditory hallucinations, often lasting for few days. His correspondence reveals his concerns about the clarity of his mind during this period, suggesting the possibility of visual hallucinations influencing his artistic work. He mentioned the term "delirium" in his letters, indicating the presence of a brief psychotic episode. This study also considers the potential influence of factors like nutrition, tobacco, alcohol consumption, somatic diseases, and psychological distress on his susceptibility to delirium.

Disorganized behavior is a primary sign of schizophrenia. Yet it is not unique to schizophrenia. Recently, there has been a growing understanding that endogenous mental disorders could be a prodromal stage of neurodegenerative dementia. As a result, the connection between endogenous mental disorders and dementia has become a subject of significant interest. In the present study, a 49-year-old female with schizophrenia since the age of 17 was diagnosed with early-onset Alzheimer's disease after a detailed examination with PET and cerebrospinal fluid, despite suspicion of organic disease due to progressive forgetfulness, abnormal behavior and delusions. Schizophrenia is not the only disorder in which disorganized behavior is seen. When such a clinical phenomenon develops, other possible causes should be considered. Dementia is one of the most important of these causes. In a psychiatric disorder with disorganized behavior, other possible causes, especially dementia, should be considered.

To describe a case of Post-Treatment Lyme Disease Syndrome (PTLDS) with an atypical cognitive profile.

There is uncertainty about whether delusion formation in Alzheimer's disease (AD) can be explained by false memories. "Metamemory," the ability to self-evaluate memory and identify memory errors, is impaired in people with delusions in schizophrenia. Our objective was to investigate whether false memory and metamemory were associated with delusions in AD. Participants with mild AD, with and without delusions, completed a computerized word recognition task and a metamemory measure. Group differences were compared using independent-samples t-tests or Mann Whitney tests. Significant findings were explored through binary logistic regression modeling. Participants with delusions ( = 10) gave more high confidence responses, significantly so for correct responses; percentage of high confidence correct responses for those with delusions (mean (SD)) was 69.7% (31.0%) and for those without ( = 14) was 43.5% (29.9%);  = -2.09,  = .049. This remained significant when sex was included in regression modeling; for each 1.0% increase in high confidence correct responses, participants were 5.4% more likely to have delusions (Exp(β) 1.054, 95% CI 1.007-1.105,  = .025). Findings provide tentative support for a link between metamemory and delusions in AD. This should be explored in a larger sample as it has potential implications for treatment.

Susac syndrome is a rare immune-mediated endotheliopathy affecting the microvasculature. It presents three main symptoms: encephalopathy, branch retinal artery occlusions, and hearing loss . Here we present a new case report focusing on the evolution of the cognitive profile. The patient underwent two neuropsychological examinations. The first, one month after the onset of the symptomatology, highlighted a prevalent involvement of verbal executive functions. The second, conducted six months later, revealed a global improvement in most previously deficient areas, although with the persistence of a difficulty in cognitive estimation. . This case illustrates the importance of a comprehensive analysis of patients with Susac syndrome to appreciate the whole range of cognitive deficits and reliably evaluate symptom evolution.

Emotional processing has been attracting increased attention. We herein present three patients with intractable epilepsy in whom emotional responses were elicited by electrical cortical stimulation. Negative feelings were produced in all patients; however, the content of each differed. In patient 1, the stimulation of the right anterior cingulate gyrus elicited unpleasant thoughts. In patient 2, the stimulation of the right posterior cingulate gyrus evoked anxiety. In patient 3, the stimulation of the right prefrontal cortex induced anxiety associated with a somatic sensation. Emotional responses comprise various aspects and the cortical areas responsible vary depending on each emotional aspect.

A diagnosis of behavioral variant frontotemporal dementia (bvFTD) often relies on informant reports of significant behavioral changes. "BvFTD-by-proxy" describes situations of neuropsychiatric changes reported solely by an informant under circumstances that may raise questions regarding their objectivity. We present three cases of bvFTD-like symptoms reported by spouses, where progression was unclear, testing showed mild but stable executive dysfunction, and neuroimaging was unremarkable. The subjective nature of bvFTD criteria leaves patients vulnerable to misleading informant reports, especially amid relational discord, and may threaten patient autonomy. Recognizing and managing this situation is critical but time-consuming, often requiring coordinated care across multiple providers.

L-2-hydroxyglutaric aciduria (L-2-HGA) is a rare autosomal recessive disease characterized by elevated levels of hydroxyglutaric acid in the body fluids and brain with abnormal white matter. We present two siblings with psychomotor retardation and quadriparesis. Their brain imaging showed diffuse bilateral symmetrical involvement of the cerebral cortex, white matter, basal ganglia and cerebellum. The whole exome sequence studies revealed a homozygous likely pathogenic variant on chromosome 14q22.1 (NM_024884.2: c.178G > A; pGly60Arg) in the gene encoding for L-2-hydroxyglutarate dehydrogenase (L2HGDH) (OMIM #236792). Therefore, using the L2HGDH gene study is beneficial for L2HGA diagnosis.