Neural activation for actual and imagined movement following unilateral hand transplantation: a case study.
We report functional magnetic resonance imaging (fMRI) findings for neural activation related to actual and imagined movement, for a 54-year-old male patient, who had received a donor hand transplant 50 years following amputation. Two assessments, conducted 3 months and 6 months post-operatively, demonstrate engagement of motor-control related brain regions for the transplanted hand, during both actual and imagined movement of the fingers. The intact hand exhibited a more intense and focused pattern of activation for actual movement relative to imagined movement, whereas activation for the transplanted hand was more widely...
Source: Neurocase - September 24, 2019 Category: Neurology Authors: Madden DJ, Melton MS, Jain S, Cook AD, Browndyke JN, Harshbarger TB, Cendales LC Tags: Neurocase Source Type: research

Transient naming deficits associated with insular lesions in a patient with encephalitis.
We describe a patient with acute herpes simplex encephalitis with left-hemispheric hippocampal, parahippocampal and insular lesions. Although prototypic language areas were unaffected, the patient suffered from an inability to name objects or animals displayed on pictures. This deficit was transient and gradually disappeared 8 weeks after the initial diagnosis. Our findings are in line with a previous report showing similar deficits in a patient with a comparable lesion pattern and support the hypothesis that left insular lesions can produce severe naming deficits. Using FDG-PET we ruled out that functional deactivati...
Source: Neurocase - September 18, 2019 Category: Neurology Authors: Marxreiter F, Utz K, Schlachetzki JCM, Seifert F, Schmidt M, Doerfler A, Schwab S, Schenk T Tags: Neurocase Source Type: research

Similar neurocognitive patterns in patients treated with lenalidomide: chemobrain effect?
Conclusions: Lenalidomide treatments can determine severe cognitive impairments especially in elderly patients. Our data suggest the need for a careful evaluation of cognitive decline risk before and after drug administration. However, larger studies are required to confirm our findings. PMID: 31522586 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - September 15, 2019 Category: Neurology Authors: Calvi E, Marchetti M, Santagata F, Luppi C, Coppo E, Massaia M, Isaia GC Tags: Neurocase Source Type: research

Is social cognitive training efficient in autism? A pilot single-case study using the RC2S+ program.
Abstract In high-functioning autism, deficits in emotional processing and theory of mind are relevant to understanding the particularities of social functioning. Here we used a multiple baseline and ABA single-case design to assess the efficacy of an individualized social cognitive training program using both pen-and-paper and computerized materials for an 18-year old patient. After the treatment phase, we found significant improvement in both emotional processes and theory of mind. These results provide further significant data showing that therapeutic tools based on digital relational simulation are a promising ...
Source: Neurocase - September 15, 2019 Category: Neurology Authors: Peyroux E, Franck N Tags: Neurocase Source Type: research

A shrunken world - micropsia after a right occipito-parietal ischemic stroke.
We examined a patient who, after a right occipito-parietal stroke, subjectively reported perceiving everything at seventy percent of the actual size. Using experimental tasks, we confirmed the extent of his micropsia at 70%. Visual half-field tests showed an impaired perception of shape, location and motion in the left visual field. As his micropsia concerns the complete visual field, we suggest that it is caused by a higher-order compensation process in order to reconcile the conflicting information from the two hemifields. PMID: 31462163 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - August 28, 2019 Category: Neurology Authors: Van Den Berg NS, Huitema RB, Spikman JM, Van Laar PJ, De Haan EHF Tags: Neurocase Source Type: research

The importance of time limits in detecting signs of left visual peripersonal neglect: a multiple single-case, pilot study.
Abstract We developed a new visual search test to assess signs of left visual peripersonal neglect. Five right-hemisphere-damaged patients, 10 healthy controls, and 10 orthopedic controls were administered the test in four conditions: easy task (no distractors)/time-limited (45''), easy task (no distractors)/time-unlimited, difficult task (distractors)/time-limited (45''), difficult task (distractors)/time-unlimited. With respect to controls, most RHDP showed signs of left visual peripersonal neglect in the time-limited condition, but not in the time-unlimited condition, particularly on th...
Source: Neurocase - August 26, 2019 Category: Neurology Authors: Priftis K, Di Salvo S, Zara D Tags: Neurocase Source Type: research

Cognitive characterization of SCAR10 caused by a homozygous c.132dupA mutation in the ANO10 gene.
allo F, Montón F Abstract Autosomal recessive spinocerebellar ataxia type 10 (SCAR10) caused by a homozygous c.132dupA mutation in the anoctamin 10 gene is infrequent and little is known about its cognitive profile. Three siblings (1 male) with this mutation were assessed with a neuropsychological battery measuring multiple cognitive domains. The deficits observed in one patient were in executive functions whereas the other two patients showed deficits in practically all the functions. Cognitive impairment seems to be a characteristic of the SCAR10 produced by this mutation, with a range from mild impairmen...
Source: Neurocase - August 19, 2019 Category: Neurology Authors: Nieto A, Pérez-Flores J, Corral-Juan M, Matilla-Dueñas A, Martínez-Burgallo F, Montón F Tags: Neurocase Source Type: research

Aprosodia and prosoplegia with right frontal neurodegeneration.
We describe a patient with progressive aprosodia and prosoplegia who had right greater than left perisylvian and temporal atrophy with an anterior predominance. We discuss the importance of assessing affective prosody and facial expression to arrive at an accurate clinical diagnosis. PMID: 31335278 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - July 23, 2019 Category: Neurology Authors: Bateman JR, Filley CM, Ross ED, Bettcher BM, Hubbard HI, Babiak M, Pressman PS Tags: Neurocase Source Type: research

Pure topographical disorientation in novel environments without anterograde amnesia: a case study.
Abstract Topographical disorientation (TD) in novel environments is considered to be a part of anterograde amnesia. A 56-year-old woman presented with pure TD only in novel environments following limbic encephalitis. She could not remember directions inside the hospital on weekly outpatient visits; however, her verbal and visual anterograde memories were normal. In the test of learning photographs of scenes, faces, and objects, only her scores for landscapes were worse than those in healthy controls. These findings suggested that her TD specific to landscapes and directions in novel environments was caused by cate...
Source: Neurocase - July 12, 2019 Category: Neurology Authors: Oishi Y, Yamakawa T, Nagasawa H, Suzuki K Tags: Neurocase Source Type: research

Pure alexia with intact perception of complex visual stimuli: a case study.
Abstract After a stroke involving the left occipitotemporal cortex our patient shows a word-length effect and has problems to identify letters or numbers in strings of symbols. But he is normal in identifying isolated letters and in non-verbally categorizing even complex images such as faces or natural scenes. His cortical lesion is stretching from the visual word form area (VWFA) anteriorly causing additional problems to name visual stimuli and to match acoustic stimuli with images. We conclude that our patient suffers from pure alexia without deficits to identify even complex visual stimuli. Our results directly...
Source: Neurocase - July 6, 2019 Category: Neurology Authors: Spang KM, Grimsen C, Brunner F, Fahle MW Tags: Neurocase Source Type: research

Verbal memory and sentence comprehension in aphasia: A case series.
Abstract This case series explores the relationship between verbal memory capacity and sentence comprehension in four patients with aphasia. Two sentence comprehension tasks showed that two patients, P1 and P2, had impaired syntactic comprehension, whereas P3 and P4's sentence comprehension was intact. The memory assessment tasks showed that P1 and P2 had severely impaired short-term memory, whereas P3 and P4 performed within the normal range in the short-term memory tasks. This finding suggests an association between short-term memory deficit and sentence comprehension difficulties. P1 and P3 exhibited impaired c...
Source: Neurocase - July 4, 2019 Category: Neurology Authors: Varkanitsa M, Kasselimis D, Boulouis G, Fugard AJB, Evdokimidis I, Druks J, Potagas C, Van de Koot H Tags: Neurocase Source Type: research

Forced person-following: a new type of stimulus-bound behavior.
We report a new type of stimulus-bound behavior, denoted forced person-following, which we documented for a patient with hypoxic encephalopathy following a suicide attempt with carbon monoxide poisoning. The patient's brain was damaged in the bilateral frontal, parietal, and temporal lobes and in the basal ganglia. The patient was compelled to follow any person who came into his sight and would continue to do so until the person went out of his sight. The patient also exhibited certain primitive reflexes. The forced person-following exhibited by our patient appears to be a consequence of stimulus-bound behavior due to fron...
Source: Neurocase - July 3, 2019 Category: Neurology Authors: Funayama M, Takata T Tags: Neurocase Source Type: research

Clinical features of bipolar disorder with idiopathic basal ganglia calcification: a review of case reports in the literature.
Abstract Although idiopathic basal ganglia calcification (IBGC) is associated with various neuropsychiatric disturbances including several cases of bipolar disorder (BD), there has been no systematic review of clinical features of patients with BD and comorbid IBGC. We undertook a literature search to identify case reports of these patients. Most cases showed complex syndromes comprising not only mood disturbance but also cognitive disability and motor symptoms limited to depressive state and had favorable treatment response. These patients should have a careful and repeated psychiatric, neurological, and cognitiv...
Source: Neurocase - July 2, 2019 Category: Neurology Authors: Ishitobi M, Kumashiro N, Nakao K Tags: Neurocase Source Type: research

High-frequency repetitive transcranial magnetic stimulation to the left dorsolateral prefrontal cortex of patients with Parkinson's disease and treatment-resistant depression: a pilot study.
Abstract An increasing amount of evidence is showing the therapeutic effects of rTMS on PD-related non-motor functions neuroanatomically linked to the DLPFC. This presents an ongoing need to apply an optimal combination of stimulation parameters to clinically heterogeneous patient populations, including those with neuropsychiatric problems and other comorbidities along with the neurodegenerative process. In this prospective pilot study, six patients with PD and treatment-resistant depression were thoroughly assessed and carefully monitored before, during, and after each stimulation procedure. The results can provi...
Source: Neurocase - July 1, 2019 Category: Neurology Authors: Randver R, Davel K, Toomsoo T Tags: Neurocase Source Type: research

A MEG study of the neural substrates of semantic processing in semantic variant primary progressive aphasia.
rt S Abstract Despite a well-documented pattern of semantic impairment, the patterns of brain activation during semantic processing in semantic variant primary progressive aphasia (svPPA) still remain poorly understood. In the current study, one svPPA patient (EC) and six elderly controls carried out a general-level semantic categorization task while their brain activity was recorded using magnetoencephalography (MEG). Despite similar behavioral performance, EC showed hyperactivation of the left inferior temporal gyrus (ITG) and right anterior temporal lobe (ATL) relative to controls. This suggests that periatroph...
Source: Neurocase - July 1, 2019 Category: Neurology Authors: Pineault J, Jolicœur P, Grimault S, Lacombe J, Brambati SM, Bier N, Chayer C, Joubert S Tags: Neurocase Source Type: research

The effects of prosody on sentence comprehension: evidence from a neurotypical control group and seven cases of chronic stroke.
Abstract Both prosody and sentence structure (e.g., canonical versus non-canonical) affect sentence comprehension. However, few previous studies have examined a possible interaction between prosody and sentence structure. In adult controls we found a significant interaction: typical sentence prosody, versus list prosody, facilitated comprehension of only some sentence structures. In seven stroke patients, impaired attentional control was related to impaired comprehension with sentence prosody but not list prosody; impaired working memory was related to impaired comprehension with list prosody, but not sentence pro...
Source: Neurocase - June 26, 2019 Category: Neurology Authors: LaCroix AN, Blumenstein N, Houlihan C, Rogalsky C Tags: Neurocase Source Type: research

Breast cancer plus adjuvant chemotherapy- related cognitive impairment: a case study.
This study longitudinally investigated cognitive function in an individual before and after breast cancer diagnosis and treatment. Comparison of test scores pre- and post- diagnosis/treatment revealed declines in multiple cognitive domains, including Performance IQ, processing speed, immediate auditory and visual memory, and delayed visual memory. Twelve years later, improvements were noted in domains of processing speed, memory and executive functioning. The results highlight the importance of heeding individuals' subjective reports of cognitive decline, and offer hope that such decrements may not be permanent. PMID:...
Source: Neurocase - June 25, 2019 Category: Neurology Authors: Fischer B Tags: Neurocase Source Type: research

Successful treatment of psychosis in a patient with Kufor-Rakeb syndrome with low dose aripiprazole: a case report.
We present a case of a 32-year-old male with Kufor-Rakeb syndrome (KRS), a form of juvenile parkinsonism due to mutations of the ATP13A2 gene at PARK9 locus. The patient was seen for daily behavioral outbursts and psychotic symptoms. At first assessment, CGI scale was estimated at 5; "Markedly ill". Aripiprazole was started at 2 mg and then increased to 3 mg. Two years later, psychotic symptoms were judged to be "much improved" (CGI-C = 2). This significant improvement without drug-induced motor side effects suggests that aripiprazole at low doses&n...
Source: Neurocase - June 24, 2019 Category: Neurology Authors: McNeil-Gauthier AL, Brais B, Rouleau G, Anoja N, Ducharme S Tags: Neurocase Source Type: research

Distinguishing logopenic from semantic & nonfluent variant primary progressive aphasia: Patterns of linguistic and behavioral correlations.
Distinguishing logopenic from semantic & nonfluent variant primary progressive aphasia: Patterns of linguistic and behavioral correlations. Neurocase. 2019 Jun 04;:1-8 Authors: Keator LM, Wright AE, Saxena S, Kim K, Demsky C, Sebastian R, Sheppard SM, Breining B, Hillis AE, Tippett DC Abstract While language characteristics of logopenic variant primary progressive aphasia (lvPPA) are well-defined, behavioral characteristics are less understood. We investigated correlations between language and behavioral scores across three variants of primary progressive aphasia (PPA) and found language performan...
Source: Neurocase - June 4, 2019 Category: Neurology Authors: Keator LM, Wright AE, Saxena S, Kim K, Demsky C, Sebastian R, Sheppard SM, Breining B, Hillis AE, Tippett DC Tags: Neurocase Source Type: research

Thought disorder in atypical schizophrenia-like psychosis after stroke: analysis of clinical cases.
We described two cases of female patients, who developed an atypical schizophrenia-like psychosis post-stroke, characterized by formal thought disorder, hallucinations, and delusion. Cerebrovascular disease affected the right temporal lobe, areas devoted to modulation of higher order cognitive processes. To date, the relation between stroke and psychosis remain unclear, maybe due to the involvement of a complex brain networks. However, the high impact of this disorder on physical, psychological and social wellbeing suggests the importance of improving specific treatments. PMID: 31138024 [PubMed - as supplied by publis...
Source: Neurocase - May 29, 2019 Category: Neurology Authors: Lo Buono V, Corallo F, Allone C, Bonanno L, Sessa E, Todaro A, Pidalà A, Bramanti A, Marino S Tags: Neurocase Source Type: research

Disproportionately enlarged subarachnoid-space hydrocephalus (DESH) in normal pressure hydrocephalus misinterpreted as atrophy: autopsy and radiological evidence.
We report a 67-year-old man with features of NPH but not diagnosed because radiographic findings were interpreted as cortical atrophy. Autopsy showed findings consistent with NPH and no neurodegenerative disease. The second patient with DESH underwent shunt surgery. Entrapped fluid diminished after the surgery, confirming this is not atrophy, but due to a CSF dynamic process. Patients with DESH have tight sulci adjacent to the entrapped fluid pockets, distinguishing it from cortical atrophy. 18F-Fluorodeoxyglucose PET can help differentiate DESH from cortical atrophy. PMID: 31130064 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - May 27, 2019 Category: Neurology Authors: McCarty AM, Jones DT, Dickson DW, Graff-Radford NR Tags: Neurocase Source Type: research

Functional imaging localization of complex organic hallucinations.
CONCLUSIONS: To our knowledge, this is the first reported case of combined functional and structural connectivity imaging demonstrating brain regions participating in a network involved in the generation of complex auditory and visual hallucinations. PMID: 31094654 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - May 16, 2019 Category: Neurology Authors: Bernal B, Guillen M, Ragheb J, Altman N, Ibrahim GM, LaPlante P, Padilla A, Duchowny M Tags: Neurocase Source Type: research

Chronic psychosis related to benzydamine hydrochloride abuse.
Ouml;rsel S Abstract Benzydamine hydrochloride is a non-steroidal anti-inflammatory drug with analgesic and antipyretic effects. In those who use medicines containing this agent at high doses (500-3000 mg), some adverse effects such as hallucinosis, stimulant effects in the central nervous system, paranoia, and convulsions can be seen. The drug is vulnerable to abuse because of the stimulant effects on the central nervous system. In this paper, we present a young male patient with symptoms of psychosis due to benzydamine hydrochloride abuse. He was admitted to the psychiatry outpatient clinic with visual hallucina...
Source: Neurocase - May 14, 2019 Category: Neurology Authors: Gürü M, Şafak Y, Cengiz GF, Kuru E, Örsel S Tags: Neurocase Source Type: research

A new cause of developmental and epileptic encephalopathy with continuous spike-and-wave during sleep: CDKL5 disorder.
Abstract The CDKL5 disorder is characterized by early onset epilepsy, stereotypical hand movement, absent speech and severe hypotonia. Herein, we report epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS) in apatient with CDKL5 disorder. She admitted with complaints of frequently recurring generalized tonic and myoclonic seizures. The diagnoses were confirmed by de novo CDKL5 mutation, c.197_198delCT (p.L67QfsX23). Interictal EEG revealed generalized spike and slow-wave activity, occurring intermittently in wakefulness but present for at least 85% of non-REM sleep, consi...
Source: Neurocase - May 3, 2019 Category: Neurology Authors: Arican P, Gencpinar P, Olgac Dundar N Tags: Neurocase Source Type: research

Frontal variant of Alzheimer's disease masquerading as behavioural-variant frontotemporal dementia: a case study comparison.
Abstract The current clinical diagnostic criteria for Alzheimer's disease (AD) recognize an atypical, non-amnestic presentation of AD, characterized by prominent executive dysfunction. Increasing evidence, however, indicates that the clinical phenotype of this so-called "frontal-variant" of AD (fv-AD) includes behavioral symptoms and deficits in social cognition, together with disproportionate frontal lobe atrophy. As these features resemble those characteristic of behavioral-variant frontotemporal dementia (bvFTD), differential diagnosis can be challenging. Here, we report a case of fv-AD who met clinic...
Source: Neurocase - May 2, 2019 Category: Neurology Authors: Wong S, Strudwick J, Devenney E, Hodges JR, Piguet O, Kumfor F Tags: Neurocase Source Type: research

Atypical clinical features associated with mixed pathology in a case of non-fluent variant primary progressive aphasia.
Abstract A 66-year-old woman presented with agrammatism and apraxia of speech, meeting criteria for non-fluent/agrammatic variant primary progressive aphasia (nfvPPA). However, three years later, she developed frontal/executive, short-term phonological memory, visuospatial, and visual memory deficits suggesting involvement of multiple brain networks. Multimodal neuroimaging showed damage of both fronto-striatal and posterior brain regions. She was found to have multiple pathological processes: corticobasal degeneration (CBD), Alzheimer's disease (AD), and TAR DNA-binding protein (TDP)-43 type A. We hypothesize tha...
Source: Neurocase - April 29, 2019 Category: Neurology Authors: De Leon J, Mandelli ML, Nolan A, Miller ZA, Mead C, Watson C, Welch AE, Henry ML, Bourakova V, La Joie R, Bajorek LP, Grinberg L, Rabinovici G, Miller BL, Gorno-Tempini ML Tags: Neurocase Source Type: research

Acute loss of psychic self-activation after cardiac arrest and delayed bilateral pallidal lesions on brain MRI.
We report an unusual case of loss of psychic self-activation appeared immediately after a cardiac arrest in a middle age patient. The first brain MRI, one month after the vascular event, did not show the classical lesions typically reported, such as lesion of the caudate nucleus or the globus pallidus. Two years later, although the cognitive performances of our patient were improved, a second brain MRI demonstrated bilateral pallidal lesions, suggesting a possible mechanism with delayed hypoxic lesions. PMID: 31020910 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - April 25, 2019 Category: Neurology Authors: Martinaud O, Le Goff F, Carlier J, Pouliquen D, Gérardin E, Savouré A Tags: Neurocase Source Type: research

Corticobasal syndrome with visual hallucinations and probable REM-sleep behavior disorder: an autopsied case report of a patient with CBD and LBD pathology.
This report highlights the importance of considering co-existing pathologies when a clinical presentation defies categorization, and demonstrates that salient features of dementia with Lewy bodies may result from pathology limited to the brainstem. PMID: 31006355 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - April 22, 2019 Category: Neurology Authors: Naasan G, Shany-Ur T, Sidhu M, Barton C, Ketelle R, Shdo SM, Kramer JH, Miller BL, Seeley WW Tags: Neurocase Source Type: research

The cognitive aspects of sexual intimacy in dementia patients: a neurophysiological review.
Abstract Changes in higher-order cognitive behaviors such as reward recognition, salience processing, novelty perception, decision-making, emotional contagion, motivation, and empathy may contribute to intimacy dysfunction. Network circuitry underlying these cognitive functions is often activated in sexually intimate behavior. We propose that sexual dysfunction in AD and bvFTD is more nuanced than is commonly believed, and propose how AD and bvFTD atrophy may correlate neuroanatomically to brain regions and networks that contribute to the higher-order cognitive aspects of intimacy. The cha...
Source: Neurocase - April 16, 2019 Category: Neurology Authors: Nordvig AS, Goldberg DJ, Huey ED, Miller BL Tags: Neurocase Source Type: research

High-functioning autism spectrum disorder with fluent speech and late-onset epilepsy: an unusual presentation of Inv-Dup (15) syndrome.
This report suggests that a diagnosis of Inv-Dup (15) can be suspected during more benign atypical condition with a better outcome than usually reported. PMID: 30991884 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - April 16, 2019 Category: Neurology Authors: Bernardo P, Del Gaudio L, Madia F, Riccio MP, Marino M, Santoro C, Caccavale C, Striano S, Bravaccio C, Coppola A Tags: Neurocase Source Type: research

Smartwatch aids time-based prospective memory in Korsakoff syndrome: a case study.
Abstract Prospective memory (PM) is the ability to remember to carry out an intention in the future. PM is particularly impaired in Korsakoff syndrome (KS). We investigated the benefit of a smartwatch and smartphone compared to no aid in supporting time accuracy and PM task performance in KS. Time accuracy was improved with a smartwatch compared to the other conditions. Furthermore, the smartwatch and phone conditions were more effective than no aid in assisting memory for task content. Together these results suggest that using an external memory aid is beneficial for successful PM in KS. PMID: 30966873 [PubM...
Source: Neurocase - April 10, 2019 Category: Neurology Authors: Lloyd B, Oudman E, Altgassen M, Postma A Tags: Neurocase Source Type: research

CSF1R mutation presenting as dementia with Lewy bodies.
We present a case of a patient with a pathogenic variant in the CSF1R gene with clinical and imaging features suggestive of Dementia with Lewy Bodies (DLB). This case expands the known clinical presentations associated with CSF1R mutations. PMID: 30968732 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - April 10, 2019 Category: Neurology Authors: Sharma R, Graff-Radford J, Rademakers R, Boeve BF, Petersen RC, Jones DT Tags: Neurocase Source Type: research

Association of late effects of single, severe traumatic brain injury with Alzheimer's disease using amyloid PET.
Abstract Traumatic brain injury (TBI) is suggested to be a risk factor for the onset of Alzheimer's disease (AD); however, the data remain controversial. This is the first report on cognitive decline in patients with TBI over 30 years post-injury. The medical significance/key learning points of this report are that (1) Functional Independence Measure (FIM) is useful in clinical settings, such as for higher brain dysfunction and dementia; (2) amyloid PET findings represent an essential biomarker for follow-up after TBI; and (3) cognitive decline can occur in patients with TBI more than 30 years post-injury. PM...
Source: Neurocase - April 5, 2019 Category: Neurology Authors: Hiraoka T Tags: Neurocase Source Type: research

Orthographic connectivity in Arabic reading: a case study of an individual with deep dyslexia and letter-by-letter reading.
Abstract Arabic orthography is complex, partly as a consequence of variations in orthographic connectivity. In this article, we present the case study of CHS, an Arabic individual with deep dyslexia associated with letter-by-letter reading. In the experimental study, we specifically explored the influence of orthographic connectivity on CHS's word recognition and reading abilities. Our results show that CHS's performance was better preserved for words and non-words made up of connected letters than made up of non-connected letters. CHS demonstrated impairment of visuoperceptual mechanisms,...
Source: Neurocase - April 2, 2019 Category: Neurology Authors: Boumaraf A, Macoir J Tags: Neurocase Source Type: research

Thalamocortical disconnection affects the somatic marker and social cognition: a case report.
Abstract Thalamo-cortical connectivity was characterised in a patient with bilateral infarct of the thalami, without evidence of cognitive deficits in everyday life. Patient underwent social and emotional tests, Iowa Gambling Task (IGT), with and without concomitant heart rate variability (HRV) recording and at 3T-MRI to assess thalamo-cortical connectivity. Patient showed impairment at the IGT, in somatic marker, in emotions and theory of mind. MRI documented a bilateral damage of the centromedian-parafascicular complex. Patient's thalamic lesions disconnected brain areas involved in decision-making and autonomic...
Source: Neurocase - March 31, 2019 Category: Neurology Authors: Serra L, Bruschini M, Ottaviani C, Di Domenico C, Fadda L, Caltagirone C, Cercignani M, Carlesimo GA, Bozzali M Tags: Neurocase Source Type: research

Clinical presentation of rapidly progressive familial Danish dementia.
We present a case of young-onset dementia in a 47-year-old patient with Danish heritage who was subsequently diagnosed FDD through genetic testing. Cognitive impairment was his initial symptom, followed by Parkinsonian symptoms, and mood disturbances. The patient experienced rapid decline over only 19 months. Increased awareness and understanding of familial forms of dementia (i.e., FDD) can contribute to an enhanced provision of care for patients with such conditions. PMID: 30843473 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - March 7, 2019 Category: Neurology Authors: Zhou Z, Ainger TJ, Han DY Tags: Neurocase Source Type: research

Goal management training ® home-based approach for mild cognitive impairment in Parkinson's disease: a multiple baseline case report.
Goal management training ® home-based approach for mild cognitive impairment in Parkinson's disease: a multiple baseline case report. Neurocase. 2019 Mar 01;:1-11 Authors: Giguère-Rancourt A, Plourde M, Doiron M, Langlois M, Dupré N, Simard M Abstract Approximately 30% of patients with Parkinson's disease experience mild cognitive impairment (PD-MCI), often affecting executive functions. Our objective was to assess tolerability, safety and preliminarily efficacy of Goal Management Training® (GMT) for PD-MCI. GMT was administered at home, for five weeks. Dysexecutive Questionnaire...
Source: Neurocase - March 1, 2019 Category: Neurology Authors: Giguère-Rancourt A, Plourde M, Doiron M, Langlois M, Dupré N, Simard M Tags: Neurocase Source Type: research

DNA methylation of the NR3C1 promoter region in brains of pediatric victims of physical abuse.
We examined DNA methylation of the 1F promoter region of NR3C1 in the postmortem brains of eight children including four ELS cases. As a result, DNA methylation was evident in ELS cases due to severe physical abuse. Epigenetic status may have potential application as a biomarker for clarifying the antemortem experiences of deceased. PMID: 30810493 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - February 27, 2019 Category: Neurology Authors: Takahashi Y, Kubo R, Sano R, Kuninaka H, Murayama M, Hayakawa A, Kominato Y Tags: Neurocase Source Type: research

Visual statistical learning deficits in memory-impaired individuals.
Abstract Visual statistical learning (VSL) refers to the learning of environmental regularities. Classically considered an implicit process, one patient with isolated hippocampal damage is severely impaired at VSL tasks, suggesting involvement of explicit memory. Here, we asked whether memory impairment (MI) alone, absent of clear hippocampal pathology, predicted deficits across different VSL tasks. A classic VSL task revealed no learning in MI participants (Exp. 1), while imposing attentional demands (Exp. 2: flicker detection, Exp. 3: gender/location categorization) during familiarization revealed modest residua...
Source: Neurocase - February 22, 2019 Category: Neurology Authors: Cerreta AGB, Vickery TJ, Berryhill ME Tags: Neurocase Source Type: research

TARDBP mutation associated with semantic variant primary progressive aphasia, case report and review of the literature.
We describe a case of svPPA associated with MND in the same family, due to a mutation of the transactive response DNA binding protein (TARDBP) gene, and review the literature. PMID: 30773994 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - February 16, 2019 Category: Neurology Authors: González-Sánchez M, Puertas-Martín V, Esteban-Pérez J, García-Redondo A, Borrego-Hernández D, Méndez-Guerrero A, Llamas-Velasco S, Herrero-San Martín A, Cordero-Vázquez P, Herrero-Manso MC, Pérez-Martínez DA, Villarejo-Galende A Tags: Neurocase Source Type: research

An unusual presentation of late-onset Alexander's disease with slow orthostatic tremor and a novel GFAP variant.
We report a case of AOAD, with onset in the eighth decade, presenting with slow variant orthostatic tremor, which has not been previously described. Genetic analysis revealed a GFAP variant (c.1158C>A) that has not been previously reported. Our case serves to expand the diagnostic spectrum of AOAD both clinically and genetically. PMID: 30755139 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - February 12, 2019 Category: Neurology Authors: Stitt DW, Gavrilova R, Watson R, Hassan A Tags: Neurocase Source Type: research

A case report of the neurocognitive and behavioral phenotype of mosaic trisomy 14.
This study provides the first case description of the cognitive-behavioral phenotype associated with mosaic trisomy 14 by contrasting the performance an adolescent female (T14) with a group of females with Down syndrome (DS; n=9). T14 performed below age expectations on most direct assessments and demonstrated weaknesses relative to the DS group on aspects of language, adaptive functioning, and executive functioning. T14 also demonstrated strengths in delayed visual recall and social skills relative to the DS group. PMID: 30714851 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - February 4, 2019 Category: Neurology Authors: Godfrey M, Udhnani M, Lee NR Tags: Neurocase Source Type: research

Altered perception might be a symptom of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.
Abstract Most patients with N-methyl-D-aspartate receptor (NMDAR) encephalitis initially present with psychiatric symptoms. Although a delayed diagnosis may lead to a poor outcome, psychiatric symptoms that could differentiate anti-NMDAR encephalitis from other psychoses have not been fully investigated. We evaluated two patients with anti-NMDAR encephalitis who were observed by psychiatrists from onset throughout the course of disease. Both patients exhibited disorientation, memory deficits, perceptual disturbances, hallucinations, and mood liability. Among those, altered perceptions were most prominent - in part...
Source: Neurocase - January 25, 2019 Category: Neurology Authors: Funayama M, Mizushima J, Takata T, Koreki A, Mimura M Tags: Neurocase Source Type: research

Effect of kinesthetic illusion induced by visual stimulation on ankle dorsiflexion dysfunction in a stroke patient: ABAB single-case design.
Abstract The purpose of this study was to investigate the effect of simultaneous intervention with the kinesthetic illusion induced by visual stimulation (KiNvis) and voluntary exercise on ankle dorsiflexion dysfunction in a patient with right-sided stroke hemiparesis. Within an ABAB single-case design, we conducted two phases each lasting five days. Phase A represented the baseline during which only voluntary ankle dorsiflexion (VAD) was performed. Phase B involved simultaneous performance of VAD and KiNvis. We measured the angle of ankle joint dorsiflexion (AJD), and the 10 m maximum walking speed (10MWS). AJD a...
Source: Neurocase - January 18, 2019 Category: Neurology Authors: Sakai K, Ikeda Y, Amimoto K Tags: Neurocase Source Type: research

Temporal-occipital glioblastoma presenting with Alice in Wonderland Syndrome in a patient with a long-time history of migraine without aura.
We present the case of a 54-year-old man, with a long-time history of migraine without aura, diagnosed with AIWS due to a glioblastoma located in the left temporal-occipital junction. To date, this is the first case of AIWS caused by glioblastoma. This case suggests that to exclude aura-mimic phenomena, a careful diagnostic workup should always be performed even in patients with a long-time history of migraine. PMID: 30599804 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - January 2, 2019 Category: Neurology Authors: Mastria G, Mancini V, Viganò A, De Sanctis R, Letteri F, Toscano M, Di Piero V Tags: Neurocase Source Type: research

Neuro-Beh çet's disease presenting as an isolated progressive cognitive and behavioral syndrome.
We describe a case of Neuro-Behçet's disease characterized by progressive behavioral and cognitive deterioration prevailing over other neurological symptoms, without evident systemic involvement. PMID: 30583716 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - December 25, 2018 Category: Neurology Authors: Saracino D, Allegorico L, Barbarulo AM, Pollo B, Giaccone G, D'Amico A, D'Incerti L, Bugiani O, Di Iorio G, Sampaolo S, Melone MAB Tags: Neurocase Source Type: research

Longitudinal neuropsychological outcomes in treatment-resistant depression following bed nucleus of the stria terminalis-area deep brain stimulation: a case review.
We present a case reviewing the longitudinal neuropsychological performance outcomes following bed nucleus of the stria terminalis-area (BNST) DBS in a patient with treatment-resistant depression (TRD). The cognitive safety of DBS is well documented for various targets, however cognitive outcomes of BNST-area DBS have not been extensively reported for patients with TRD. Neuropsychological assessment was conducted pre- and post-DBS. Twelve months following DBS, augmented general cognitive performance was observed with significant changes in specific domains. PMID: 30507338 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - December 3, 2018 Category: Neurology Authors: Cassimjee N, van Coller R, Slabbert P, Fletcher L, Vaidyanathan J Tags: Neurocase Source Type: research

Phelan-McDermid syndrome in adult patient with atypical bipolar psychosis repeatedly triggered by febrility.
We describe a long diagnostic odyssey of a 30-year-old woman who was diagnosed with Phelan-McDermid syndrom. Array comparative genomic hybridization analysis confirmed a 22q13.33 deletion, encompassing exon 9-23 of the SHANK3 gene and exon 1 of the ACR gene. We provide an uncommon feature of the disease, where psychotic alteration is repeatedly triggered by the same physical factor in our patient - mild fever episodes. PMID: 30376408 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - October 30, 2018 Category: Neurology Authors: Jungová P, Čumová A, Kramarová V, Lisyová J, Ďurina P, Chandoga J, Bӧhmer D Tags: Neurocase Source Type: research

Left hemispatial neglect with a splenial lesion.
Abstract With injury of the anterior two-thirds of the corpus callosum, each hemisphere's attentional bias to contralateral hemispace becomes manifest with each hand deviating ipsilaterally during line bisection tasks. Patients with infarctions in the right posterior cerebral artery distribution with occipital and splenial damage can also exhibit spatial neglect. The goal of this report is to learn the role of the splenium of the corpus callosum in mediating visuospatial attention. A right-handed woman with Marchiafava-Bignami disease and damage to the splenium of her corpus callosum without evidence of a mesial f...
Source: Neurocase - October 28, 2018 Category: Neurology Authors: Kesayan T, Gasoyan H, Heilman KM Tags: Neurocase Source Type: research

A neuropathologic diagnosis of Alzheimer's disease in an older adult with HIV-associated neurocognitive disorder.
Abstract We discuss the challenges associated with diagnosing neurodegenerative disorders in older adults living with HIV, illustrated through a case report where neurologic co-diagnosis of Alzheimer's disease (AD) and HIV-associated Neurocognitive Disorder (HAND) are considered. The patient was followed and evaluated for over 4 years and underwent post-mortem neuropathologic evaluation. Further work is needed to identify diagnostic tests that can adequately distinguish HAND from early stage neurodegenerative disorders among older adults living with HIV and cognitive changes. PMID: 30304986 [PubMed - as suppl...
Source: Neurocase - October 10, 2018 Category: Neurology Authors: Hellmuth J, Milanini B, Masliah E, Tartaglia MC, Dunlop MB, Moore DJ, Javandel S, DeVaughn S, Valcour V Tags: Neurocase Source Type: research