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Journal of Medical Case Reports Jan 2022The relatively isolated atrophy of the temporal lobes leads to a clinical radiological pattern, referred to as the temporal variant of frontotemporal dementia. While...
BACKGROUND
The relatively isolated atrophy of the temporal lobes leads to a clinical radiological pattern, referred to as the temporal variant of frontotemporal dementia. While semantic dementia and behavioral variant frontotemporal dementia are classically related to this syndrome, the logopenic variant of primary progressive aphasia has been less commonly reported. This case report aims to give a pictorial description of a case in which a patient with asymmetric temporal lobe atrophy presented with the logopenic variant of primary progressive aphasia and complex rituals of cleanliness.
CASE PRESENTATION
We report on the case of a 68-year-old, right-handed White woman with complex rituals and progressive speech impairment. The obsessive-compulsive rituals represented an exacerbation of lifelong preoccupations with cleanliness and orderliness that were praised by her relatives. Neuropsychological assessment revealed a striking impairment of language and memory, with relative sparing of tool-use praxis and visuospatial skills. Magnetic resonance imaging and fluorodeoxyglucose-positron emission tomography scans showed bilateral asymmetrical temporal lobe atrophy and hypometabolism. A year later, she was still able to entertain conversation for a short while, but her vocabulary and fluency had further declined. Praxis and visuospatial skills remained intact. She did not experience pathological elation, delusions, or hallucinations. The disease followed a relentless progression into a partial Klüver-Bucy syndrome, abulia, and terminal dementia. She died from acute myocardial infarction 8 years after the onset of aphasia. The symptoms and their temporal course supported a diagnosis of logopenic variant of primary progressive aphasia due to asymmetric temporal variant frontotemporal lobar degeneration.
CONCLUSIONS
This report gives a pictorial description of a temporal variant of frontotemporal dementia in a patient who presented with worsening of a lifelong obsessive-compulsive disorder and logopenic variant of primary progressive aphasia.
Topics: Aged; Aphasia, Primary Progressive; Atrophy; Female; Frontotemporal Dementia; Humans; Magnetic Resonance Imaging; Temporal Lobe
PubMed: 35045865
DOI: 10.1186/s13256-021-03228-z -
BMJ Case Reports Jul 2015The word 'abulia' means a lack of will, initiative or drive. The symptoms of abulia include lack of spontaneous action and speech, reduced emotional responsiveness and...
The word 'abulia' means a lack of will, initiative or drive. The symptoms of abulia include lack of spontaneous action and speech, reduced emotional responsiveness and social interaction, poor attention and easy distractibility. These symptoms are independent of reduced levels of consciousness or cognitive impairment. We describe a case of a socially active 72-year-old female patient who presented with symptoms of abulia which may have occurred due to damage of the frontosubcortical circuits following an episode of cardiac arrest. The patient's symptoms improved dramatically following treatment with bromocriptine.
Topics: Aged; Brain Injuries; Bromocriptine; Diagnosis, Differential; Dopamine Agonists; Female; Heart Arrest; Humans; Mental Disorders; Motivation; Treatment Outcome
PubMed: 26135487
DOI: 10.1136/bcr-2015-209357 -
Journal of Neurosciences in Rural... 2024[This corrects the article DOI: 10.4103/0976-3147.168438.].
[This corrects the article DOI: 10.4103/0976-3147.168438.].
PubMed: 38746534
DOI: 10.25259/JNRP_157_2024 -
Frontiers in Psychiatry 2023Fahr's disease (FD) is a rare disorder, characterized by basal ganglia calcification and presenting with movement disorders, speech impairment, cognitive deficits, and...
Fahr's disease (FD) is a rare disorder, characterized by basal ganglia calcification and presenting with movement disorders, speech impairment, cognitive deficits, and neuropsychiatric symptoms. Psychotic disorders related to FD are barely described in the literature, and knowledge is missing concerning pathophysiology, course, and management. Here, we report on the long-term follow-up of a patient who had three acute episodes of FD-psychosis characterized by bizarre delusions and behavioral disorganization, without hallucinations. Genetic and metabolic causes of FD were ruled out. In all three episodes, olanzapine monotherapy rapidly and completely resolved psychosis, without inducing metabolic syndrome and extrapyramidal symptoms. In addition to the acute decompensations, the patient presented a tame, introverted, industrious, and perfectionistic personality, which we could interpret as the "" described for many other basal ganglia disorders. Moreover, bizarre appearance, reduced affectivity, abulia, concrete speech, and stiff motricity in the context of a mild asymmetric extrapyramidal syndrome characterized the mental status. The cognitive profile was initially marked by executive difficulties and partial agnosia, with an IQ of 86. In the course of 10 years, the patient suffered from an ischemic stroke in the left superior temporal gyrus, which provoked a decline in memory and executive functions, without any impact on the psychiatric picture. Antiphospholipid antibody syndrome emerged as the underlying cause; thus, for the first time in the literature, an overlap of FD and antiphospholipid antibody syndrome is described here. This case report stresses once more the need for better integration of psychiatry and neurology and for the investigation of secondary causes of late-onset psychosis.
PubMed: 38016060
DOI: 10.3389/fpsyt.2023.1268982 -
Frontiers in Neurology 2023Frontotemporal lobe disorders (FTD) are amongst the most common brain neurodegenerative disorders. Their relatively covert, frequently subtle presentations and diverse...
BACKGROUND
Frontotemporal lobe disorders (FTD) are amongst the most common brain neurodegenerative disorders. Their relatively covert, frequently subtle presentations and diverse etiologies, pose major challenges in diagnosis and treatments. Recent studies have yielded insights that the etiology in the majority are due to environmental and sporadic causes, rather than genetic in origin.
AIMS
To retrospectively examine the cognitive and behavioral impairments in the veteran population to garner the range of differing syndrome presentations and etiological subcategories with a specific focus on frontotemporal lobe disorders.
METHODOLOGY
The design is a retrospective, observational registry, case series with the collection of epidemiological, clinical, cognitive, laboratory and radiological data on people with cognitive and behavioral disorders. Inclusion criteria for entry were veterans evaluated exclusively at Orlando VA Healthcare System, neurology section, receiving a diagnosis of FTD by standard criteria, during the observation period dated from July 2016 to March 2021. Frontotemporal disorders (FTD) were delineated into five clinical 5 subtypes. Demographic, cardiovascular risk factors, cognitive, behavioral neurological, neuroimaging data and presumed etiological categories, were collected for those with a diagnosis of frontotemporal disorder.
RESULTS
Of the 200 patients with FTD, further cognitive, behavioral neurological evaluation with standardized, metric testing was possible in 105 patients. Analysis of the etiological groups revealed significantly different younger age of the traumatic brain injury (TBI) and Gulf War Illness (GWI) veterans who also had higher Montreal Cognitive Assessment (MOCA) scores. The TBI group also had significantly more abnormalities of hypometabolism, noted on the PET brain scans. Behavioral neurological testing was notable for the findings that once a frontotemporal disorder had been diagnosed, the four different etiological groups consistently had abnormal FRSBE scores for the 3 principal frontal presentations of (i) abulia/apathy, (ii) disinhibition, and (iii) executive dysfunction as well as abnormal Frontal Behavioral Inventory (FBI) scores with no significant difference amongst the etiological groups. The most common sub-syndromes associated with frontotemporal syndromes were the Geschwind-Gastaut syndrome (GGS), Klüver-Bucy syndrome (KBS), involuntary emotional expression disorder (IEED), cerebellar cognitive affective syndrome (CCA), traumatic encephalopathy syndrome (TES) and prosopagnosia. Comparisons with the three principal frontal lobe syndrome clusters (abulia, disinhibition, executive dysfunction) revealed a significant association with abnormal disinhibition FRSBE T-scores with the GGS. The regression analysis supported the potential contribution of disinhibition behavior that related to this complex, relatively common behavioral syndrome in this series. The less common subsyndromes in particular, were notable, as they constituted the initial overriding, presenting symptoms and syndromes characterized into 16 separate conditions.
CONCLUSION
By deconstructing FTD into the multiple sub-syndromes and differing etiologies, this study may provide foundational insights, enabling a more targeted precision medicine approach for future studies, both in treating the sub-syndromes as well as the underlying etiological process.
PubMed: 38264092
DOI: 10.3389/fneur.2023.1305071 -
Journal of Korean Medical Science Jun 2001The purpose of this study is to determine the characteristic clinical features, radiologic findings, and precipitating and prognostic factors in the patients with breast...
The purpose of this study is to determine the characteristic clinical features, radiologic findings, and precipitating and prognostic factors in the patients with breast cancer and with 5-Fluorouracil (5-FU)-induced leukoencephalopathy. We reviewed the medical records of six breast cancer patients who developed leukoencephalopathy after chemotherapy which included 5-FU and also evaluated thorough neurological examinations including mini-mental status examination, cerebrospinal fluid studies, brain images and brain biopsies. Six patients exhibited slowly progressing neurologic symptoms characterized by the impairment of cognitive function, abulia, ataxic gait, and/or akinetic mutism. None of the patients had any specific causes or etiologic factors for leukoencephalopathy. Brain MRI in all patients showed diffuse periventricular white matter changes in the T2-weighted MR image. Brain biopsy in Patient 1 showed fragmented axonal fiber and minimally deprived myelination with many scattered macrophages. Five patients who treated with steroids at the onset of neurological symptoms showed clinical improvement, regardless of their age, sex, the pathology and stage of breast cancer, or the total dosage of chemotherapeutic agents. We conclude that leukoencephalopathy in these cases could be attributable to 5-FU neurotoxicity and suggest that the administration of steroids might be the treatment of choice.
Topics: Adenocarcinoma, Mucinous; Adult; Anti-Inflammatory Agents; Antineoplastic Agents; Brain; Breast Neoplasms; Carcinoma, Ductal, Breast; Cyclophosphamide; Epirubicin; Female; Fluorouracil; Glucocorticoids; Humans; Magnetic Resonance Imaging; Methylprednisolone; Middle Aged; Nervous System Diseases; Prednisolone; Radiography
PubMed: 11410695
DOI: 10.3346/jkms.2001.16.3.328 -
ENeurologicalSci Mar 2018From its initial report on two female patients in 1979 by J.O. Susac, Susac syndrome (SuS) or SICRET (small infarctions of cochlear, retinal and encephalic tissue) has...
INTRODUCTION
From its initial report on two female patients in 1979 by J.O. Susac, Susac syndrome (SuS) or SICRET (small infarctions of cochlear, retinal and encephalic tissue) has persisted as an elusive entity. To date the available evidence for its treatment is based on case reports and case series. The largest systematic review described only 304 reported cases since the 1970s. Here we presented the first reported case to our knowledge in Mexican population and the unusual presentation in a pregnant patient.
CASE PRESENTATION
A 34-year-old Hispanic woman was brought to the ER in our hospital for apathy and behavioral changes. Upon arrival at the ER, her husband described a one-month history of behavioral changes with apathy, progressive abulia, visuospatial disorientation, and gait deterioration. The initial lab test shows no significance except by a positive qualitative hCG. An MRI was obtained and showed hyperintense periventricular white matter lesions in T2 and FLAIR sequences also involving bilateral basal ganglia and with predominant affection of the corpus callosum, in addition to infratentorial cerebellar lesions. After treatment with intravenous immunoglobulins a marked and prompt clinical and radiological improvement was observed.
CONCLUSION
SuS is still an elusive disease. To date, no definitive score or clinical feature can predict the outcome of the disease. The presentation during pregnancy is also rare and therefore the optimal treatment and the prognosis is unknown. We hope that this article will serve as a foundation for future research.
PubMed: 29736421
DOI: 10.1016/j.ensci.2017.12.004 -
Indian Journal of Psychological Medicine Mar 2024
PubMed: 38725724
DOI: 10.1177/02537176231201559 -
BMC Neurology Jul 2014The Kii peninsula of Japan is one of the foci of amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS/PDC) in the world. The purpose of this study is to...
BACKGROUND
The Kii peninsula of Japan is one of the foci of amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS/PDC) in the world. The purpose of this study is to clarify the neuropsychological features of the patients with ALS/PDC of the Kii peninsula (Kii ALS/PDC).
METHODS
The medical interview was done on 13 patients with Kii ALS/PDC, 12 patients with Alzheimer's disease, 10 patients with progressive supranuclear palsy, 10 patients with frontotemporal lobar degeneration and 10 patients with dementia with Lewy bodies. These patients and their carer/spouse were asked to report any history of abulia-apathy, hallucination, personality change and other variety of symptoms. Patients also underwent brain magnetic resonance imaging (MRI), single photon emission computed tomography (SPECT), and neuropsychological tests comprising the Mini Mental State Examination, Raven's Colored Progressive Matrices, verbal fluency, and Paired-Associate Word Learning Test and some of them were assessed with the Frontal Assessment Battery (FAB).
RESULTS
All patients with Kii ALS/PDC had cognitive dysfunction including abulia-apathy, bradyphrenia, hallucination, decrease of extraversion, disorientation, and delayed reaction time. Brain MRI showed atrophy of the frontal and/or temporal lobes, and SPECT revealed a decrease in cerebral blood flow of the frontal and/or temporal lobes in all patients with Kii ALS/PDC. Disorientation, difficulty in word recall, delayed reaction time, and low FAB score were recognized in Kii ALS/PDC patients with cognitive dysfunction.
CONCLUSIONS
The core neuropsychological features of the patients with Kii ALS/PDC were characterized by marked abulia-apathy, bradyphrenia, and hallucination.
Topics: Aged; Amyotrophic Lateral Sclerosis; Dementia; Female; Humans; Japan; Male; Middle Aged; Neuropsychological Tests; Parkinsonian Disorders
PubMed: 25041813
DOI: 10.1186/1471-2377-14-151 -
Neurological Sciences : Official... Nov 2022A 43-year-old woman presented 1 day after whiplash injury with behavior change, hypersomnia, and abulia. MRI showed symmetrical globus pallidus infarction and bilateral...
A 43-year-old woman presented 1 day after whiplash injury with behavior change, hypersomnia, and abulia. MRI showed symmetrical globus pallidus infarction and bilateral watershed hypoperfusion. Magnetic resonance angiography (MRA) showed bilateral carotid artery dissection. To our knowledge, isolated symmetrical globus pallidus infarction related to bilateral carotid dissection has never been reported earlier.
Topics: Female; Humans; Adult; Globus Pallidus; Magnetic Resonance Imaging; Carotid Artery Diseases; Aortic Dissection; Infarction; Carotid Arteries
PubMed: 35915270
DOI: 10.1007/s10072-022-06278-3