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Cureus Apr 2022Galactose-α-1,3-galactose, an oligosaccharide epitope better acknowledged as α-Gal, is present in non-primate mammal meat, tick bites, microorganisms, and vaccines as...
Novel Case Presentation of Abulia After Lone Star Tick Bite As Evidenced by Raised Titers of Alpha-Gal Specific IgM Immunoglobulin and a Possibility of Alpha-Gal Driven Hypothalamic Dysfunction As the Pathomechanism.
Galactose-α-1,3-galactose, an oligosaccharide epitope better acknowledged as α-Gal, is present in non-primate mammal meat, tick bites, microorganisms, and vaccines as a glycoprotein or glycolipid moiety. This can manifest hyperimmune reactions as it enters the human body, known as α-Gal syndrome (AGS). AGS and Guillain-Barré syndrome share cognate immunogenic pathomechanism via conquering immune tolerance further speculating galactose navigated neurological sequel. Unusual symptomatic presentation of abulia in our case, with incidental finding of high titers of α-Gal specific IgE immunoglobulin further supported by temporal resolution of symptoms on abstinence of meat products, raises a high degree of suspicion of neuro-psychiatric manifestation in sensitized α-Gal patients. The pathomechanism is blurry, and an absence of an objective diagnostic tool makes the neurological diagnosis challenging. α-Gal driven immune-related hypothalamic dysfunction could be a possibility that needs further exploration and is a topic of research.
PubMed: 35651471
DOI: 10.7759/cureus.24551 -
Psychiatria Polska Apr 2022Research on predictors of adolescent schizophrenia, especially that based on long-term follow-up, is rare in the literature. In our analysis, we examine the relationship...
A 45-year follow-up study of juvenile schizophrenia. Part III: Clinical picture and effect of the first hospitalization in the context of disease course and long-term social functioning of patients.
OBJECTIVES
Research on predictors of adolescent schizophrenia, especially that based on long-term follow-up, is rare in the literature. In our analysis, we examine the relationship of the clinical picture and effect of the first hospitalization with clinical and social indicators of the disease.
METHODS
A total of 69 patients at an average age of 16 years (time point 0), hospitalized due to schizophrenia (retrospectively re-diagnosed according to ICD-10 criteria) and re-examined 5 years later (time point 1 - personal examination of 41 individuals), were re-evaluated for clinical and social parameters 45 years after their initial hospitalization (time point 2 - personal examination of 21 individuals).
RESULTS
The clinical picture of the first episode of schizophrenia in terms of autism, apathy and abulia symptoms, splitting symptoms, formal thought disorders, catatonic symptoms, hebephrenic symptoms, delusions, hallucinations, and total severity of the schizophrenic psychopathology as a whole, as well as the effect of the first hospitalization (measured by the level of improvement, insight, and relational abilities), revealed numerous and various correlations with both the symptomatic picture and clinical course of schizophrenia and distant social functioning of the subjects. Variables of the greatest prognostic value were: initial autism and the level of clinical improvement, insight, and the ability to establish relationships, as measured at the end of the first hospitalization.
CONCLUSIONS
Negative symptoms during the first episode of schizophrenia and the quality of improvement in the areas of symptoms, insight, and the ability to establish relationships, achieved during the first hospital stay, turned out to be significant prognostic factors in juvenile schizophrenia.
Topics: Adolescent; Disease Progression; Follow-Up Studies; Hospitalization; Humans; Retrospective Studies; Schizophrenia; Schizophrenic Psychology; Social Interaction
PubMed: 35988071
DOI: 10.12740/PP/125937 -
BMJ Case Reports May 2021We present a 24-year-old man with a 2-year history of progressive right-sided monocular vision loss with no other symptoms. An MRI showed a meningioma compressing the...
We present a 24-year-old man with a 2-year history of progressive right-sided monocular vision loss with no other symptoms. An MRI showed a meningioma compressing the right optic nerve and the cavernous sinus. The tumour was partially resected. Eight days after discharge the patient was admitted with fever, a severe stabbing headache, insomnia, nausea and vomiting. A FilmArray panel and a cerebral biopsy were performed which were positive for herpes simplex virus 1 (HSV-1). An MRI of the brain showed asymmetric bilateral lesions in the frontobasal region with predominance of the right side. Acyclovir was started and continued until completing 21 days. A month after discharge, he started experiencing insomnia, trichotillomania, limb tremor, persistence of abulia, apathy and emotional lability. An HSV-1 encephalitis relapse was suspected, acyclovir and foscarnet were started. Due to the poor response to antiviral therapy CSF was tested, which was positive for anti-NMDA receptor encephalitis. A treatment course of intravenous immunoglobulin was started with a favourable outcome.
Topics: Acyclovir; Adult; Antiviral Agents; Encephalitis, Herpes Simplex; Foscarnet; Humans; Male; Neoplasm Recurrence, Local; Young Adult
PubMed: 34039543
DOI: 10.1136/bcr-2020-241136 -
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 -
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 -
Neurocase Apr 2021A 65-year-old right-handed man gradually became socially indifferent and less active. Four years later, he was indicted for molesting children on multiple occasions....
A 65-year-old right-handed man gradually became socially indifferent and less active. Four years later, he was indicted for molesting children on multiple occasions. Psychomotor slowness and executive impairment contrasted with sparing of language, semantic memory, visuospatial perception, construction praxis, and right-left orientation. Neuroimaging showed asymmetric atrophy of dorsomedial frontal and anterior temporal lobes, and hypoperfusion of medial prefrontal cortex consistent with a diagnosis of behavioral variant of frontotemporal dementia. Further information revealed that the patient exhibited pedophilic behavior several years prior to symptom onset. We conclude that preexisting developmental pedophilia was "unmasked" by the underlying progressive frontotemporal degeneration.
Topics: Aged; Child; Frontotemporal Dementia; Humans; Male; Neuroimaging; Pedophilia; Pick Disease of the Brain; Temporal Lobe
PubMed: 33856971
DOI: 10.1080/13554794.2021.1886310 -
Indian Journal of Psychological Medicine Mar 2024
PubMed: 38725724
DOI: 10.1177/02537176231201559 -
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 -
Cureus Jul 2021We report a case of a 48-year-old female who presented with abulia and headaches. Head CT (HCT) demonstrated a left caudate intracerebral hemorrhage (ICH) with extension...
We report a case of a 48-year-old female who presented with abulia and headaches. Head CT (HCT) demonstrated a left caudate intracerebral hemorrhage (ICH) with extension into the left lateral ventricle. Diagnostic cerebral angiogram showed a left distal medial lenticulostriate artery (MLSA) aneurysm with remote left parietal Spetzler-Martin grade 3 arteriovenous malformation (AVM). The patient underwent an endoscope-assisted transcortical-transventricular approach to the distal MLSA aneurysm with complete excision and evacuation of the intraventricular hemorrhage (IVH) postoperatively; the patient had no further neurologic deficits and recovered well from her initial hemorrhage. Ruptured MLSA aneurysms are a very rare cause of spontaneous ICH. Urgent treatment of these aneurysms is needed to prevent further rebleeding. We present a novel operative technique utilizing an endoscope-assisted transcortical-transventricular approach to a ruptured distal MLSA aneurysm with successful excision of the aneurysm.
PubMed: 34367793
DOI: 10.7759/cureus.16186