scholarly journals Corticobasal syndrome with Balint syndrome: a clue for Alzheimer disease pathology

2021 ◽  
Author(s):  
João Henrique Fregadolli Ferreira ◽  
Amanda Maieski ◽  
Caio Disserol ◽  
Helio Afonso Ghizoni Teive
Keyword(s):  
Alzheimer Disease ◽  
Fdg Pet ◽  
Brain Mri ◽  
Corticobasal Degeneration ◽  
Corticobasal Syndrome ◽  
Optic Ataxia ◽  
Left Hand ◽  
Oculomotor Apraxia ◽  
Dystonic Posturing ◽  
History Of

Context: Balint syndrome (BS), first described in 1909, has three core features: optic ataxia, oculomotor apraxia and simultanagnosia, and has been described after various conditions amongst vascular, infectious, demyelinating and degenerative diseases1 . It has already been reported concomitant with corticobasal syndrome (CBS)2 . Case report: 59 year-old male without history of previous diseases presented with behavior changes in the last two years. He had a previous diagnosis of “stroke” because frequent falls to the left side and difficulty in using his left hand for simple daily activities. After that, he gradually evolved with visual problems (bumped into objects inside his house), fear of walking or sitting, and required constant assistance for basic activities of daily living. On physical examination he presented with clear visuospatial dysfunction, characterized by simultanagnosia, oculomotor apraxia and optic ataxia. Bilateral asymmetric upper limb apraxia (worse on left side), dystonic posturing and stimulus-sensitive myoclonus in the left arm were also present. No signs of parkinsonism or language/speech disturbances were identified. Brain MRI showed severe asymmetric biparietal lobe atrophy (right more than left). DISCUSSION: The pathologic findings underlying CBS are variable, including Corticobasal Degeneration, Progressive Supranuclear Palsy, Frontotemporal Lobar Degeneration and Alzheimer Disease (AD). The association of BS and CBS favors the possibility of AD pathologic findings3 . Imaging methods like FDG-PET have recently been shown to be capable of distinguishing AD-related CBS from those associated with other pathologies4 . FDG-PET is not widely available in our country; than the presence of BS in CBS patients may individualize their treatment.

scholarly journals FDG-PET patterns associated with underlying pathology in corticobasal syndrome

Neurology ◽  
2019 ◽  
Vol 92 (10) ◽  
pp. e1121-e1135 ◽  
Author(s):  
Matteo Pardini ◽  
Edward D. Huey ◽  
Salvatore Spina ◽  
William C. Kreisl ◽  
Silvia Morbelli ◽  
...  
Keyword(s):  
Basal Ganglia ◽  
Fdg Pet ◽  
Primary Motor Cortex ◽  
Rating Scale ◽  
Corticobasal Degeneration ◽  
Anterior Cingulate ◽  
Corticobasal Syndrome ◽  
Control Group ◽  
Pathologic Diagnosis ◽  
Pet Scans

ObjectiveTo evaluate brain 18Fluorodeoxyglucose PET (FDG-PET) differences among patients with a clinical diagnosis of corticobasal syndrome (CBS) and distinct underling primary pathologies.MethodsWe studied 29 patients with a diagnosis of CBS who underwent FDG-PET scan and postmortem neuropathologic examination. Patients were divided into subgroups on the basis of primary pathologic diagnosis: CBS-corticobasal degeneration (CBS-CBD) (14 patients), CBS-Alzheimer disease (CBS-AD) (10 patients), and CBS–progressive supranuclear palsy (CBS-PSP) (5 patients). Thirteen age-matched healthy patients who underwent FDG-PET were the control group (HC). FDG-PET scans were compared between the subgroups and the HC using SPM-12, with a threshold of pFWE < 0.05.ResultsThere were no differences in Mattis Dementia Rating Scale or finger tapping scores between CBS groups. Compared to HC, the patients with CBS presented significant hypometabolism in frontoparietal regions, including the perirolandic area, basal ganglia, and thalamus of the clinically more affected hemisphere. Patients with CBS-CBD showed a similar pattern with a more marked, bilateral involvement of the basal ganglia. Patients with CBS-AD presented with posterior, asymmetric hypometabolism, including the lateral parietal and temporal lobes and the posterior cingulate. Finally, patients with CBS-PSP disclosed a more anterior hypometabolic pattern, including the medial frontal regions and the anterior cingulate. A conjunction analysis revealed that the primary motor cortex was the only common area of hypometabolism in all groups, irrespective of pathologic diagnosis.Discussion and conclusionsIn patients with CBS, different underling pathologies are associated with different patterns of hypometabolism. Our data suggest that FDG-PET scans could help in the etiologic diagnosis of CBS.

scholarly journals O08 Neurosarcoidosis mimicking CNS lymphoma

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Tarunya Arun
Keyword(s):  
Case Report ◽  
Ct Scan ◽  
Fdg Pet ◽  
Facial Pain ◽  
Brain Mri ◽  
Whole Body ◽  
Pet Ct ◽  
History Of ◽  
Fdg Pet Ct ◽  
Pet Ct Scan

Abstract Case report - Introduction Sarcoidosis is a multisystem disease which involves formation of inflammatory lesions known as granulomas. Central nervous system’s involvement is rare. Clinical neurologic complications occur in approximately 5% of patients. Diagnostic criteria for neurosarcoidosis in the absence of central nervous system (CNS) histology are not firmly established. A clinically compatible picture, exclusion of other neurological diseases, and histological confirmation of disease elsewhere are generally required.  We present a case report of neurosarcoidosis presenting as a lymphoma mimic.  Case report - Case description A 45-year-old right-handed white male with past medical history of obstructive sleep apnoea, presented to the acute neurology clinic with several weeks’ history of cognitive decline and severe L facial pain. He had lost 2 stone in weight and there was loss of appetite over 2 months. Neurology examination (including cranial nerves) was unremarkable except for a mini mental state score of 25/30, where he lost points on the attention and recall tasks. CT head revealed a mass in the L cavernous sinus. Brain MRI with contrast revealed an enhancing lesion in the left. suspicious of lymphoma. Additional work up included whole body FDG-PET/CT scan, lumbar puncture. Lumbar puncture showed normal CSF. Serum ACE was normal and a paraneoplastic panel. Whole body PET/CT scan showed FDG avid areas in the bilateral neck, axillary regions, chest and pelvis and inguinal regions, highly consistent with lymphoma. Bone marrow biopsy was negative for lymphoma. Further EBUS biopsy before start of prednisolone revealed multiple non caseating granulomas, diagnostic of sarcoidosis. The patient was treated with oral prednisolone, followed by anti-tumour necrosis factor-a infliximab infusion. A repeat brain MRI with contrast done at five months after initiation of steroids, methotrexate and infliximab showed complete resolution of the intracranial lesion. Neurological and neuropsychological evaluation three months after diagnosis demonstrated resolution of facial pain and cognitive decline.  Case report - Discussion There exists several mimics of neurosarcoidosis. Both clinically and radiographically, neurosarcoidosis can be difficult to diagnose. MRI and PET scan in neurosarcoidosis can often mimic malignancy. Early symptomatic treatment is advised for neurosarcoidosis, thus there is a clear need for more prompt diagnosis to allow commencement of the appropriate therapy.  There is no known cure for neurosarcoidosis. Immunosuppression is the primary means of controlling the disease, and corticosteroids are the cornerstone of therapy. Treatment options are limited; however, there is more evidence suggesting that steroids and immunomodulatory agents such as infliximab may improve clinical outcomes, which may be due to the anti-TNF-α effect on reducing oxidative stress.  Case report - Key learning points Our patient had a clinical presentation suspicious of lymphoma, however he did not have lymphoma and had a good response to corticosteroids and infliximab. Often, FDG PET/CT scan can be misleading and may appear to be neoplastic rather than inflammatory. ACE levels in both CSF and serum are not always positive. Biopsy in these cases is necessary to establish correct diagnosis. Prompt treatment can lead to significant reduction in mortality and morbidity

scholarly journals An autopsy-proven case of Corticobasal degeneration heralded by Pontine infarction

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Dallah Yoo ◽  
Sung-Hye Park ◽  
Sungwook Yu ◽  
Tae-Beom Ahn
Keyword(s):  
Neurodegenerative Disorders ◽  
Clinical Manifestations ◽  
Neuronal Loss ◽  
Corticobasal Degeneration ◽  
Executive Dysfunction ◽  
Lacunar Infarction ◽  
Cortical Atrophy ◽  
Dystonic Posturing ◽  
Proven Case ◽  
The Right

Abstract Background Neurodegenerative disorders are characterized by insidious progression with poorly-delineated long latent period. Antecedent clinical insult could rarely unmask latent neurodegenerative disorders. Here, we report an autopsy-proven case of corticobasal degeneration which was preceded by a lacunar infarction. Case presentation A 58-year-old man presented with acute ataxia associated with a lacunar infarction in the right paramedian pons. His ataxia persisted with additional progressive gait difficulty and left arm clumsiness. Six months later, a follow-up neurological examination showed asymmetrical bradykinesia, apraxia, dystonic posturing, postural instability, and mild ataxia of the left limbs. Cognitive examination revealed frontal executive dysfunction and visuospatial difficulties. Dopamine transporter imaging scan demonstrated bilateral reduced uptakes in mid-to-posterior putamen, more prominent on the right side. Levodopa-unresponsive parkinsonism, asymmetric limb dystonia, and ideomotor apraxia became more conspicuous, while limb ataxia gradually vanished. The patient became unable to walk without assistance after 1 year, and died 4 years after the symptom onset. Autopsy findings showed frontoparietal cortical atrophy, ballooned neurons, and phosphorylated tau-positive astrocytic plaques and neuropil threads with gliosis and neuronal loss, confirming the corticobasal degeneration. Conclusions The case illustrates that precedent clinical events such as stroke might tip a patient with subclinical CBS into overt clinical manifestations.

scholarly journals Teaching NeuroImages: Brain MRI and FDG-PET in malformations of cortical development and hippocampal hypoplasia

Neurology ◽  
2011 ◽  
Vol 77 (8) ◽  
pp. e47-e47 ◽  
Author(s):  
D. Renard ◽  
G. Castelnovo ◽  
D. Daubin ◽  
L. Collombier ◽  
C. Briere ◽  
...  
Keyword(s):  
Fdg Pet ◽  
Brain Mri ◽  
Cortical Development ◽  
Malformations Of Cortical Development

FDG-PET patterns associated with ideomotor apraxia and imitation apraxia in patients with corticobasal syndrome

2021 ◽  
Author(s):  
Sungyang Jo ◽  
Jungsu S. Oh ◽  
E-Nae Cheong ◽  
Hyung Ji Kim ◽  
Sunju Lee ◽  
...  
Keyword(s):  
Fdg Pet ◽  
Corticobasal Syndrome ◽  
Ideomotor Apraxia

Intracranial meningioma in a patient with osteogenesis imperfecta

Open Medicine ◽  
2008 ◽  
Vol 3 (4) ◽  
pp. 517-520
Author(s):  
Parmenion Tsitsopoulos ◽  
Ioannis Anagnostopoulos ◽  
Vasileios Tsitouras ◽  
Ioannis Venizelos ◽  
Philippos Tsitsopoulos
Keyword(s):  
Osteogenesis Imperfecta ◽  
Muscle Power ◽  
Brain Mri ◽  
Intracranial Meningioma ◽  
Type I ◽  
Mri Scans ◽  
Heritable Disorder ◽  
History Of ◽  
One Year ◽  
Gait Disturbances

AbstractOsteogenesis imperfecta (OI) is a heritable disorder characterized mainly by connective tissue manifestations. In dinstinct cases, several neurological features have also been described. A 46-year-old male with a known family history of OI type I presented with progressive gait disturbances and diminished muscle strength. Brain MRI scans revealed an infiltrative intracranial mass occupying both frontoparietal lobes. The patient underwent surgical intervention. The histological diagnosis was an atypical (Grade II) meningioma. The bony parts demonstrated a mixture of osseous defects due to OI and infiltration by the tumor. At one-year follow up the patient′s muscle power partially returned while repeat MRI scans were negative for tumor recurrence.

scholarly journals Miliary tuberculosis in a paediatric patient with psoriasis

2021 ◽  
Vol 14 (3) ◽  
pp. e237580
Author(s):  
Jacob Kilgore ◽  
Jonathon Pelletier ◽  
Bradford Becken ◽  
Stephen Kenny ◽  
Samrat Das ◽  
...  
Keyword(s):  
Brain Mri ◽  
Miliary Tuberculosis ◽  
Pulmonary Nodules ◽  
Chronically Ill ◽  
Community Acquired Pneumonia ◽  
Oral Antibiotic ◽  
Cavitary Lesion ◽  
Oral Antibiotic Therapy ◽  
History Of ◽  
Chest X Ray

We present a 16-year-old girl with a history of well-controlled psoriasis, on immunosuppression, who sought evaluation in the emergency department for 4 months of fever, cough and unintentional weight loss. The patient had seen multiple providers who had diagnosed her with community-acquired pneumonia, but she was unimproved after oral antibiotic therapy. On presentation, she was noted to be febrile, tachycardic and chronically ill-appearing. Her chest X-ray showed diffuse opacities and a right upper lobe cavitary lesion concerning for tuberculosis. A subsequent chest CT revealed miliary pulmonary nodules in addition to the cavitary lesion. The patient underwent subsequent brain MRI, which revealed multifocal ring-enhancing nodules consistent with parenchymal involvement. The patient was diagnosed with miliary tuberculosis and improved on quadruple therapy. Though rates of tuberculosis are increasing, rates remain low in children, though special consideration should be given to children who are immunosuppressed.

Abstract 36: Association of Parental Stroke with Cognitive and Brain MRI Measures in Offspring - The Framingham Heart Study

Stroke ◽  
2012 ◽  
Vol 43 (suppl_1) ◽  
Author(s):  
GALIT WEINSTEIN ◽  
Alexa Beiser ◽  
Rhoda Au ◽  
Charles DeCarli ◽  
Philip A Wolf ◽  
...  
Keyword(s):  
Executive Function ◽  
Brain Injury ◽  
Verbal Learning ◽  
Brain Mri ◽  
Abstract Reasoning ◽  
Parental History ◽  
Brain Volumes ◽  
Regional Brain ◽  
Increased Risk ◽  
History Of

Objectives- Parental stroke is related to an increased risk of stroke among the offspring. Vascular related brain changes, however, often occur before clinical stroke and the association of parental history of stroke and structural brain measures and cognition has not been fully explored. We hypothesized that prospectively verified parental stroke will be associated with increased vascular brain injury and poorer cognitive performance. Methods- A total of 1,297 Framingham offspring (mean age: 61 ± 9 years, 54% women) were studied. Of these, 9.9% had prospectively identified stroke in one or both parents before age 65. Volumetric brain MRI measures of total cerebral brain volume (TCBV), regional brain volumes, white matter hyperintensity volume (WMHV), and covert brain infarcts (CBI) and performance on tests of verbal memory, abstract reasoning, verbal learning and visuospatial memory (VRd) were compared for offspring with and without parental history of stroke. All measures were assessed cross-sectionally and longitudinally (mean duration of follow-up was 6.1±1.2 years). We used models adjusted only for age, sex, education and also additionally adjusted for vascular risk factors and for WMHV as an index of subclinical vascular brain injury. GEE models were used to adjust for sibling relationships among offspring. Results- Higher WMHV (β±SE=0.17±0.08;p=0.027) and lower VRd scores (β±SE=-0.80±0.34; p=0.017) at baseline were found in offspring with parental history of stroke. In addition, participants with parental stroke by age 65 years were more likely to be in the highest quintile of increase in WMHV (OR=1.87;p=0.04) as well as worsening executive function (Trails B-A) (OR:1.81;p=0.03). Parental stroke was not associated with total and regional brain volumes or with memory, abstract reasoning and verbal learning. Conclusions- In our community-based sample of middle-aged asymptomatic subjects, the occurrence of parental stroke by age 65 years is associated with higher baseline WMHV and with a more rapid increase in WMHV. Further, parental stroke is also associated with poorer performance on VRd and a decline in executive function. The effects on baseline WMH and VRd were substantial equivalent to 2.8 and 7 years of brain aging, respectively.

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