The clinical utility of 99mTc-HMPAO SPECT in Fahr’s disease

Öneş, Tunç; Dede, Fuat; Günal, Dilek İnce; Ekinci, Gazanfer; Memis, Hatice; Erdıl, Tanju Yusuf; İnanır, Sabahat; Turoğlu, Halil Turgut

doi:10.1007/s12149-007-0121-0

Cited by 7 publications

(6 citation statements)

References 20 publications

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“…1,2,[13][14][15] Several studies of DNTC and Fahr's disease have suggested that neuronal degeneration and calcification occur independently, because regional CBF or metabolism was normal in portions with severe calcification including the basal ganglia and cerebellum. 6,7,11 In addition, similar findings have been reported in autopsy cases, with no apparent degeneration or neuron loss observed in the basal ganglia and cerebellum, despite distinct calcification in those areas. 14,16 Therefore, the CBF asymmetry noted in our case appears to be independent of neuronal degeneration associated with DNTC.…”

Section: Discussionsupporting

confidence: 84%

“…Neuropathological changes of DNTC include neurofibrillary tangles, calcareous deposits, glial fibrillary tangles, and vascular changes . Several studies of DNTC and Fahr's disease have suggested that neuronal degeneration and calcification occur independently, because regional CBF or metabolism was normal in portions with severe calcification including the basal ganglia and cerebellum . In addition, similar findings have been reported in autopsy cases, with no apparent degeneration or neuron loss observed in the basal ganglia and cerebellum, despite distinct calcification in those areas .…”

Section: Discussionsupporting

confidence: 61%

“…Segmental CBF was calculated as the area‐weighted mean value for each of the 24 segments in both hemispheres based on regional CBF in each ROI. To evaluate the laterality of CBF, an asymmetry index was calculated for each ROI, as follows:

Asymmetry index = [(Right - Left) / {(Right + Left) / 2} 100]

…”

Section: Methodsmentioning

confidence: 99%

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Evaluation of regional cerebral blood flow in patient with atypical senile dementia with asymmetrical calcification

2015

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We report an 83-year-old woman with atypical senile dementia with Fahr-type calcification. Brain computed tomography demonstrated asymmetrical calcification predominant in the basal ganglia on the right side and pronounced diffuse cortical atrophy in the frontotemporal areas. The patient was clinically diagnosed with diffuse neurofibrillary tangles with calcification. Brain single photon emission computed tomography findings revealed that cerebral blood flow was reduced on the right side, as compared with the left side, in widespread areas. Hemispheric asymmetry in both calcification and cerebral blood flow suggests a relationship between calcification and vascular changes.

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Section: Discussionsupporting

confidence: 84%

Section: Discussionsupporting

confidence: 61%

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Evaluation of regional cerebral blood flow in patient with atypical senile dementia with asymmetrical calcification

2015

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“…The usefulness of 99 m Tc-HMPAO brain perfusion SPECT in deciding clinical approach to Fahr's syndrome has been suggested. 12 The treatment of Fahr's syndrome is directed to the identifiable cause especially hypoparathyroidism. In other cases, symptomatic or conservative therapy with clinical follow-up is the rule.…”

Section: Discussionmentioning

confidence: 99%

Fahr's syndrome: a rare clinico-radiologic entity

Rastogi¹,

Singh²,

Rastogi³

et al. 2011

Medical Journal Armed Forces India

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In the white matter, the calcification was noted at the corticomedullary junction with no evidence of any adjacent atrophy. In addition, midline irregular calcification was noted in the pons (Figure 1). Based on the clinico-radiological and biochemical findings, the provisional diagnosis of Fahr's syndrome due to idiopathic striopallidodentate calcinosis or Fahr's disease was strongly suggested. As there was no known cause, the patient was advised anti-convulsants and regular follow-up. The latter was unremarkable for nine months.

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“…As regards secondary forms, the diagnosis is made, independently of the presence of symptoms, when cerebral calcifications are present at brain imaging, and a specific factor able to cause them is demonstrated. Among the neuroradiologic exams, brain CT is more sensitive than traditional MRI in the detection of calcium deposits [52], but the recently introduced SWI (Susceptibility-weighted imaging) MRI seems to be more sensitive for early changes [53, 54]; another useful tool is the 99mTehexamethyl-propylenamine oxime (99mTc-HMPAO) single proton emission computed tomography (SPECT)[55], revealing markedly decreased perfusion involving the basal ganglia bilaterally and cerebral cortices [56]. …”

Section: Revision Of Literaturementioning

confidence: 99%

Bilateral strio-pallido-dentate calcinosis (Fahr’s disease): report of seven cases and revision of literature

et al. 2016

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BackgroundFahr’s disease is rare a neurodegenerative idiopathic condition characterized by symmetric and bilateral calcifications of basal ganglia, usually associated with progressive neuropsychiatric dysfunctions and movement disorders. The term “Fahr’s syndrome” is used in presence of calcifications secondary to a specific cause, but the variability of etiology, pathogenesis, and clinical picture underlying this condition have raised the question of the real existence of a syndrome. Several classifications based on the etiology, the location of brain calcifications and the clinical presentation have been proposed. Here we describe seven clinical cases of basal ganglia calcifications, in order to search for pathognomonic features and correlations between clinical picture and imaging findings.Cases presentationThe patients came to our attention for different reasons (most of them for memory/behavior disturbances); all underwent neuro-psychologic evaluation and neuro-imaging. All patients showed variable degrees of deterioration in cognitive function; anxiety and depression were frequent too, and resistant to treatment in all cases. Less frequent, but severe if present, were psychotic symptoms, with different grade of structure and emotional involvement, and always resistant to treatment. We observed only few cases of extrapyramidal disorders related to the disease itself; anyway, mild extrapyramidal syndrome occurred quite frequently after treatment with antipsychotics.ConclusionBased on these findings we discourage the use of the term “Fahr’s syndrome”, and suggest to refer to Idiopathic or Secondary basal ganglia calcification. Unlike early onset forms (idiopathic or inherited), the clinical presentation of late onset form and Secondary basal ganglia calcification seems to be really heterogeneous. Case–control studies are necessary to determine the actual significance of basal ganglia calcification in the adult population and in the elderly, in cognitive, physical and emotional terms.

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The clinical utility of 99mTc-HMPAO SPECT in Fahr’s disease

Cited by 7 publications

References 20 publications

Evaluation of regional cerebral blood flow in patient with atypical senile dementia with asymmetrical calcification

Evaluation of regional cerebral blood flow in patient with atypical senile dementia with asymmetrical calcification

Fahr's syndrome: a rare clinico-radiologic entity

Bilateral strio-pallido-dentate calcinosis (Fahr’s disease): report of seven cases and revision of literature

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