2-carbomethoxy-8-(3-fluoropropyl)-3-(4-iodophenyl)tropane and Basal-Ganglia-Diseases

Fahr's syndrome due to hypoparathyroidism refers to bilateral basal ganglia (BG) calcifications and manifests with movement disorders, seizures, cognitive and behavioral symptoms.. We report a case of a 74-year-old woman, who presented with parkinsonism due to post-surgical hypoparathyroidism and normal DaT scan, despite extensive calcifications of the BG, periventricular white matter, and cerebellum.. A comprehensive literature review of all reported cases of Fahr's syndrome due to hypoparathyroidism was conducted in the electronic databases PubMed and Web of science. Moreover, demographic and clinical characteristics of the patients overall were calculated and associated with radiological findings.. We reviewed a total of 223 cases with Fahr's syndrome due to hypoparathyroidism (124 female, 99 male). Mean age on presentation was 44.6 ± 17.7 years. Thirty nine percent of patients had idiopathic hypoparathyroidism, 35.4 % acquired and 25.6 % pseudohypoparathyroidism. Almost half of the patients had tetany, seizures or a movement disorder and approximately 40 % neuropsychiatric symptoms. The patients with a movement disorder had a 2.23 likelihood of having neuropsychiatric symptoms as well (OR 2.23, 95 % CI 1.29-3.87). Moreover, there was a statistically significant association between the phenotype severity (i.e. the presence of more than one symptom) and the extent of brain calcifications (χ. Fahr's syndrome is a rare disorder, which nonetheless manifests with several neurological symptoms. A head CT should be considered for patients with hypoparathyroidism and neurological symptoms. More studies using DaT scan are needed to elucidate the effects of calcifications on the dopaminergic function of the BG.

Topics: Aged; Basal Ganglia Diseases; Calcinosis; Female; Humans; Hypoparathyroidism; Magnetic Resonance Imaging; Neurodegenerative Diseases; Nortropanes; Parkinsonian Disorders; Positron-Emission Tomography; Postoperative Complications; Severity of Illness Index; Thyroidectomy; Tomography, X-Ray Computed

The occurrence of parkinsonism in Alzheimer's disease (AD) is quite common, however the molecular and neurochemical changes underlying such extrapyramidal features in AD have been not fully understood. Post-mortem as well as in vivo imaging study have produced conflicting results as regards the existence of dopaminergic changes in AD. Aim of the present study was to investigate in vivo the nigro-striatal dopaminergic function in a group of AD patients with parkinsonism. Thirteen patients with AD and extrapyramidal features not related to past neuroleptic use (AD-P) underwent SPECT with 123I-FP-CIT, a ligand of dopamine transporter, and the data were compared with those obtained in 15 patients with Diffuse Lewy Body Dementia (DLBD), 20 patients with Parkinson's disease (PD), and 8 healthy elderly controls. The analysis of the data was performed by regions-of-interest approach and calculations of the striatal-to-non specific (occipital lobes) radioactivity ratios were made. The 123I-FP-CIT striatal uptake in patients with AD-P was similar to that obtained in the control population. Both the DLBD and PD groups showed significantly lower 123I-FP-CIT uptake in all striatal areas with respect to AD-P and control groups (p < 0.005). The lack of dopamine transporter changes in our series of AD-P patients can indicate that dopaminergic presynaptic function is preserved in this population and that different dopaminergic changes such as postsynaptic ones, or different neurotransmitter alterations might underlie the extrapyramidal features in AD.

Topics: Aged; Alzheimer Disease; Basal Ganglia Diseases; Diagnosis, Differential; Dopamine Plasma Membrane Transport Proteins; Female; Humans; Lewy Body Disease; Male; Membrane Glycoproteins; Membrane Transport Proteins; Neostriatum; Nerve Tissue Proteins; Parkinson Disease; Radiopharmaceuticals; Receptors, Presynaptic; Substantia Nigra; Tomography, Emission-Computed, Single-Photon; Tropanes

Topics: Aged; Basal Ganglia Diseases; Cerebral Cortex; Dopamine Plasma Membrane Transport Proteins; Female; Humans; tau Proteins; Tomography, Emission-Computed, Single-Photon; Tropanes

Topics: Basal Ganglia Diseases; Brain; Calcinosis; Cognition Disorders; Diagnosis, Differential; Female; Humans; Middle Aged; Movement Disorders; Nortropanes; Radiopharmaceuticals; Tomography, Emission-Computed, Single-Photon

Pure akinesia with gait freezing (PAGF) has characteristic features, including freezing of gait and prominent speech disturbance without rigidity or tremor. The purpose of this study was to investigate changes in brain glucose metabolism and presynaptic dopaminergic function in PAGF. By using [(18)F] fluorodeoxyglucose (FDG) PET, 11 patients with PAGF were compared with 14 patients with probable progressive supranuclear palsy (PSP), 13 patients with Parkinson's disease (PD), and 11 normal controls. [(18)F] N-(3-fluoropropyl)-2beta-carbon ethoxy-3beta-(4-iodophenyl) nortropane (FP-CIT) PET was performed in 11 patients with PAGF and with 10 normal controls. The PAGF patients showed decreased glucose metabolism in the midbrain when compared with normal controls. PSP patients showed a similar topographic distribution of glucose hypometabolism with additional areas, including the frontal cortex, when compared with normal controls. The FP-CIT PET findings in patients with PAGF revealed severely decreased uptake bilaterally in the basal ganglia. These findings suggest that both PAGF and PSP may be part of the same pathophysiologic spectrum of disease. However, the reason why PAGF manifests clinically in a different manner needs to be further elucidated.

Topics: Aged; Aged, 80 and over; Basal Ganglia Diseases; Brain; Carbon Radioisotopes; Female; Fluorine Radioisotopes; Fluorodeoxyglucose F18; Gait Disorders, Neurologic; Glucose; Humans; Hypokinesia; Iodine Radioisotopes; Magnetic Resonance Imaging; Male; Mesencephalon; Middle Aged; Parkinson Disease; Positron-Emission Tomography; Radiopharmaceuticals; Supranuclear Palsy, Progressive; Tropanes

The quality of visually and semi-quantitatively assessed DaTSCAN images is crucial for differential diagnostics of extrapyramidal diseases. Neuroimaging with the use of presynaptic tracers of the dopaminergic system provides evidence of nigrostriatal degeneration and may support the clinical diagnosis of Parkinsonism. During the last two years (2007-2008) we tried to elaborate the optimal methodology of SPECT/CT examination with the use of DaTSCAN ((123)I-Ioflupane), and we sought to evaluate the effect of the reconstruction and attenuation correction method on semi-quantitative measures of relative uptake in the striatum. In a present study, we retrospectively studied DaTSCAN scans of 44 consecutive patients with clinical indications of Parkinson's disease or uncertain Parkinsonian syndromes. The quality of DaTSCAN images reconstructed with the use of ordered-subset expectation maximization reconstruction technique (OSEM) with attenuation correction based on CT maps was found to be superior to that provided by the commonly applied filtered backprojection method (FBP) with Chang attenuation correction. OSEM reconstructed transverse slices were more legible for clinical interpretation because of increased contrast and improved delineation between striatum structures. Semi-quantitative assessments of relative striatum uptake for OSEM reconstructed slices secured better intra-operator reproducibility than that obtained by FBP method.

Topics: Adult; Aged; Aged, 80 and over; Artifacts; Basal Ganglia Diseases; Essential Tremor; Female; Humans; Image Enhancement; Male; Middle Aged; Nortropanes; Parkinson Disease; Radiopharmaceuticals; Reproducibility of Results; Sensitivity and Specificity; Tomography, Emission-Computed, Single-Photon; Tomography, X-Ray Computed

Ataxia with oculomotor apraxia type 1 (AOA1) is a rare autosomal recessive neurodegenerative disease, recently associated with mutations in the aprataxin gene. Main features are early onset cerebellar ataxia, oculomotor apraxia and peripheral neuropathy. The presence of choreoathetosis or dystonia in some patients suggests basal ganglia involvement, but these structures appear preserved in a single case in which neuropathological examination was performed. To evaluate in vivo the nigrostriatal function we studied dopamine transporter (DAT) density with [(123)I] 2beta-carbometoxy-3beta-(4-iodophenyl)-N-(3-fluoropropyl) nortropane (FPCIT)-SPECT in four AOA1 patients and eight healthy volunteers. All patients showed ataxia and neuropathy; only one had chorea and none had dystonia. Comparing with controls, AOA1 patients showed a slight reduction of the average striatal DAT density, which was bilateral and uniform in caudate and putamen. Nigrostriatal impairment occurred even in the absence of extrapyramidal features. Our data suggest subclinical involvement of basal ganglia in AOA1.

Topics: Adult; Apraxias; Basal Ganglia Diseases; Corpus Striatum; Dopamine; Dopamine Plasma Membrane Transport Proteins; Female; Humans; Iodine Radioisotopes; Male; Middle Aged; Neural Pathways; Neurodegenerative Diseases; Ocular Motility Disorders; Peripheral Nervous System Diseases; Substantia Nigra; Tomography, Emission-Computed, Single-Photon; Tropanes

Extrapyramidal symptoms are observed in frontotemporal dementia (FTD). (123)I-FP-CIT (DaT scan) single photon emission computed tomography (SPECT) can detect loss of presynaptic dopamine transporters in the striatum. We aimed to evaluate the dopaminergic status of the striatum in patients with FTD using DaT scan. Seven patients (age range 65-76 years), who fulfilled the Neary criteria and in whom the diagnosis of FTD was confirmed by hexamethylpropyleneamine oxime SPECT, were included in the study. The severity of the extrapyramidal symptoms was evaluated by the motor part of the Unified Parkinson's Disease Rating Scale (UPDRS). SPECT using (123)I-FP-CIT was done. A (region - occipital)/occipital ratio was calculated for the striatum, putamen and caudate nucleus. The results were compared with those of the 7 age-matched normal controls. The uptake of the radiotracer in the right and left striatum was reduced to 62% (p = 0.000) and 68% (p = 0.000), respectively, compared to controls. The motor UPDRS score of the patients with FTD showed a negative correlation to the uptake of the radiotracer. The presynaptic dopamine transporter in FTD is impaired, related to the severity of the extrapyramidal symptoms. Since an effective treatment for FTD is still to be established, there is a need for evaluating the efficacy of dopaminergic drugs.

Topics: Aged; Basal Ganglia Diseases; Brain; Corpus Striatum; Dementia; Dopamine; Dopamine Plasma Membrane Transport Proteins; Female; Humans; Hypokinesia; Male; Muscle Rigidity; Tomography, Emission-Computed, Single-Photon; Tropanes

Elderly patients with recurrent falls are frequently diagnosed with an extrapyramidal syndrome. This study aims to characterise a distinct group of patients with recurrent falls and postural instability as a hallmark of the clinical examination. The study took place in the Movement Disorders Unit, Rabin Medical Center, Petah Tiqva, Israel among 26 patients with recurrent falls who had no clinical evidence of a neurodegenerative disease. Medical records, neurological examination and brain imaging studies were assessed. Falls in these patients were sudden, unprovoked, with no vertigo or loss of consciousness. All had postural instability with minimal or no abnormality on the neurological examination. Brain imaging showed diffuse ischaemic changes in 65%. [(123)I]-FPCIT SPECT with the dopamine transporter ligand, performed in five patients, was normal in all. Recurrent falls might be caused by a neurological syndrome that primarily affects balance control. The importance of identifying this disorder is its distinction from other parkinsonian syndromes causing falls.

Topics: Accidental Falls; Aged; Aged, 80 and over; Basal Ganglia Diseases; Brain Infarction; Female; Humans; Iodine Radioisotopes; Male; Movement Disorders; Neurodegenerative Diseases; Neurologic Examination; Postural Balance; Posture; Tomography, Emission-Computed, Single-Photon; Tropanes