Substantia Nigra Swelling and Dentate Nucleus T2 Hyperintensity May Be Early Magnetic Resonance Imaging Signs of β‐Propeller Protein‐Associated Neurodegeneration
Camilla RussoAnna ArdissoneElena FreriSerena GasperiniMarco MoscatelliGiovanna ZorziCeleste PanteghiniBarbara CastellottiBarbara GaravagliaNardo NardocciLuisa Chiapparini
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ABSTRACT Background and Methods Mutations in WDR45 cause β‐propeller protein‐associated neurodegeneration (BPAN), a type of neurodegeneration with brain iron accumulation (NBIA). We reviewed clinical and MRI findings in 4 patients with de novo WDR45 mutations. Results Psychomotor delay and movement disorders were present in all cases; early‐onset epileptic encephalopathy was present in 3. In all cases, first MRI showed: prominent bilateral SN enlargement, bilateral dentate nuclei T2‐hyperintensity, and corpus callosum thinning. Iron deposition in the SN and globus pallidus (GP) only became evident later. Diffuse cerebral atrophy was present in 3 cases. Conclusions In this series, SN swelling and dentate nucleus T2 hyperintensity were early signs of BPAN, later followed by progressive iron deposition in the SN and GP. When clinical suspicion is raised, MRI is crucial for identifying early features suggesting this type of NBIA.Keywords:
Dentate nucleus
Putamen
Movement Disorders
Functional Magnetic Resonance Imaging Evidence for Disrupted Basal Ganglia Function in Schizophrenia
OBJECTIVE: This study was an examination of basal ganglia dysfunction in schizophrenia using functional magnetic resonance imaging (fMRI). METHOD: The authors used a motor sequencing task to investigate activation of the caudate, anterior putamen plus globus pallidus, and posterior putamen plus globus pallidus in eight subjects with schizophrenia and 12 group-matched comparison subjects. Differences in activation of the thalamus, the target of direct output from the globus pallidus, were also examined. RESULTS: The schizophrenia subjects showed significant bilateral deficits in the posterior putamen, globus pallidus, and thalamus but not the anterior putamen plus globus pallidus or caudate. Functional connectivity analysis revealed that the deficits in thalamic activation were related to deficits in posterior putamen and globus pallidus activation. CONCLUSIONS: These results provide fMRI evidence for basal ganglia dysfunction in subjects with schizophrenia and suggest that this deficit results in disrupted outflow to the thalamus. These deficits may underlie the behavioral impairments in goal-directed action observed in schizophrenia.
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Objective To observe the changes of brain iron in deep gray nucleus(including substantia nigra,red nucleus,globus pallidus,putamen,caudate nucleus)with susceptibility weighted imaging(SWI)in healthy people.Methods A total of 171 healthy adults(86 males and 85 females)were selected to perform SWI examination.All of volunteers were divided into five age groups in term of ten years:20-29(n=31),30-39(n=33),40-49(n=35),50-59(n=40)and 60-69(n=32).The signal values of substantia nigra,red nucleus,globus pallidus,putamen,caudate nucleus on SWI were quantificationally measured.The relationship between SWI signal values of deep brain gray nucleus and age was analyzed.Results In the 20-39 years old group,SWI signal values of substantia nigra,globus pallidus,red nucleus,caudate nucleus,putamen had statistical differences(P0.001).The SWI signal values changed in the smooth in caudate nucleus and globus pallidus during the period of 20-39 years old,and increased rapidly after 40 years old in all brain gray nucleus(especially red nucleus and putamen).Obvious correlations between age and SWI signal values were shown in substantia nigra(r=-0.759),red nucleus(r=-0.875),putamen(r=-0.880)and caudate nucleus(r=-0.524),while weakly correlation was displayed in globus pallidus(r=-0.211).Conclusion SWI provides a new method to detect brain iron and quantificationally measure the anatomical localization and age-related iron concentration changes.
Putamen
Red nucleus
Caudate nucleus
5-HT4 receptor
Susceptibility Weighted Imaging
Subthalamic Nucleus
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In 16 adult cats with electrolytically destructed external and internal parts of the globus pallidus and in 8 cats with destructed putamen direct strio-pallido-hypothalamic and strio-pallido-subthalamic pathways have been studied. Degeneration of the axonal preterminals and terminals have been examined in preparations treated after Nauta--Gygax, Nauta--Laidlow, Finck--Heimer with simultaneous additional staining of the nuclei with cresyl violet after Kawamura--Niimi. Direct pallido- and putamen-hypothalamic pathways to nuclei of the grey tubercle, posterior and lateral nuclei of the hypothalamus were stated. Direct pathways from the putamen to the subthalamic nucleus have been revealed, however, these pathways are represented in less degree than those of pallido-subthalamic connections. Direct pathways from the external portion of the globus pallidus and putamen to the subthalamic nucleus are more pronounced and represented by greater numbers of projections than those of strio-pallido-hypothalamic origin.
Putamen
Subthalamic Nucleus
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Objective To compare the volume of the basal ganglia in patients with Tourette syndrome(T S) and the normal volunteers and to explore the underlying anatomical basis of TS.Methods Thirty-one cases of TS (TS subjects),31 gender and age-matched subjects (the control subjects) were examined on a 3.0 T MRI system.The volume of the caudate nucleus,globus pallidus,putamen of the two sides and the brain volume were measured with volume analysis software,and the data were normalized according to the individual brain volume.Statistical analysis was performed using t test to compare between the TS subjects and the controls.Results The volume of the both sides of the caudate nucleus,putamen and globus pallidus of TS subjects were (4.11 ±0.12) and (3.76 ±0.11),(2.28 ±0.12)and(2.35 ±0.28),(4.98 ±0.20) and (4.89 ±0.31)cm3,while they were (4.88 ±0.19) and (4.30 ±0.12),(2.28 ±0.12)and (2.35 ± 0.28),(4.98 ± 0.20) and (4.89 ± 0.31) cm3 in the controls,respectively.There were significant differences in the bilateral caudate nucleus and globus pallidus between the TS subjects and control subjects (t =2.97,1.74,3.72,3.93,P ＜ 0.05),but there were no significant differences of the volume in the bilateral putamen between the TS and control subjects(t =0.47,1.31,P ＞0.05).The volume was not significantly different between the left and right caudate nucleus in the TS subjects (t =1.81,P ＞0.05),but the left volume of the caudate nucleus was bigger in the control subjects compared with the right volume,however,there was significant difference between the bilateral caudate nucleus in the control subjects (t =2.34,P ＜ 0.05).There were no differences of volume between the bilateral globus pallidus and putamen in both the TS and control subjects (t =1.12,1.44,1.68,0.38,P ＞ 0.05).Conclusion The abnormal volume of caudate nucleus,putamen,and the globus pallidus may be involved in the pathogenesis of TS.
Key words:
Tourette's symdrome; Magnetic resonance imaging
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Caudate nucleus
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Cerebral blood flow (CBF) images obtained using Xe-CT have a much higher spatial resolution than SPECT or PET images. The regional CBF (rCBF) of deep brain regions, the basal ganglia and thalamus, was able to be measured using Xe-CT in 6 subjects. Average rCBF was 87.1 +/- 20.7 ml/100 g/min in the caudate nucleus, 83.5 +/- 15.8 ml/100 g/min in the putamen, 50.0 +/- 8.7 ml/100 g/min in the globus pallidus and 88.9 +/- 12.4 ml/100 g/min in the thalamus. The average rCBF value of the globus pallidus was lower than the values of the caudate nucleus, putamen and thalamus. These observations may be explained by reduced cellularity of the globus pallidus in comparison to the other regions. SPECT and PET are not able to clearly demonstrate the globus pallidus on CBF images. However, precise rCBF values can be measured in the globus pallidus using Xe-CT.
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Caudate nucleus
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It has been well documented that high field magnetic resonance imaging can detect the location of physiological deposition of iron in the brain; such as in the globus pallidus, red nucleus, substantia nigra and dentate nucleus. The areas depict very low signal intensity in the long TR and the long TE imaging (T2 weighted image), because the iron enhances the T2 relaxation time. The purpose of this study was to determine the detectability of iron deposition in the brain by our 1.0-T high field MR, compared with 1.5-T MR which was used by most authors. We collected 160 MRI cases of the brain, and observed also signal void areas in the basal ganglion, brainstem or cerebellum. Our T2WI was TR 2000-3000 msec, TE 80-120 msec. We found that the 1.0-T MR clearly detected the iron deposition in the globus pallidus, red nucleus, substantia nigra and dentate nucleus of adults, with additional areas in the putamen and caudate nucleus of aged people. Shortcomings of the 1.0-T MRI for detecting iron deposition include: the margin of iron deposition was not very sharp in some cases, and for children under detectability was markedly inferior to the 1.5-T MR.
Dentate nucleus
Red nucleus
Putamen
Caudate nucleus
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High-field strength magnetic resonance imaging is an accurate clinical technique for detecting the relative distribution of ferritin in the brain. In normal adults, iron is found in highest concentrations in the globus pallidus, red nucleus, pars reticulata of the substantia nigra, and dentate nucleus of the cerebellum; its distribution is clearly mapped as signal hypointensity (darkness) on a T2-weighted image due to local-field heterogeneities produced by ferritin. Iron is absent at birth and increases in concentration in the putamen in the elderly. Poorly drug-responsive Parkinson's disease (multiple-system atrophy) is characterized by premature signal hypointensity in the putamen and caudate, while Hallervorden-Spatz disease exhibits abnormal hypointensity in the globus pallidus in children. Dyskinetic disorders often have abnormal signal hyperintensity (whiteness) in the putamen related to gliosis.
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Red nucleus
Caudate nucleus
Subthalamic Nucleus
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Carbon monoxide (CO) poisoning may result in focal and diffuse neuropathological changes, including basal ganglia lesions. The effect of CO poisoning on basal ganglia volumes over time is unclear. We assessed basal ganglia volumes longitudinally following CO poisoning. We prospectively enrolled 73 CO poisoned patients who underwent brain MR imaging on day 1 (baseline), 2 weeks, and 6 months post-CO poisoning. Basal ganglia volumes were obtained. One patient had bilateral globus pallidus lesions at two weeks and 6 months. Of the CO-poisoned patients 28% had volume reduction in at least one basal ganglia structure by 6 months, of which 21% had putamen, 15% had caudate, 15% had globus pallidus, and 16% had total basal ganglia volume reduction. Putamen volumes were significantly smaller from baseline to six months (p = 0.02). Verbal memory and mental processing speed correlated with smaller putamen and globus pallidus volumes. Carbon monoxide poisoning results in basal ganglia volume reduction 6 months post CO poisoning. Slow mental processing speed and impaired memory correlated with smaller putamen and globus pallidus volumes. Clinicians need to be aware of basal ganglia neuropathologic changes in the absence of observable lesions following CO poisoning.
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Carbon monoxide poisoning
Caudate nucleus
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Caudate nucleus
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