OBJECTIVEThe involvement of globus pallidus has been found in neonates with acute bilirubin encephalopathy (ABE), but so far the relationship between the severity of hyperbilirubinemia and changes of globus pallidus has not been studied further. The present study was conducted to understand possible relationship between the MRI signal changes of globus pallidus and severe hyperbilirubinemia in the neonates to provide evidences for diagnosis of bilirubin encephalopathy and prediction of outcome.

METHODSThirty-six neonates with severe hyperbilirubinemia (total serum bilirubin, i.e., TSB > 342 micromol/L) received MRI examination in a magnetic field with the strength of 1.5 - 3.0 Tesla at ages of 10 +/- 6 (2 - 34) days of life, of whom 15 were assessed as acute bilirubin encephalopathy (ABE). Routine T1WI with three kinds of sequences (SE, IR and FFE), T2WI and DWI with two kinds of sequences (EPI-SE and DW SSh SENSE) were applied. Two neuroradiologists who knew nothing about the clinical history analyzed MRI findings. TSB, unconjugated bilirubin (UCB), conjugated bilirubin (CB) and albumin (ALB) in all these neonates were measured with the same methods and analyzer.

RESULTSSymmetric hyperintense globus pallidus was shown on T1WI in 20 neonates, of whom three had symmetric hyperintense globus pallidus on T2WI (TSB = 745.3 micromol/L, 735.7 micromol/L, 707.6 micromol/L, respectively) at the same time. Remarkably higher TSB, molar ratio of TSB and ALB (B/A) and UCB were found in 20 neonates with hyperintense globus pallidus as compared to 16 cases without changes of globus pallidus (P = 0.000). No abnormal signal changes were found on DWI for all neonates; 9 neonates with TSB ranged from 342.0 micromol/L to 427.5 micromol/L did not show any abnormalities of globus pallidus, 3 of 7 neonates with TSB ranged from 427.5 micromol/L to 513.0 micromol/L, but 17 of 20 neonates with TSB more than 513.0 micromol/L showed distinct hyperintense globus pallidus. Hyperintense globus pallidus on T1WI was related to the severity of hyperbilirubinemia (Fisher's Exact Test, P = 0.000). Hyperintense globus pallidus on T1WI was found in all the 15 neonates with ABE (Fisher's Exact Test, P = 0.000), at the same time, hyperintense globus pallidus on T2WI was found in 3 cases with severe ABE. Six infants received the second MRI at ages from 2 months to 12 months, two of three infants with ABE in neonatal period showed the signal changes of globes pallidus from T1WI hyperintensity to T2WI hyperintensity and they developed cerebral palsy. The other one with normal signal showed hearing dysfunction. Another three infants without history of ABE did not show abnormal signals of globus pallidus and developed normally.

CONCLUSIONSThe symmetric involvement of globus pallidus with hyperintense signals on MRI T1WI indicates the severity of hyperbilirubinemia, which relates to time of exposure to hyperbilirubinemia and is a characteristic finding of ABE. The signal switch of hyperintense globus pallidus from T1WI to T2WI predicts poor outcome.

Bilirubin ; blood ; Globus Pallidus ; pathology ; Humans ; Hyperbilirubinemia, Neonatal ; diagnosis ; pathology ; Infant, Newborn ; Magnetic Resonance Imaging

Acute mountain sickness (AMS) occurs commonly in hikers who are rapidly exposed to high altitude environments. Despite the numerous reports of AMS, few studies have reported pallidal lesions associated with altitude sickness. A previously healthy 49-yr-old Korean patient, after ascent to 4,700 m, suffered symptoms consistent with AMS. After returning home, the patient showed changes in personality characterized by abulia, indifference, and indecisiveness. T2 weighted brain magnetic resonance imaging showed high signal lesions involving bilateral globus pallidus. Our case suggests that globus pallidus injury should be included in the differential diagnosis of patients with personality or cognitive change after recovery from AMS.
Altitude ; Altitude Sickness/*complications ; Behavior ; Brain/pathology ; Brain Damage, Chronic/*etiology/pathology ; Environment ; Globus Pallidus/*pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Mountaineering

OBJECTIVEVariations of the signal intensities in the magnetic resonance (MR) T(1)-weighted image (T(1)WI) of globus pallidus among manganese(Mn)-exposed workers were explored to provide a scientific basis for exposed biomarker of manganese-injured central nervous system (CNS).

METHODSThe brain MR T(1) and T(2) WI in eighteen male asymptomatic Mn-exposed, eight manganism and nine healthy control workers were examined routinely by adopting a 1.5 Tesla signal superconducting system. The SIGP and the signal intensity in frontal white matter (SIFWM) in the same side were determined, then pallidal index (PI) was calculated. Concentration of MnO(2) in workplaces and content of manganese in red blood cell (MnRBC) among workers were respectively determined by flame atomic absorption spectrometer (AAS) and inductively coupled plasma-atomic emission spectrophotometry (ICP-AES). The follow-up investigation in the eight high Mn-exposed workers was made one year later.

RESULTSThe results showed that the median of air MnO(2) in smelting workplace was 0.64 mg/m(3)(0.07 - 5.40 mg/m(3)), which were respective 0.56 mg/m(3)(0.09 - 1.71 mg/m(3)) in power distribution room (low Mn-exposure) and 0.89 mg/m(3) (0.07 - 5.40 mg/m(3)) in furnace (high Mn-exposure). PI in the Mn-exposed and high Mn-exposed workers were both higher than those of the manganism and control workers(116.4 +/- 8.2, 119.0 +/- 7.9, 105.3 +/- 8.4 and 102.2 +/- 1.5, respectively. Mn vs control, t' = 7.146, P = 0.000; Mn vs manganism, t = 3.181, P = 0.004. High Mn-exposure vs control, t' = 7.446, P = 0.000; high Mn-exposure vs manganism, t = 3.763, P = 0.001). The increased signal in T(1)WI of globus pallidus was observed in Mn-exposed workers, especially in high Mn-exposed workers. The content of manganese in red blood cell of Mn-exposed and control workers was significantly higher than those of the manganism workers [(151.6 +/- 40.5) ng/ml, (149.2 +/- 21.3) ng/ml, (154.5 +/- 46.6) ng/ml, (144.4 +/- 14.2) ng/ml, (20.8 +/- 7.4) ng/ml respectively. The difference was significant in statistics. Manganism vs control, t = 20.206, P = 0.000; manganism vs Mn, t' = 13.144, P = 0.000; manganism vs low and high Mn, t' = 12.964, 9.957, respectively, P = 0.000]. Only a decreased median of air MnO(2) in furnace was found one year later (0.89, 0.31 mg/m(3), Z = -2.142, P = 0.032). The difference was significant in statistics.

CONCLUSIONOur data suggests that SIGP of MR T(1)WI among workers was obviously increased by manganese-exposure. PI may be taken as the signal of CNS injury which was induced by manganese-exposure.

Adult ; Air Pollutants, Occupational ; analysis ; Case-Control Studies ; Globus Pallidus ; pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Manganese Poisoning ; pathology ; Middle Aged ; Occupational Exposure

Brain ; pathology ; Globus Pallidus ; pathology ; Hepatitis B, Chronic ; complications ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Spinal Cord Diseases ; diagnosis ; etiology

We investigated a topographical distribution of managanese, and immunohistochemical density of tyrosine hydroxylase (TH), and histopathologic findings in globus pallidus and substantia nigra according to manganese dose and time course in the brain of rats which received MnCl2 intravenously. Topographical distribution of manganese was also investigated after injection of FeCl2. The manganese concentrations of brain in control and experimental group were highest in pituitary gland and thalamus, and lowest in the cerebral cortex. The manganese concentration of blood was increased proportionally to the dose administered, and the biological half-life of blood manganese was between 21 and 42 days. The manganese concentrations of brain were increased proportionally to the dose, and increase rate was highest in olfactory bulb, and the biological half-lives of brain manganese ranged from 42 days to 90 or more days; the longest were observed in pituitary gland, medulla oblongata and cerebral cortex. In case of administration of FeCl2, the manganese concentrations of brain were higher than that of control group in dose of 2.5 mg/kg, and decreased proportionally to the administered dose, resulting in lower level compared with control group in high dose of FeCl2 administered. Significantly decreased number of nerve cell and increased gliosis in globus pallidus were observed in experimental group, which were closely correlated with the duration after manganese injection, but no significant change of number of nerve cell expressing TH and gliosis were observed in substantia nigra. Density of immunohistochemical reaction for TH in globus pallidus made little difference between control and experimental group. These results suggest that pathology of manganese intoxication is caused by the loss of nerve cells in globus pallidus, and closely correlated with the duration after manganese exposure.
Animals ; Brain* ; Cerebral Cortex ; Gliosis ; Globus Pallidus ; Half-Life ; Manganese* ; Medulla Oblongata ; Neurons ; Olfactory Bulb ; Pathology ; Pituitary Gland ; Rats* ; Substantia Nigra ; Thalamus ; Tyrosine 3-Monooxygenase

Calcinosis ; diagnostic imaging ; etiology ; Child ; Child, Preschool ; Female ; Globus Pallidus ; pathology ; Humans ; Male ; Osteogenesis Imperfecta ; complications ; genetics ; Skull ; diagnostic imaging ; Tomography, X-Ray Computed

BACKGROUND: Quantitative susceptibility mapping (QSM) has been used to measure iron accumulation in the deep nuclei of patients with Parkinson's disease (PD). This study examined the relationship between non-motor symptoms (NMSs) and iron accumulation in the deep nuclei of patients with PD. METHODS: The QSM data were acquired from 3-Tesla magnetic resonance imaging (MRI) in 29 patients with early PD and 19 normal controls. The Korean version of the NMS scale (K-NMSS) was used for evaluation of NMSs in patients. The patients were divided into high NMS and low NMS groups. The region-of-interest analyses were performed in the following deep nuclei: red nucleus, substantia nigra pars compacta, substantia nigra pars reticulata, dentate nucleus, globus pallidus, putamen, and head of the caudate nucleus. RESULTS: Thirteen patients had high NMS scores (total K-NMSS score, mean = 32.1), and 16 had low NMS scores (10.6). The QSM values in the deep were not different among the patients with high NMS scores, low NMS scores, and controls. The QSM values were not correlated linearly with K-NMSS total score after adjusting the age at acquisition of brain MRI. CONCLUSION: The study demonstrated that the NMS burdens are not associated with iron accumulation in the deep nuclei of patients with PD. These results suggest that future neuroimaging studies on the pathology of NMSs in PD should use more specific and detailed clinical tools and recruit PD patients with severe NMSs.
Basal Ganglia ; Brain ; Caudate Nucleus ; Cerebellar Nuclei ; Globus Pallidus ; Head ; Humans ; Iron* ; Magnetic Resonance Imaging ; Neuroimaging ; Parkinson Disease* ; Pars Compacta ; Pars Reticulata ; Pathology ; Putamen ; Red Nucleus

OBJECTIVETo assess the efficacy of phase-weighted imaging in differentiating multiple system atrophy (MSA) from idiopathic Parkinson disease (IPD).

METHODSA phase-weighted sequence was performed covering striatum in 21 patients with MSA, 20 patients with IPD, and 26 age-matched healthy volunteers after conventional brain magnetic resonance imaging examination on a 3.0T magnetic resonance imaging system. An experienced neuroradiologist who were blind to the clinical diagnosis applied region of interest (ROI) on post-processing phase images to measure the phase shift value of bilateral globus pallidus, putamen, and caudate head.

RESULTSThe phase shift values at bilateral putamen of MSA group were significantly lower than those of IPD group and control group (P < 0.05), while phase shift values at other locations of MSA group were not statistically different from those of other groups. The phase shift values showed no statistical difference between IPD group and control group at all regions.

CONCLUSIONQuantitative phase-weighted imaging is a useful sequence to reflect the abnormal iron accumulation in brains of patients with movement disorders and is helpful in the differential diagnosis between MSA and IPD.

Aged ; Aged, 80 and over ; Case-Control Studies ; Caudate Nucleus ; pathology ; Diagnosis, Differential ; Female ; Globus Pallidus ; pathology ; Humans ; Magnetic Resonance Imaging ; methods ; Male ; Middle Aged ; Multiple System Atrophy ; diagnosis ; Parkinson Disease ; diagnosis ; Putamen ; pathology

OBJECTIVETo explore the clinical and genetic characteristics of patients with dentatorubro-pallidoluysian atrophy (DRPLA).

METHODSDNA analysis for DRPLA gene was performed in two patients. Clinical features and genetic testing of Chinese DRPLA patients reported in the literature were reviewed in terms of initial symptoms, CAG repeat and age of onset.

RESULTSBoth families were confirmed by genetic analysis. In family 1, the number of CAG repeat in the proband, his brother and his mother was determined respectively as 8/65, 8/53 and 8/18. In family 2, the number of CAG repeat was respectively 13/63, 13/18, 18/52 and 13/13 in the proband, his brother, his father and his mother. The size of the expanded CAG repeats has inversely correlated with the age at onset (P<0.05, r=- 0.555). The age at onset of epilepsy was 10 and that for the onset of ataxia is forty years in initial symptom.

CONCLUSIONThe clinical characteristics of DRPLA include epilepsy, ataxia and cognitive impairment. The initial symptoms are epilepsy in adolescence and ataxia in adults. The size of expanded CAG repeats inversely correlates with the age at onset. The initial symptoms are different with different age of onset. It is difficult to diagnose DRPLA at an early stage.

Adolescent ; Adult ; Aged ; Atrophy ; genetics ; Basal Ganglia Diseases ; diagnosis ; genetics ; DNA Mutational Analysis ; Dentate Gyrus ; pathology ; Family Health ; Female ; Globus Pallidus ; pathology ; Humans ; Male ; Middle Aged ; Nerve Tissue Proteins ; genetics ; Pedigree ; Trinucleotide Repeat Expansion ; genetics ; Young Adult

In recent years, several radiotracers that selectively bind to pathological tau proteins have been developed. Evidence is emerging that binding patterns of in vivo tau positron emission tomography (PET) studies in Alzheimer's disease (AD) patients closely resemble the distribution patterns of known neurofibrillary tangle pathology, with the extent of tracer binding reflecting the clinical and pathological progression of AD. In Lewy body diseases (LBD), tau PET imaging has clearly revealed cortical tau burden with a distribution pattern distinct from AD and increased cortical binding within the LBD spectrum. In progressive supranuclear palsy, the globus pallidus and midbrain have shown increased binding most prominently. Tau PET patterns in patients with corticobasal syndrome are characterized by asymmetrical uptake in the motor cortex and underlying white matter, as well as in the basal ganglia. Even in the patients with multiple system atrophy, which is basically a synucleinopathy, ¹⁸F-flortaucipir, a widely used tau PET tracer, also binds to the atrophic posterior putamen, possibly due to off-target binding. These distinct patterns of tau-selective radiotracer binding in the various degenerative parkinsonisms suggest its utility as a potential imaging biomarker for the differential diagnosis of parkinsonisms.
Alzheimer Disease ; Basal Ganglia ; Diagnosis, Differential ; Electrons ; Globus Pallidus ; Humans ; Lewy Bodies ; Mesencephalon ; Motor Cortex ; Multiple System Atrophy ; Neurofibrillary Tangles ; Parkinsonian Disorders ; Pathology ; Positron-Emission Tomography ; Putamen ; Supranuclear Palsy, Progressive ; tau Proteins ; White Matter