Holmes' tremor is a condition characterized by a mixture of postural, rest, and action tremors due to midbrain lesions in the vicinity of the red nucleus. Hypertrophic olivary degeneration (HOD) is a rare type of neuronal degeneration involving the dento-rubro-olivary pathway and may present clinically as Holmes tremor. We report on a 59-year-old female patient who developed Holmes tremor in association with bilateral HOD, following brain stem hemorrhage.
Brain Stem* ; Female ; Hemorrhage* ; Humans ; Mesencephalon ; Middle Aged ; Neurons ; Olivary Nucleus ; Red Nucleus ; Tremor*

Hallervorden-Spatz disease is a rare, autosomal recessive disorder of mainly early childhood which is characterized by pigmentary degeneration of the globus pallidus, substantia nigra, and red nucleus. Clinically it manifests various symptoms and signs of extrapyramidal and pyramidal involvement. Authors report a 28-year-old female patient with suspected Hallervorden-Spatz disease in the aspects of clinical and MRI findings suggesting metal deposition in the globus pallidus, substantia nigra, and red nucleus on both side.
Adult ; Female ; Globus Pallidus ; Humans ; Magnetic Resonance Imaging ; Pantothenate Kinase-Associated Neurodegeneration* ; Red Nucleus ; Substantia Nigra

BACKGROUND AND PURPOSE: Previous T2 relaxometry studies have provided evidence for regional brain iron deficiency in patients with restless legs syndrome (RLS). Measurement of the iron content in several brain regions, and in particular the substantia nigra (SN), in early- and late-onset RLS patients using T2 relaxometry have yielded inconsistent results. In this study the regional iron content was assessed in patients with early- and late-onset RLS using magnetic resonance imaging (MRI), and compared the results with those in controls. METHODS: Thirty-seven patients with idiopathic RLS (20 with early onset and 17 with late onset) and 40 control subjects were studied using a 3.0-tesla MRI with a gradient-echo sampling of free induction decay and echo pulse sequence. The regions of interest in the brain were measured independently by two trained analysts using software known as medical image processing, analysis, and visualization. The results were compared and a correlation analysis was conducted to investigate which brain areas were related to RLS clinical variables. RESULTS: The iron index in the SN was significantly lower in patients with late-onset RLS than in controls (p=0.034), while in patients with early-onset RLS there was no significant difference. There was no significant correlation between the SN iron index of the late-onset RLS group and clinical variables such as disease severity. CONCLUSIONS: Late-onset RLS is associated with decreased iron content in the SN. This finding supports the hypothesis that regional brain iron deficiency plays a role in the pathophysiology of late-onset RLS.
Brain* ; Humans ; Iron ; Magnetic Resonance Imaging* ; Red Nucleus ; Restless Legs Syndrome* ; Substantia Nigra

The common causal lesions of pure dysarthria syndrome were known as anterior or posterior internal capsule, genu of internal capsule, adjacent corona radiata, or pons. And there are a few reports showed that bilateral small thalamic infarctions or paravermal cerebellar infarction also caused pure dysarthria. The rostral cerebellum, especially paravermal region is thought to have a major role in coordination of speech. The paravermal region is Intimately connected to caudal red nucleus. We report a patient with acute dysarthria and minlmal contralateral limb ataxia, in whom the causal lesion was in caudal red nucleus and superior part of decussation of brachium conjunctivum.
Ataxia ; Cerebellum ; Dysarthria* ; Humans ; Infarction* ; Internal Capsule ; Mesencephalon* ; Pons ; Red Nucleus

AIMTo explore an accurate neurophysiological technique that demonstrates small functional differences after spinal cord injury and assesses therapeutic interventions.

METHODSA modified weight drop (WD) technique was used at T8 in rats to build graded spinal cord injury model. Rubrospinal MEPs were recorded at T13 epidurally to monitor spinal cord function in end week 4 after graded spinal cord injury. The efficacy of this techniques to monitor spinal cord function was compared to BBB locomotor rating scale and histologic evaluation.

RESULTSA characteristic peak complex of rubrospinal MEPs in sham-operated group consisted of 5-7 positive waves and 4-5 negative waves emerging after red nucleus stimulation. The summed peak to peak amplitude (for practical reasons, called peak amplitude) was (195.25 +/- 34.35) microV and decreased following spinal cord injury. The latency of the first peak (positive wave) was (1.57 +/- 0.15) ms and prolonged following spinal cord injury. Significant Linear relationship existed between the peak amplitude and the BBB scores (r = 0.79) and between the peak amplitude and the residual matter obtained from the section with maximum tissue damage( r = 0.87). The close relationship between the latency of the first peak and the BBB scores (r = -0.88) and between the latency of the first peak and residual matter (r = -0.86) were observed.

CONCLUSIONAmplitudes and latencies of rubrospinal MEPs are very valuable parameters to demonstrate small function differences. Rubrospinal MEPs can be used as a reliable measure for motor function prognosis after spinal cord injury.

Animals ; Evoked Potentials, Motor ; Male ; Rats ; Rats, Wistar ; Red Nucleus ; physiopathology ; Spinal Cord Injuries ; pathology ; physiopathology

Animals ; Electric Stimulation ; Neurons ; pathology ; physiology ; Pain Threshold ; Rats ; Rats, Sprague-Dawley ; Red Nucleus ; pathology ; physiology

PURPOSE: Electrophysiologically assess conduction pathways of the pyramidal and extrapyramidal systems in rats and predict the pathways involved in spinal cord injuries. METHOD: The motor area of the cerebral cortex, medullary reticular nucleus, lateral vestibular nucleus, and red nucleus of adult Sprague-Dawley rats were stimulated with microelectrodes. Laminectomies were performed at the C6, T10 and L2 cord level. Field potentials evoked by stimulation of the cerebral cortex and the three motor nuclei were recorded with a glass microelectrode of 1.5~2.5 Mohm filled with 0.2M NaCl. To construct a cross-sectional map of field potentials, recording was made in 7 tracks equally spaced across the spinal cord. In each track, field potentials were recorded at seven equally spaced points from the ventral to dorsal cord. RESULT: Stimulation of the cerebral motor cortex evoked 5 wanes, such as P1, P2, P3, P4, P5. P1 was monitored mainly in the bilateral dorsal half of the spinal cord and other wades mainly in the ventral half of the spinal cord. With lateral vestibular nucleus stimulation, 1 or 2 short duration biphasic waves followed by a longer duration positive wave were monitored mainly in the ipsilateral ventrolateral funiculus of the cord. Field potentials produced by stimulating the medullary reticular nucleus were shown mainly in the ventromedial funiculus, and their latencies were longer than those from the vestibular nucleus stimulation. Field potentials generated by the stimulation of the red nucleus were monitored mainly in the dorsolateral funiculus. CONCLUSION: motor evoked potential is clinically useful to evaluate the descending pathways of the spinal cord.
Adult ; Animals ; Cerebral Cortex ; Evoked Potentials, Motor* ; Glass ; Humans ; Laminectomy ; Microelectrodes ; Motor Cortex ; Rats* ; Rats, Sprague-Dawley ; Red Nucleus ; Spinal Cord ; Spinal Cord Injuries ; Vestibular Nucleus, Lateral

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

PURPOSE: Metabolic encephalopathy is not an infrequent condition. However it is very difficult to diagnose and treat because of its various causes and clinical manifestations. Our purpose was to clarify the cause of metabolic encephalopathy by evaluation of MR findings and clinical features. METHODS: We reviewed MR images and clinical features for 25 patients with metabolic encephalopathy who showed abnormal signal changes on the MR images with neurologic deterioration. RESULTS: The 25 patients had underlying diseases such as chronic liver disease (n=16) or renal failure (n=9). The MR findings showed significant differences in the involved sites according to the underlying disease. In 10 of the 16 patients with liver disease, corpus callosal involvement was observed. Red nucleus involvement was seen in 6 patients, dentate nucleus involvement in 5 patients. These lesions were seen to have a high signal intensity on the diffusion weighted image. Contrary to liver disease, encephalopathy with renal disease showed typical central pontine myelinolysis in 6 of the 9 patients and a relatively benign clinical course. CONCLUSION: Our results showed that the typically involved site and clinical manifestations depended on the underlying disease. We think that involvement of the corpus callosum, the red nucleus, and the dentate nucleus is a typical pattern of injury in metabolic encephalopathy with chronic liver disease and that these findings will be helpful for diagnosing and treating metabolic encephalopathy.
Brain Diseases, Metabolic* ; Cerebellar Nuclei ; Corpus Callosum ; Diffusion ; Humans ; Liver Diseases ; Myelinolysis, Central Pontine ; Red Nucleus ; Renal Insufficiency

Claude syndrome is a well known midbrain syndrome which is characterized by ipsilateral oculomotor nerve palsy and contralateral cerebellar ataxia by the lesion of the red nucleus. Although this syndrome was reported as early as in 1924 by Claude, only a few cases have been reported. Moreover, the midbrain infarction as a cause of Claude syndrome has quite rarely been described. Firstly, we report a 61-year-old patient with partial oculomotor nerve palsy and contralateral cerebellar ataxia who demonstrated an infarction just caudal to the red nucleus on MRI. Secondly, we also discuss the probable vertical fascicular arrangement of the oculomotor nerve in the midbrain.
Brain Stem Infarctions* ; Cerebellar Ataxia ; Humans ; Infarction ; Magnetic Resonance Imaging ; Mesencephalon* ; Middle Aged ; Oculomotor Nerve ; Oculomotor Nerve Diseases ; Red Nucleus