Humans ; Neurons ; Olivary Nucleus ; Superior Olivary Complex

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*

Hypertrophic olivary degeneration resulting from lesions of the dento-rubro-olivary pathway, also called Guillain-Mollaret-triangle, has been described previously in a number of cases. Reports about bilateral hypertrophic olivary degeneration of the inferior olivary nuclei are very limited, and the magnetic resonance imaging findings of hypertrophic olivary degeneration in Wilson disease have not yet been described to the best of our knowledge. Herein, we present the first report of bilateral hypertrophic olivary degeneration diagnosed by magnetic resonance imaging in a patient suffering from Wilson disease.
Diagnosis, Differential ; Hepatolenticular Degeneration/*pathology ; Humans ; Hypertrophy/pathology ; Magnetic Resonance Imaging/*methods ; Male ; Nerve Degeneration/*pathology ; Olivary Nucleus/*pathology ; Young Adult

BACKGROUND: Leber's hereditary optic neuropathy (LHON) is a mitochondrial disorder with optic nerve atrophy. Although there are no other associated neurological abnormalities in most cases of LHON, cases of "LHON plus" have been reported. CASE REPORT: The proband was a 37-year-old man who had visual and gait disturbances that had first appeared at 10 years of age. He showed horizontal gaze palsy, gaze-evoked nystagmus, dysarthria, and cerebellar ataxia. Brain and orbit MRI disclosed atrophy of the optic nerve and cerebellum, and degenerative changes in the bilateral inferior olivary nucleus. Mutational analyses of mitochondrial DNA identified the coexistence of heteroplasmic G11778A and homoplasmic T3394C mutations. CONCLUSIONS: These results suggest that the combination of G11778A and T3394C mutations leads to an atypical LHON phenotype.
Adult ; Atrophy ; Brain ; Cerebellar Ataxia ; Cerebellum ; DNA, Mitochondrial ; Dysarthria ; Gait ; Humans ; Mitochondria ; Mitochondrial Diseases ; Olivary Nucleus ; Optic Atrophy, Hereditary, Leber ; Optic Nerve ; Orbit ; Paralysis ; Phenotype

OBJECTIVE: While many factors contribute to aging, changes in calcium homeostasis and calcium related neuronal processes are likely to be important. High intracellular calcium is toxic to cells and alterations in calcium homeostasis are associated with changes in calcium-binding proteins, which confine free Ca2+. We therefore assayed the expression of the calcium binding proteins calretinin and calbindin in the central auditory nervous system of rats. METHODS: Using antibodies to calretinin and calbindin, we assayed their expression in the cochlear nucleus, superior olivary nucleus, inferior colliculus, medial geniculate body and auditory cortex of young (4 months old) and aged (24 months old) rats. RESULTS: Calretinin and calbindin staining intensity in neurons of the cochlear nucleus was significantly higher in aged than in young rats (p<0.05) The number and staining intensity of calretinin-positive neurons in the inferior colliculus, and of calbindin-positive neurons in the superior olivary nucleus were greater in aged than in young rats (p<0.05). CONCLUSION: These results suggest that auditory processing is altered during aging, which may be due to increased intracellular Ca2+ concentration, consequently leading to increased immunoreactivity toward calcium-binding proteins.
Aged ; Aging ; Animals ; Antibodies ; Auditory Cortex ; Auditory Pathways ; Calcium ; Calcium-Binding Protein, Vitamin D-Dependent ; Calcium-Binding Proteins ; Cochlear Nucleus ; Geniculate Bodies ; Homeostasis ; Humans ; Inferior Colliculi ; Nervous System ; Neurons ; Olivary Nucleus ; Rats

OBJECTIVE: To investigate the distribution and morphology of olivocochlear neurons of superior olivary complex in cats. METHODS: Eight adult cats were divided into 2 groups randomly. Cholera toxin B subunit was injected to the left cochlea and fluoro-gold was injected to the right cochlea in the experimental group (n=5). Saline was injected to bilateral cochlea in the control group (n=3). Brainstem tissue was sectioned serially. All of the sections were immunohistochemically treated with ABC and stained with DAB, and then the labelled olivocochlear neurons were observed. RESULTS: The labelled olivocochlear neurons in the experimental group were 2 518 in total. Of them, the number of lateral olivocochlear (LOC) neurons was 1 738 (69.0%), mainly located in the middle of the pons, predominantly projected ipsilaterally. The total of medial olivocochlear (MOC) neurons was 780 (31%), mainly located in dorsomedial periolivary nucleus, medial nucleus of the trapezoid body and ventral nucleus of the trapezoid body, mainly distributed in the rostral extent of the pons, predominantly projected contralaterally. CONCLUSION In the distribution of olivocochlear neurons in cats, LOC neurons mainly project to the ipsilateral. While the projection of MOC neurons is predominantly contralateral, the distribution of MOC neurons is more adjacent to the rostral extent of the pons than LOC neurons.
Animals ; Auditory Pathways ; cytology ; Brain Stem ; cytology ; Cats ; Cholera Toxin ; administration & dosage ; Cochlea ; innervation ; Cochlear Nucleus ; cytology ; Female ; Injections ; Male ; Neurons ; cytology ; Neurons, Efferent ; cytology ; Olivary Nucleus ; cytology

OBJECTIVETo observe the effect of Astragalus membranaceus (AM) on insulin-like growth factor 1 (IGF-1) expression in a rat model of olivo-cerebellar degeneration and assess the neuroprotective actions of AM meanwhile.

METHODRats model of olivo-cerebellar degeneration was established by using 3-acetylpyridine. The effect of AM on the expression of Calbindin D-28K in inferior olive (IO) neurons by immunohistochemistry, the serum IGF-1 level by Elisa, the IGF-1 mRNA level in the cerebellum by RT-PCR were detected respectively.

RESULTAM effectively improve the serum IGF-1 level, Cerebellar IGF-1 mRNA level and the survival of the 10 neurons in a rat model of olivo-cerebellar degeneration, even at a lower dose (9 g x kg(-1)), and the effect was in a dose-dependent manner.

CONCLUSIONAM could effectively upregulate the IGF-1 expression in the rat model of olivo-cerebellar degeneration, and have neuroprotective effect on IO neurons.

Animals ; Astragalus membranaceus ; chemistry ; Calbindins ; Cerebellum ; drug effects ; metabolism ; Dose-Response Relationship, Drug ; Drugs, Chinese Herbal ; administration & dosage ; isolation & purification ; pharmacology ; Enzyme-Linked Immunosorbent Assay ; Gene Expression ; drug effects ; Immunohistochemistry ; Insulin-Like Growth Factor I ; biosynthesis ; genetics ; Male ; Motor Activity ; drug effects ; Neuroprotective Agents ; administration & dosage ; isolation & purification ; pharmacology ; Olivary Nucleus ; drug effects ; metabolism ; Plants, Medicinal ; chemistry ; Pyridines ; RNA, Messenger ; biosynthesis ; genetics ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Reverse Transcriptase Polymerase Chain Reaction ; S100 Calcium Binding Protein G ; metabolism ; Spinocerebellar Degenerations ; blood ; chemically induced ; metabolism

The dorsolateral medullary syndrome (Wallenberg's syndrome) is produced by infarction of a wedge of lateral medulla posterior to the inferior olivary nucleus, and is usually caused by vertebral artery occlusion. Ipsilateral axial lateropulsion as an initial symptom of vertebral artery occlusion is rare, and the responsible anatomical structure is still uncertain. Here we describe a patient presenting with ipsilateral axial lateropulsion as an initial symptom of vertebral artery occlusion.
Humans ; Infarction* ; Lateral Medullary Syndrome ; Magnetic Resonance Imaging ; Olivary Nucleus ; Vertebral Artery

Hypertrophic olivary degeneration (HOD) is regarded as a secondary degenerative change subsequent to the formation of lesions in the "Guillain-Mollaret Triangle," and this is the result of the loss of transsynaptic neurologic input to the inferior olivary nucleus. HOD usually occurs unilaterally, but bilateral hypertrophic olivary degeneration is known to be rare. We experienced one case of this lesion, and we report here on the bilateral HOD that was secondary to infarction of the brainstem and cerebellum.
Brain Stem* ; Cerebellum* ; Infarction* ; Olivary Nucleus*

The development of involuntary movement by the lesion in the brainstem and spinal cord has been rarely reported. We report a 56-year-old man who suffered from bilateral dystonic tremor and left hemiparesis by an acute cervicomedullary infarction. The patient had acute infarcted lesion on the anterior-medial cervicomedullary junction located at the inferior olivary nucleus. Current study suggests that the involvement of inferior olivary nucleus in lower medulla could be a cause to develop dystonic tremor by cervicomedullary lesions.
Brain Stem ; Dyskinesias ; Dystonia ; Humans ; Infarction* ; Middle Aged ; Olivary Nucleus ; Paresis* ; Spinal Cord ; Tremor*