[Olivopontocerebellar atrophy in the context of progressive cerebellar atrophies (a clinico-anatomical study)]. 1990

C Ionel
Clinica de Neurologie, Spital Colentina Bucuresti.

The study of 15 cases of progressive cerebellar atrophies, and especially of the olivopontocerebellar atrophy, that was investigated both clinically and anatomically, has attempted to evidence particularities and correlations existing between these two types of atrophy. Olivopontocerebellar atrophy appears to be an abiotrophy of the cerebellum, considered to be spontaneous, sporadic, and sometimes with a hereditary familial background. It is a systemic disease, predominantly of the neocerebellum and involving the cerebellopedal system. It is at the opposite end of Holmes-type atrophies, and of the cerebello-olivary atrophies of the young (I. T. Niculescu, Th. Hornet, 1936) which mainly involve the paleocerebellum. The disease has a polymorphous symptomatology, it has a slow, progressive evolution with mostly cerebellar signs, with extrapyramidal phenomena and psychical disturbances due to lesions of the telencephalic pathways, and sometimes of the spinal, cerebellar and bulbar proprioceptive afferences, with spinal and bulbar involvement (Cezar Ionel, 1949, 1972).

UI MeSH Term Description Entries
D008297 Male Males
D009847 Olivary Nucleus A brainstem nuclear complex. in the hindbrain, also referred to as the olivary body. The olivary nuclear complex is a part of the MEDULLA OBLONGATA and the PONTINE TEGMENTUM. It is involved with motor control and is a major source of sensory input to the CEREBELLUM. Basal Nucleus, Olivary,Nucleus Basalis, Olivary,Olivary Body,Olivary Complex,Olivary Nuclei,Complex, Olivary,Nucleus, Olivary,Nucleus, Olivary Basal,Olivary Basal Nucleus,Olivary Bodies
D009849 Olivopontocerebellar Atrophies A group of inherited and sporadic disorders which share progressive ataxia in combination with atrophy of the CEREBELLUM; PONS; and inferior olivary nuclei. Additional clinical features may include MUSCLE RIGIDITY; NYSTAGMUS, PATHOLOGIC; RETINAL DEGENERATION; MUSCLE SPASTICITY; DEMENTIA; URINARY INCONTINENCE; and OPHTHALMOPLEGIA. The familial form has an earlier onset (second decade) and may feature spinal cord atrophy. The sporadic form tends to present in the fifth or sixth decade, and is considered a clinical subtype of MULTIPLE SYSTEM ATROPHY. (From Adams et al., Principles of Neurology, 6th ed, p1085) Dejerine-Thomas Syndrome,Olivopontocerebellar Atrophy, Idiopathic,Olivopontocerebellar Hypoplasia,Familial Olivopontocerebellar Atrophy,Inherited Olivopontocerebellar Atrophy,Nonfamilial Olivopontocerebellar Atrophy,Olivo-Ponto-Cerebellar Atrophy,Olivo-Ponto-Cerebellar Degeneration,Olivopontocerebellar Atrophy,Olivopontocerebellar Degeneration,Pontoolivocerebellar Atrophy,Presenile Ataxia,Ataxia, Presenile,Atrophy, Familial Olivopontocerebellar,Atrophy, Idiopathic Olivopontocerebellar,Atrophy, Inherited Olivopontocerebellar,Atrophy, Nonfamilial Olivopontocerebellar,Atrophy, Olivo-Ponto-Cerebellar,Atrophy, Olivopontocerebellar,Atrophy, Pontoolivocerebellar,Degeneration, Olivo-Ponto-Cerebellar,Degeneration, Olivopontocerebellar,Dejerine Thomas Syndrome,Familial Olivopontocerebellar Atrophies,Hypoplasia, Olivopontocerebellar,Idiopathic Olivopontocerebellar Atrophies,Idiopathic Olivopontocerebellar Atrophy,Inherited Olivopontocerebellar Atrophies,Nonfamilial Olivopontocerebellar Atrophies,Olivo Ponto Cerebellar Atrophy,Olivo Ponto Cerebellar Degeneration,Olivo-Ponto-Cerebellar Degenerations,Olivopontocerebellar Atrophies, Familial,Olivopontocerebellar Atrophies, Nonfamilial,Olivopontocerebellar Atrophy, Familial,Olivopontocerebellar Atrophy, Inherited,Olivopontocerebellar Atrophy, Nonfamilial,Olivopontocerebellar Degenerations,Olivopontocerebellar Hypoplasias,Pontoolivocerebellar Atrophies,Presenile Ataxias,Syndrome, Dejerine-Thomas
D011149 Pons The front part of the hindbrain (RHOMBENCEPHALON) that lies between the MEDULLA and the midbrain (MESENCEPHALON) ventral to the cerebellum. It is composed of two parts, the dorsal and the ventral. The pons serves as a relay station for neural pathways between the CEREBELLUM to the CEREBRUM. Pons Varolii,Ponte,Pons Varolius,Pontes,Varolii, Pons,Varolius, Pons
D011689 Purkinje Cells The output neurons of the cerebellar cortex. Purkinje Cell,Purkinje Neuron,Purkyne Cell,Cell, Purkinje,Cell, Purkyne,Cells, Purkinje,Cells, Purkyne,Neuron, Purkinje,Neurons, Purkinje,Purkinje Neurons,Purkyne Cells
D002531 Cerebellum The part of brain that lies behind the BRAIN STEM in the posterior base of skull (CRANIAL FOSSA, POSTERIOR). It is also known as the "little brain" with convolutions similar to those of CEREBRAL CORTEX, inner white matter, and deep cerebellar nuclei. Its function is to coordinate voluntary movements, maintain balance, and learn motor skills. Cerebella,Corpus Cerebelli,Parencephalon,Cerebellums,Parencephalons
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001284 Atrophy Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes. Atrophies

Related Publications

C Ionel
January 1984, Advances in neurology,
C Ionel
June 1953, Medicina contemporanea (Lisbon, Portugal),
C Ionel
December 1988, The Medical journal of Malaysia,
C Ionel
April 1891, Buffalo medical and surgical journal,
C Ionel
March 1987, Electromyography and clinical neurophysiology,
Copied contents to your clipboard!