Biomaterial Database

[Striato-nigral degeneration (SND): a multisystem atrophy?]. 1990

M Bergmann, and K Schmidtke, and A Danek, and F Gullotta, and P Mehraein

Institut für Neuropathologie, Universität Münster.

Two cases of striato-nigral degeneration are reported. In case 1 the female patient showed a Parkinson syndrome, cardiac arrhythmias and vasomotor disturbances. Morphologically mainly the putamen and substantia nigra revealed severe atrophic changes according to the restricted form of striato-nigral degeneration (SND). In case 2 there was a disorder of the upper and lower motor neuron in a female patient. Morphologically this case was an example of a multisystem atrophy with changes in the striato-nigral, olivo-ponto-cerebellar systems and spinal motor and autonomic neurons. These cases demonstrate the variability of the striato-nigral degenerations which are met with in two forms: a "pure" form as in case one and as a part of multisystem atrophies. This is underlined in a review of 69 literature cases, which also shows that SND, Shy-Drager syndrome and olivo-ponto-cerebellar atrophy represent very probably different varieties of one and the same degenerative process.

UI	MeSH Term	Description	Entries
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D009410	Nerve Degeneration	Loss of functional activity and trophic degeneration of nerve axons and their terminal arborizations following the destruction of their cells of origin or interruption of their continuity with these cells. The pathology is characteristic of neurodegenerative diseases. Often the process of nerve degeneration is studied in research on neuroanatomical localization and correlation of the neurophysiology of neural pathways.	Neuron Degeneration,Degeneration, Nerve,Degeneration, Neuron,Degenerations, Nerve,Degenerations, Neuron,Nerve Degenerations,Neuron Degenerations
D009474	Neurons	The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the NERVOUS SYSTEM.	Nerve Cells,Cell, Nerve,Cells, Nerve,Nerve Cell,Neuron
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
D010302	Parkinson Disease, Secondary	Conditions which feature clinical manifestations resembling primary Parkinson disease that are caused by a known or suspected condition. Examples include parkinsonism caused by vascular injury, drugs, trauma, toxin exposure, neoplasms, infections and degenerative or hereditary conditions. Clinical features may include bradykinesia, rigidity, parkinsonian gait, and masked facies. In general, tremor is less prominent in secondary parkinsonism than in the primary form. (From Joynt, Clinical Neurology, 1998, Ch38, pp39-42)	Atherosclerotic Parkinsonism,Secondary Parkinsonism,Symptomatic Parkinson Disease,Parkinson Disease, Secondary Vascular,Parkinson Disease, Symptomatic,Parkinsonism, Secondary,Parkinsonism, Symptomatic,Secondary Vascular Parkinson Disease,Parkinsonism, Atherosclerotic,Secondary Parkinson Disease,Symptomatic Parkinsonism
D011699	Putamen	The largest and most lateral of the BASAL GANGLIA lying between the lateral medullary lamina of the GLOBUS PALLIDUS and the EXTERNAL CAPSULE. It is part of the neostriatum and forms part of the LENTIFORM NUCLEUS along with the GLOBUS PALLIDUS.	Nucleus Putamen,Nucleus Putamens,Putamen, Nucleus,Putamens,Putamens, Nucleus
D002421	Caudate Nucleus	Elongated gray mass of the neostriatum located adjacent to the lateral ventricle of the brain.	Caudatus,Nucleus Caudatus,Caudatus, Nucleus,Nucleus, Caudate
D003342	Corpus Striatum	Striped GRAY MATTER and WHITE MATTER consisting of the NEOSTRIATUM and paleostriatum (GLOBUS PALLIDUS). It is located in front of and lateral to the THALAMUS in each cerebral hemisphere. The gray substance is made up of the CAUDATE NUCLEUS and the lentiform nucleus (the latter consisting of the GLOBUS PALLIDUS and PUTAMEN). The WHITE MATTER is the INTERNAL CAPSULE.	Lenticular Nucleus,Lentiform Nucleus,Lentiform Nuclei,Nucleus Lentiformis,Lentiformis, Nucleus,Nuclei, Lentiform,Nucleus, Lenticular,Nucleus, Lentiform,Striatum, Corpus
D005260	Female		Females
D005917	Globus Pallidus	The representation of the phylogenetically oldest part of the corpus striatum called the paleostriatum. It forms the smaller, more medial part of the lentiform nucleus.	Paleostriatum,Pallidum,Pallidums