Striatonigral Degeneration

A sporadic neurodegenerative disease with onset in middle-age characterized clinically by Parkinsonian features (e.g., MUSCLE RIGIDITY; HYPOKINESIA; stooped posture) and HYPOTENSION. This condition is considered a clinical variant of MULTIPLE SYSTEM ATROPHY. Pathologic features include a prominent loss of neurons in the zona compacta of the SUBSTANTIA NIGRA and PUTAMEN. (From Adams et al., Principles of Neurology, 6th ed, p1075-6)

Also Known As:

Networked: 86 relevant articles (2 outcomes, 16 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Parkinson Disease (Parkinson's Disease)
2.	Multiple System Atrophy
3.	Parkinsonian Disorders (Parkinsonism)
4.	Olivopontocerebellar Atrophies (Olivopontocerebellar Atrophy)
5.	Progressive Supranuclear Palsy (Steele Richardson Olszewski Syndrome)

Experts

1.	Tison, F: 12 articles (10/2005 - 01/2000)
2.	Wenning, G K: 12 articles (08/2005 - 01/2000)
3.	Poewe, W: 9 articles (08/2005 - 01/2000)
4.	Scherfler, C: 9 articles (08/2005 - 01/2000)
5.	Puschban, Z: 8 articles (08/2005 - 01/2000)
6.	Ghorayeb, I: 7 articles (10/2005 - 01/2000)
7.	Stefanova, Nadia: 6 articles (10/2015 - 08/2003)
8.	Poewe, Werner: 6 articles (10/2015 - 08/2003)
9.	Wenning, Gregor K: 6 articles (10/2015 - 08/2003)
10.	Fernagut, P O: 6 articles (10/2005 - 05/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Striatonigral Degeneration:

1.	Levodopa (L Dopa)FDA LinkGeneric 05/01/2001 - "The chief discussant arrived at the conclusion that the patient had striatonigral degeneration because of poor response to levodopa in the later course, autonomic failures, and MRI changes. " 10/14/1972 - "Levodopa therapy in striatonigral degeneration." 09/30/1972 - "Response to levodopa therapy in striatonigral degeneration." 07/08/1972 - "Levodopa therapy in striatonigral degeneration." 07/01/1972 - "Striatonigral degeneration response to levodopa therapy."
2.	Dopamine (Intropin)FDA LinkGeneric 12/01/1996 - "PET imaging of dopamine D1 and D2 receptors may be useful for the differential diagnosis of PD and striatonigral degeneration. " 08/01/1988 - "This case suggests that the hyperthermia of NMS is due to peripheral heat production associated with severe muscular rigidity secondary to withdrawal of striatal dopamine drive, rather than being centrally mediated by the hypothalamus, which is not involved in striatonigral degeneration." 01/03/2000 - "Loss of dopamine uptake sites and dopamine D2 receptors in striatonigral degeneration." 01/01/2001 - "We suggest that dopamine rich embryonic grafts fail to induce functional recovery in a novel 3-nitropropionic acid rat model of advanced striatonigral degeneration (multiple system atrophy)." 08/01/1989 - "In this study, concentrations of homovanillic acid (HVA), a principal metabolite of dopamine (DA), in the cerebrospinal fluid (CSF) were measured in 17 patients with Parkinson's disease (PD) who had been effectively treated with L-DOPA and in 8 patients clinically diagnosed as having striatonigral degeneration (SND) and for whom treatment with L-DOPA had proved ineffective. "
3.	Homovanillic AcidIBA 08/01/1989 - "In this study, concentrations of homovanillic acid (HVA), a principal metabolite of dopamine (DA), in the cerebrospinal fluid (CSF) were measured in 17 patients with Parkinson's disease (PD) who had been effectively treated with L-DOPA and in 8 patients clinically diagnosed as having striatonigral degeneration (SND) and for whom treatment with L-DOPA had proved ineffective. "
4.	CalcineurinIBA 10/01/1996 - "In this study, we examined the topographic involvement of the putaminal projection neurons and their axons in the globus pallidus and substantia nigra, which we visualized by calcineurin immunostaining, in the basal ganglia of patients with striatonigral degeneration (SND). " 01/01/1990 - "An immunohistochemical topographic study was carried out in the putamen from three patients with striatonigral degeneration (SND) using antibody to calcineurin (CaN), a neurochemical marker for the striatal medium-size spinous neurons. " 12/01/1989 - "The numbers of dopaminergic neurons and of calcineurin-immunoreactive fibers were markedly reduced in the lateral portion of the substantia nigra in all patients with striatonigral degeneration. " 01/01/1989 - "Calcineurin immunoreactivity in striatonigral degeneration." 01/01/1989 - "The basal ganglia (including substantia nigra) of two patients with striatonigral degeneration, who had clinical histories of Parkinson's disease, were studied immunohistochemically using a purified antibody to calcineurin (CaN). "
5.	Quinolinic AcidIBA 07/01/2000 - "In the present experiment we studied the ability of embryonic striatal grafts to protect against striatal quinolinic acid (QA)-induced excitotoxicity in a previously established double lesion rat model of striatonigral degeneration (SND), the neuropathological substrate of parkinsonism associated with multiple system atrophy (MSA). " 11/01/2014 - "A rat model of striatonigral degeneration generated by simultaneous injection of 6-hydroxydopamine into the medial forebrain bundle and quinolinic acid into the striatum." 01/01/2001 - "Simultaneous intrastriatal 6-hydroxydopamine and quinolinic acid injection: a model of early-stage striatonigral degeneration." 10/01/2005 - "Their combination with specific striatal toxins, such as quinolinic acid or 3-nitropropionic acid, has led to the development of experimental models replicating the salient pathological and clinical features of multiple system atrophy of the striatonigral degeneration subtype both in rodents and primates. " 01/01/2000 - "Autoradiographic study of striatal dopamine re-uptake sites and dopamine D1 and D2 receptors in a 6-hydroxydopamine and quinolinic acid double-lesion rat model of striatonigral degeneration (multiple system atrophy) and effects of embryonic ventral mesencephalic, striatal or co-grafts."
6.	SilverIBA 04/01/1994 - "In this study a semiquantitative mapping of oligodendroglial cytoplasmic inclusions (GCIs), a feature of oligodendroglial degeneration in multiple system atrophy (MSA), was undertaken by means of a sensitive silver technique in 14 brains and 11 spinal cords of patients with various combinations of striatonigral degeneration, olivopontocerebellar atrophy and autonomic failure. " 02/01/1992 - "In 8 brains of patients with various combinations of striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome, inclusion bodies were demonstrated in the cytoplasm and nucleus of both neuronal and oligodendroglial cells and in neuronal processes by means of silver staining, immunocytochemistry and electron microscopy. " 12/01/1989 - "Glial cytoplasmic inclusions (GCIs) were demonstrated by silver staining, immunocytochemistry and by electron microscopy in the central nervous system (CNS) of 11 patients with various combinations of striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome. " 12/01/1995 - "Neuronal cytoplasmic inclusions (NCIs) were demonstrated by means of silver staining (Gallyas staining) in the central nervous systems of 18 deceased patients with multiple system atrophy (MSA) - 6 with olivopontocerebellar atrophy (OPCA), 6 with striatonigral degeneration (SND) and 6 with Shy-Drager syndrome (SDS). "
7.	Oxidopamine (6 Hydroxydopamine)IBA 11/01/2014 - "A rat model of striatonigral degeneration generated by simultaneous injection of 6-hydroxydopamine into the medial forebrain bundle and quinolinic acid into the striatum." 01/01/2001 - "Simultaneous intrastriatal 6-hydroxydopamine and quinolinic acid injection: a model of early-stage striatonigral degeneration." 01/01/2000 - "Autoradiographic study of striatal dopamine re-uptake sites and dopamine D1 and D2 receptors in a 6-hydroxydopamine and quinolinic acid double-lesion rat model of striatonigral degeneration (multiple system atrophy) and effects of embryonic ventral mesencephalic, striatal or co-grafts."
8.	Substance PIBA 05/01/1992 - "We designed this study to evaluate the expression of substance P (SP) in the striatum and the globus pallidus in normal controls, idiopathic Parkinson's disease (PD), and striatonigral degeneration (SND). " 06/01/1991 - "Using a computer-assisted image analyser, an immunohistochemical quantification method of substance P-like immunoreactivity (SPLI) in laminae I + II of spinal dorsal horn was established and applied to 13 patients with multiple system atrophy (MSA) with no disturbance of pain sensation, including olivo-ponto-cerebellar atrophy and striatonigral degeneration, and 13 neurologically normal controls. "
9.	Glucose (Dextrose)FDA LinkGeneric 05/01/1993 - "Normalized glucose metabolism was significantly reduced in the caudate (p < 0.03) and putamen (p < 0.003) as compared with that in normal and PD control subjects, and discriminated patients with striatonigral degeneration from control subjects (p < 0.002). " 05/01/1993 - "Regional and global metabolic rates for glucose were estimated using 18F-fluorodeoxyglucose and positron emission tomography in 10 patients with a clinical likelihood of striatonigral degeneration (2 men and 8 women; mean age, 61.8 +/- 6.9 years; mean disease duration, 4.7 +/- 2.2 years; mean Hoehn and Yahr score, 3.5 +/- 0.8). " 08/01/1989 - "When compared with values obtained in normal volunteers, mean cerebral glucose metabolism was slightly reduced in subjects with striatonigral degeneration who, in addition, had a marked (20.5%, +/- 3 SD) relative hypometabolism in putaminal and caudate nuclei. " 08/01/1989 - "Decreased glucose utilization in the striatum and frontal lobe in probable striatonigral degeneration." 08/01/1989 - "Nine positron emission tomography studies of regional cerebral glucose metabolism were performed in 7 patients with probable striatonigral degeneration, a disorder characterized by parkinsonian features and absent or poor response to L-dopa. "
10.	Carbon MonoxideIBA 12/01/2010 - "However, CO activity in the STN was significantly increased in MPTP-induced parkinsonism but not in MPTP+3NP-induced striatonigral degeneration. " 01/01/2000 - "Autoradiographic study of striatal dopamine re-uptake sites and dopamine D1 and D2 receptors in a 6-hydroxydopamine and quinolinic acid double-lesion rat model of striatonigral degeneration (multiple system atrophy) and effects of embryonic ventral mesencephalic, striatal or co-grafts." 01/01/2000 - "The influence of embryonic mesencephalic, striatal and mesencephalic/striatal co-grafts on amphetamine- and apomorphine-induced rotation behaviour was assessed in a rat model of multiple system atrophy/striatonigral degeneration type using dopamine D1 ([3H]SCH23390) and D2 ([3H]spiperone) receptor and dopamine re-uptake ([3H]mazindol) autoradiography. "

Therapies and Procedures

1.	Transplantation (Transplant Recipients) 01/01/2000 - "Tests of forelimb akinesia and complex motor behaviour appear to provide a reliable tool that will be helpful for monitoring the effects of interventional strategies such as embryonic neuronal transplantation in the rat model of striatonigral degeneration."
2.	Transplants (Transplant) 01/01/2000 - "Our study confirms that functional improvement may be obtained from embryonic neuronal grafts in a double-lesion rat model of multiple system atrophy/striatonigral degeneration type. " 08/01/2000 - "In vivo magnetic resonance imaging of embryonic neural grafts in a rat model of striatonigral degeneration (multiple system atrophy)." 07/01/2000 - "Failure of neuroprotection by embryonic striatal grafts in a double lesion rat model of striatonigral degeneration (multiple system atrophy)." 01/01/2001 - "No functional effects of embryonic neuronal grafts on motor deficits in a 3-nitropropionic acid rat model of advanced striatonigral degeneration (multiple system atrophy)." 07/01/2000 - "In the present experiment we studied the ability of embryonic striatal grafts to protect against striatal quinolinic acid (QA)-induced excitotoxicity in a previously established double lesion rat model of striatonigral degeneration (SND), the neuropathological substrate of parkinsonism associated with multiple system atrophy (MSA). "
3.	Homologous Transplantation (Allograft) 09/01/1996 - "Reversal of behavioural abnormalities by fetal allografts in a novel rat model of striatonigral degeneration." 01/01/2001 - "We used 3-nitropropionic acid to create a rat model of advanced striatonigral degeneration in order to assess the effects of embryonic allografts upon rotational and complex-motor behavioural abnormalities. "
4.	Pallidotomy 01/01/1997 - "Medial pallidotomy in late-stage Parkinson's disease and striatonigral degeneration."