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striatal degeneration, autosomal dominant (ADSD) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

striatal degeneration, autosomal dominant

MONDO:0000211

Also known as:ADSDautosomal dominant striatal neurodegeneration

Overview

An adult-onset movement disorder characterized by bradykinesia, dysarthria and muscle rigidity.

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include very common findings: Dysarthria, Muscle stiffness (rigidity), Slowness of movement (bradykinesia), and Dysdiadochokinesis and others; and common findings: Difficulty walking (gait disturbance) and Difficulty swallowing (dysphagia).

Organ System	Phenotype Count	Example Features
Brain and nerves	6	Dysarthria, Difficulty walking (gait disturbance), Difficulty swallowing (dysphagia)

Diagnosis

Biomarker and diagnostic research for striatal degeneration, autosomal dominant has been reported in the published literature.

Prognosis

Phenotype severity distribution: 5 very common features, 2 common features.

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for striatal degeneration, autosomal dominant.

15 publications have been identified in PubMed for striatal degeneration, autosomal dominant. Research spans Review / Meta-Analysis (40%), Basic Science / Preclinical (27%), and Case Report / Case Series (13%).

Research Type	Count	% of Total
Research summaries	6	40%

Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 2:21 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:228169

GARD

Genetic and Rare Diseases Info Center

GARD:17146

Questions for Your Care Team

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striatal degeneration, autosomal dominant

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

striatal degeneration, autosomal dominant

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Subtypes

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications