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Disease Profile

Pallidopyramidal syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

Adolescent

ICD-10

G20

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Pallido-pyramidal disease

Categories

Congenital and Genetic Diseases; Nervous System Diseases

Summary

The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
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Orpha Number: 171695

Definition
Parkinsonian-pyramidal syndrome is a rare, genetic, neurological disorder characterized by the association of both parkinsonian (i.e. bradykinesia, rigidity and/or rest tremor) and pyramidal (i.e. increased reflexes, extensor plantar reflexes, pyramidal weakness or spasticity) manifestations, which vary according to the underlying associated disease (e.g. neurodegenerative disease, inborn errors of metabolism).

Visit the Orphanet disease page for more resources.

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
100% of people have these symptoms
Parkinsonism
0001300
30%-79% of people have these symptoms
Abnormal autonomic nervous system physiology
0012332
Babinski sign
0003487
Bradykinesia
Slow movements
Slowness of movements

[ more ]

0002067
Dysphagia
Poor swallowing
Swallowing difficulties
Swallowing difficulty

[ more ]

0002015
Dystonia
0001332
Hyperreflexia
Increased reflexes
0001347
Hypomimic face
Dull facial expression
0000338
Intention tremor
0002080
Monotonic speech
0031435
Myoclonus
0001336
Neurogenic bladder
Lack of bladder control due to nervous system injury
0000011
Postural instability
Balance impairment
0002172
Rigidity
Muscle rigidity
0002063
Shuffling gait
Shuffled walk
0002362
Sleep disturbance
Difficulty sleeping
Trouble sleeping

[ more ]

0002360
Slow saccadic eye movements
Slow eye movements
0000514
Spasticity
Involuntary muscle stiffness, contraction, or spasm
0001257
Substantia nigra gliosis
0011960
Talipes equinovarus
Club feet
Club foot
Clubfeet
Clubfoot

[ more ]

0001762
Visual hallucinations
0002367
5%-29% of people have these symptoms
Dementia
Dementia, progressive
Progressive dementia

[ more ]

0000726
Lewy bodies
0100315
Percent of people who have these symptoms is not available through HPO
Abnormality of extrapyramidal motor function
0002071
Autosomal recessive inheritance
0000007
Dysarthria
Difficulty articulating speech
0001260
Lower limb spasticity
0002061
Parkinsonism with favorable response to dopaminergic medication
0002548
Scissor gait
Scissor walk
Scissors gait

[ more ]

0012407
Slow progression
Signs and symptoms worsen slowly with time
0003677
Tremor
0001337
Young adult onset
0011462

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Pallidopyramidal syndrome. Click on the link to view a sample search on this topic.