Hemiparkinsonism-hemiatrophy syndrome

Hemiparkinsonism-hemiatrophy syndrome is a rare parkinsonian disorder characterized by unilateral body atrophy and slowly progressive, ipsilateral, hemiparkinsonian signs (bradykinesia, rigidity, and tremor). Patients typically present with unilateral, action-induced dystonia, in upper or lower limbs, that progresses and becomes bilateral or with tremor which occurs predominantly at rest and progresses to hemiparkinsonism. Scoliosis, scapular winging, raised shoulders, brisk reflexes and extensor plantar responses are frequently associated.



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Narrow down the case reports



Total: 5 (papers)

   


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)		 PMID
(PMCID)
1
(45.7%)		 23986429
 Hemiparkinsonism-hemiatrophy syndrome - report on two cases and review of the literature.
Dziadkiewicz A, Bialecka M, Janik P, Slawek J.
Neurol Neurochir Pol. 2013;47(4):387-92.
Parkinsonism Hemiatrophy
Adult Age of Onset Brain Diseases Dystonia Facial Hemiatrophy Functional Laterality Globus Pallidus Homo sapiens Leukoencephalopathy Magnetic Resonance Imaging Male Middle Aged Muscular Atrophy Parkinsonian Disorders Syndrome
1
(45.7%)		 15960180
 [Late onset hemiparkinsonism-hemiatrophy syndrome: a case report].
Fukae J, Mochizuki H, Ohashi R, Mitani K, Kawada J, Mizuno Y.
Rinsho Shinkeigaku. 2005;45(5):390-3.
Parkinsonism Hemiatrophy
DRD2
Differential Diagnosis Facial Hemiatrophy Females Functional Laterality Homo sapiens Magnetic Resonance Imaging Middle Aged Neurodegenerative Disorders Parkinsonian Disorders Tomography, Emission-Computed, Single-Photon
1
(45.7%)		 7565831
 Hemiatrophy, juvenile-onset exertional alternating leg paresis, hypotonia, and hemidystonia and adult-onset hemiparkinsonism: the spectrum of hemiparkinsonism-hemiatrophy syndrome.
Lang AE.
Mov Disord. 1995;10(4):489-95.
Hypertonia Hemiatrophy
Antiparkinson Agents Blood Glucose Contrast Media Deoxyglucose Dominance, Cerebral Dopa Drug Combinations Females Functional Laterality Hemiplegia Homo sapiens Middle Aged Muscular Atrophy Neurologic Examination Physical Exertion Secondary Parkinson Disease Tomography, Emission-Computed
4
(4.0%)		 21361726
 Hemiparkinsonism-hemiatrophy syndrome.
Ayromlou H, Najmi S, Arami MA.
Arch Iran Med. 2011;14(2):152-4.
Parkinsonism
Adult Antiparkinson Agents Benzothiazoles Cerebral Ventricles Dopamine Agonists Drug Combinations Homo sapiens Indans Magnetic Resonance Imaging Male Monoamine Oxidase Inhibitors Secondary Parkinson Disease X-Ray Computed Tomography
4
(4.0%)		 19766249
 Hemibody mirror movements in hemiparkinsonism-hemiatrophy syndrome.
Silvers DS, Menkes DL.
J Neurol Sci. 2009;287(1-2):260-3.
Parkinsonism
Antiparkinson Agents Electromyography Facial Muscles Functional Laterality Homo sapiens Male Middle Aged Movement Disorders Muscle Contraction Muscular Atrophy Parkinsonian Disorders Peripheral Nervous System Diseases Syndrome
        

Phenotype(s) retrieved from Orphanet

    Total: 17

HPO ID Term Frequency
HP:0001260 Dysarthria Frequent (79-30%)
HP:0001269 Hemiparesis Frequent (79-30%)
HP:0001290 Generalized hypotonia Frequent (79-30%)
HP:0001300 Parkinsonism Frequent (79-30%)
HP:0001332 Dystonia Frequent (79-30%)
HP:0001337 Tremor Frequent (79-30%)
HP:0002067 Bradykinesia Frequent (79-30%)
HP:0006801 Hyperactive deep tendon reflexes Frequent (79-30%)
HP:0006956 Dilation of lateral ventricles Frequent (79-30%)
HP:0011331 Hemifacial atrophy Frequent (79-30%)
HP:0012444 Brain atrophy Frequent (79-30%)
HP:0012768 Neonatal asphyxia Frequent (79-30%)
HP:0100556 Hemiatrophy Frequent (79-30%)
HP:0000716 Depressivity Occasional (29-5%)
HP:0002355 Difficulty walking Occasional (29-5%)
HP:0002650 Scoliosis Occasional (29-5%)
HP:0100308 Cerebral cortical hemiatrophy Occasional (29-5%)


Phenotype(s) retrieved from case reports

    Total: 3

HPO ID Term # of case reports
HP:0001300 Parkinsonism 2
HP:0032005 Hemidystonia 1
HP:0100556 Hemiatrophy 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID