Moyamoya-induced paroxysmal dyskinesia

Pedro Gonzalez-Alegre1, Zakaria Ammache, Patricia H Davis

  • 1Department of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA.

Insights

Moyamoya disease (MMD) can initially present as rare movement disorders, specifically paroxysmal dyskinesias. This suggests MMD should be considered in the differential diagnosis for these conditions.

Area of Science:

  • Neurology
  • Vascular Neurology
  • Movement Disorders

Background:

  • Moyamoya disease (MMD) is a rare cerebrovascular disorder characterized by progressive stenosis of intracranial arteries.
  • While typically presenting with stroke, MMD can manifest with other neurological symptoms.

Observation:

  • This study reports two cases where paroxysmal dyskinesia, resembling paroxysmal kinesigenic dyskinesia (PKD) and paroxysmal non-kinesigenic dyskinesia (PNKD), was the initial symptom of MMD.
  • A review of prior literature found no reported cases of moyamoya-induced chorea mimicking PKD or PNKD.

Findings:

  • The presented cases highlight that MMD can manifest with specific types of paroxysmal dyskinesia as an early, presenting symptom.
  • These involuntary movements may be linked to both hormonal influences and ischemic factors in MMD pathogenesis.

Implications:

  • The findings suggest that MMD should be included in the differential diagnosis for patients presenting with paroxysmal kinesigenic dyskinesia and paroxysmal non-kinesigenic dyskinesia.
  • This broadens the clinical recognition of MMD beyond typical stroke presentations, potentially leading to earlier diagnosis and intervention.

Related Concept Videos

Parkinson's Disease: Treatment01:24

Parkinson's Disease: Treatment

Neurodegenerative disorders, such as Parkinson's Disease (PD), involve the gradual and irreversible destruction of neurons in particular brain areas. These disorders exhibit standard features like proteinopathies, selective vulnerability of some neurons, and an interaction of intrinsic properties, genetics, and environmental influences in neural injury.
Parkinson's Disease is primarily a result of the loss of dopaminergic neurons in the substantia nigra pars compacta. The cornerstone of its...
Alterations in Muscle Tone lll01:11

Alterations in Muscle Tone lll

Rigidity and myotonia are distinct abnormalities of muscle tone that affect resistance and relaxation during movement. Although both involve altered muscle contraction, they arise from different neurological and muscular mechanisms.CharacteristicsRigidity is characterized by uniform resistance to passive movement across the entire range, independent of speed, affecting flexors and extensors equally. It may appear as lead-pipe rigidity (smooth, constant resistance) or cogwheel rigidity...
Drugs Affecting GI Tract Motility: Dopamine Receptor Antagonists01:28

Drugs Affecting GI Tract Motility: Dopamine Receptor Antagonists

Prokinetic agents are specialized medications that stimulate gastrointestinal (GI) motility, promoting food movement through the GI tract. Dopamine, an inhibitory neurotransmitter, plays a significant role in this process, reducing GI motility and indirectly controlling the speed of digestion. Dopamine receptor antagonists, such as metoclopramide and domperidone, offer a unique advantage as prokinetic agents. By blocking the dopamine receptors, these drugs increase GI motility, improving food...
Alterations in Muscle Tone ll01:12

Alterations in Muscle Tone ll

Alterations in muscle tone are common manifestations of neurological disorders and reflect dysfunction within different nervous system regions. Spasticity, paratonia, and dystonia represent distinct forms of hypertonia, each with unique mechanisms, clinical features, and diagnostic importance.CharacteristicsSpasticity happens from upper motor neuron lesions and is characterized by velocity-dependent resistance to passive movement. Clinical features include:Exaggerated deep tendon reflexesClonus...
Depolarizing Blockers: Mechanism of Action01:28

Depolarizing Blockers: Mechanism of Action

Depolarizing blockers act on skeletal muscle fibers' membranes and induce their depolarization. Most depolarizing blockers have two quaternary N+ atoms that bind the nicotinic acetylcholine receptors and cause neuromuscular blockade within minutes.
Succinylcholine is the most commonly used depolarizing blocker. Chemically, it constitutes two molecules of acetylcholine joined together by an acetate methyl group. They act on the receptors in the same way as acetylcholine. Because succinylcholine...
Dysrhythmias IV: Characteristics of Bradyarrhythmias01:18

Dysrhythmias IV: Characteristics of Bradyarrhythmias

Bradyarrhythmias are cardiac rhythm disorders characterized by a slower-than-normal heart rate, typically defined as fewer than 60 beats per minute. Some of which are discussed here:Sinus BradycardiaSinus bradycardia presents a heart rate lower than 60 beats per minute, with a regular rhythm originating from the SA node. The ECG typically shows normal P waves preceding each QRS complex, a normal PR interval (0.12 to 0.20 seconds), and a normal QRS duration (0.06 to 0.10 seconds).First-Degree AV...