Hyperkinetic Movement Disorders Practice Flashcards

Comprehensive practice vocabulary flashcards covering hyperkinetic movement disorders, including tremors, chorea, and associated genetic syndromes based on the lecture transcript.

Resting Tremor

A tremor that occurs when a body part is at rest, relaxed, or supported, often characterized by pill-rolling movements of the fingers.

Activation of Contralateral Limb

A distraction maneuver that can exacerbate the amplitude of a resting tremor.

Enhanced Physiological Tremor

A fine, high-frequency ($7\text{--}12\,Hz$) action-postural tremor aggravated by stress, caffeine, or medical illnesses like thyrotoxicosis.

Essential Tremor (ET) Core Criteria

The presence of bilateral arm or forearm tremor, isolated head, chin, voice, or tongue tremor, and the absence of other neurological findings.

Propranolol

A first-line long-acting beta-blocker used for Essential Tremor that reduces the tremor rate by $6\%$, contraindicated in asthma or heart block.

Ventral-Intermediate Nucleus (VIM)

The specific thalamic target for Deep Brain Stimulation (DBS) used in medically refractory cases of disabling tremor.

Sensory Trick (Geste Antagoniste)

A physical maneuver, such as touching the chin or cheek, that improves dystonic posture and reduces dystonic tremor frequency.

Primary Orthostatic Tremor

The fastest tremor ($13\text{--}18\,Hz$), characterized by unsteadiness on standing and localized in weight-bearing muscles like the legs.

Isometric Tremor

A tremor occurring during muscle contraction against a stationary object, such as pushing against a wall or squeezing a hand.

Intentional (Terminal) Kinetic Tremor

A classic cerebellar tremor that worsens as the hand approaches a target during visually guided movement.

Psychological Tremor

A tremor characterized by sudden onset, decrease in amplitude during distraction, and entrainment to the frequency of voluntary movement in another limb.

Holmes's (Rubral) Tremor

A low-frequency ($3\text{--}5\,Hz$) tremor resulting from brainstem or cerebellar lesions, involving resting, postural, and intentional components.

Titubation

Rhythmic to-and-fro oscillations involving the head and neck associated with cerebellar structural or functional pathology.

FXTAS (Fragile X tremor/ataxic syndrome)

A neurodegenerative syndrome in older men involving postural tremor, late-onset ataxia, and $T2/FLAIR$ hyperintensity in the middle cerebellar peduncles.

FMR1 Premutation

A genetic condition characterized by $50\text{--}200$ CGG repeats, associated with FXTAS and premature ovarian failure.

MCP Sign

A highly tested MRI finding for FXTAS showing hyperintensity of the middle cerebellar peduncles.

Chorea

A movement disorder characterized by un-sustained, non-stereotypic, random movements that flow like a dance from one body part to another.

Ballismus

A severe form of chorea involving large-amplitude, violent, and forceful flinging movements, mainly affecting proximal muscles like the shoulder.

Milkmaid's Grip

A sign of motor non-persistence in chorea where the patient cannot maintain a steady handgrip, producing alternating squeezing and releasing.

Flycatcher's Tongue

A manifestation of motor impersistence where a patient repeatedly pulls their protruded tongue back into the mouth.

HTT Gene

The gene located on chromosome $4$ that causes Huntington Disease when it contains an expanded CAG trinucleotide repeat.

Huntington Disease (HD) Classic Triad

The clinical combination of Chorea, Dementia, and Depression.

Westphal Variant

A juvenile form of Huntington Disease characterized by early dementia, parkinsonism, and akinetic-rigid syndrome instead of chorea.

Tetrabenazine

An FDA-approved VMAT2 inhibitor used for Huntington chorea by preventing dopamine storage in presynaptic vesicles.

Dentatorubral-Pallidoluysian Atrophy (DRPLA)

An autosomal dominant disorder featuring chorea, cognitive decline, ataxia, and progressive myoclonus epilepsy.

Sydenham Chorea

An autoimmune chorea associated with Group A-B Hemolytic Streptococcus infection, often seen in children after pharyngitis.

Spooning

A hand posture in Sydenham chorea consisting of intermittent wrist flexion with finger hyperextension.

Hemiballismus Localization

Classically associated with a lesion in the contralateral Subthalamic Nucleus.

Chorea Gravidarum

Chorea mediated by female hormones that can present in the first trimester of pregnancy and resolves spontaneously after delivery.

Symptomatic Palatal Tremor

A rhythmic myoclonus of the soft palate associated with lesions in the Guillain-Mollaret triangle and hypertrophic olivary degeneration.