Hospital Medicine

Movement Disorders

I. Problem/Condition.

A movement disorder is an abnormality of movement, defined as either abnormal involuntary movements (hyperkinetic movement disorders) or disruption/poverty of normal movements (hypokinetic movement disorders) with a CENTRAL nervous system etiology. The key is that neurologists define movement disorders very strictly, as ALS patients or diabetic neuropathy patients will have abnormalities of their movements, but these are neuromuscular disorders because they have a peripheral nervous system cause.

II. Diagnostic Approach.

A. What is the differential diagnosis for this problem?

Two classes: Hyperkinetic movement disorders and hypokinetic movement disorders.

  1. Hyperkinetic movement disorders (too much movement)

    • ◦ Ataxia

      • Can be acquired or hereditary, long differential of causes, see chapter on Ataxias

    • ◦ Chorea

      • Huntington's disease or Tardive dyskinesia

    • ◦ Dystonia

      • Focal (torticollis, blepharospasm, writer's cramp), segmental, or generalized

      • Can have acute or tardive drug-induced dystonia

    • ◦ Myoclonus

      • Many causes, ranging from brain to spinal cord, from acquired to hereditary

      • One of the most common in the hospital setting is post-anoxic myoclonus

    • ◦ Tics

      • Tourette's syndrome

    • ◦ Tremor

      • Primary neurologic causes: Essential tremor or Parkinson's disease

      • Secondary causes: medications, toxicity/poisoning, severe stress

  2. Hypokinetic movement disorders (overall problem is not enough movement)

    • ◦ Bradykinesia/ Parkinsonism

      • Idiopathic Parkinson's Disease

      • Parkinson's Plus Syndromes / Atypical parkinsonism

        • Corticobasal ganglionic degeneration (CBGD)

        • Progressive supranuclear palsy (PSP)

        • Multiple Systems Atrophy (MSA)

        • Dementia with Lewy Bodies (DLB)

      • Drug-induced parkinsonism

B. Describe a diagnostic approach/method to the patient with this problem.

  1. Describe what the dominating abnormal movement looks like. See diagnostic criteria below for full descriptions of each type of movement.

  2. Decide if it is overall a hyperkinetic or hypokinetic problem.

  3. If you decide hyperkinetic, determine which problem it is based on the criteria below.

    1. Once you've isolated which hyperkinetic problem it is, look at the differential above of what are the common causes. The key is determining if it is acquired or not. If it is acquired, especially if newly acquired during the hospitalization, then you need to isolate that cause (illicit drugs, medications, stroke, etc) and address that.

  4. If you decide hypokinetic, then you are looking at a form of parkinsonism. For full details, see that chapter, but briefly:

    1. If you have ANY of these features early (within 3 years of onset of motor symptoms), suspect an atypical variant of Parkinson's disease: prominent instability/falls, hallucinations, freezing, dementia, severe dysautonomia.

    2. Absence of these features plusasymmetric and insidious onset of symptoms suggests idiopathic Parkinson's disease.

    3. New/abrupt onset during the hospitalization suggests vascular or drug-induced parkinsonism.

  5. For all tardive (drug-induced) movement disorders, please note that they:

    1. Are 100% iatrogenic and the #1 most frequent single diagnostic code for which physicians are sued in the United States.

    2. Are caused by potent dopamine-blocking drugs, which are anti-emetics (Metoclopramide, lesser degree promethazine) and antipsychotics (typicals like haloperidol, thorazine but also atypicals like risperidone, olanzapine - the only antipsychotics that never cause tardive dyskinesia are quetiapine and clozapine).

    3. Can occur even after only a few days on the drug (particularly if IV).

    4. Reversal of the abnormal movement can take months after stopping the offending drug.

Diagnostic pearls for the hospitalist:

  • Tremor is the most common movement disorder.

  • The most common overall cause of tremor is essential tremor.

  • The most common cause of tremor in the hospital is drug or stress-induced.

  • Remember that all primary neurologic movement disorders (except very unusual movement disorders like palatal myoclonus) disappear with sleep.

1. Historical information important in the diagnosis of this problem.

  1. Was the movement disorder ever present before the hospitalization?

  2. Is there a task-specific component of the movement disorder (can be seen with certain tremors or dystonias - i.e. with handwriting only or with holding a cup only, etc)?

  3. Was the onset of the movement disorder sudden/abrupt or slowly progressive?

  4. Is there a history of anti-emetic (particularly metoclopramide) or anti-psychotic usage?

  5. Is there a family history of similar abnormal movements?

    1. If yes, strong AD inheritance pattern occurs in: essential tremor, hereditary spinocerebellar ataxias, and familial Parkinson's disease (almost exclusively onset < age 50)

  6. Are there any associated non-motor features?

    1. Vision/hearing problems can be seen with some of the spinocerebellar ataxias

    2. Loss of smell can be seen with Parkinson's disease

    3. REM-sleep behavior disorder can be seen with all forms of parkinsonism

    4. Dysautonomia can be seen with Parkinson's disease or MSA

    5. Constipation is seen in >80% of Parkinson's patients

2. Physical Examination maneuvers that are likely to be useful in diagnosing the cause of this problem.

  • For movement disorders, it is ALL about the exam. Movement disorder neurologists love to talk about thephenomenology,which just means what is the description of the movement problem.

  • If you are thinking of parkinsonism, you must check muscletone (with patient relaxed, move hand up/down to check tone around the wrist and move forearm up/down to check tone around the elbow). Tone is increased in all forms of parkinsonism.

  • Assess fortremor. Tremor isrhythmic and either rest, postural (have patients hold arms out in front of them), or action (do finger to nose task). See tremor chapter for more details, but postural/action tremor is essential tremor, rest tremor is Parkinson's disease. Tremor is frequently absent in atypical parkinsonism (usually more rigidity/bradykinesia).

  • If the movement is NOT rhythmic, then is it a brief rapid jerk (myoclonus) or is it more writhing/irregular (chorea).

  • Performgait exam: unstressed (normal walking), heel, toe, and tandem (tightrope). Parkinson's patients have decreased arm swing on the side where their symptoms are dominant. Gait is often affected in movement disorders but an isolated gait abnormality can have many causes (neurologic or non-neurologic).

  • Limb ataxia is assessed by finger-to-nose and heel-to-shin tasks.

3. Laboratory, radiographic and other tests that are likely to be useful in diagnosing the cause of this problem.

TSH (as hyperthyroidism can cause tremor). Occasionally severe B12 deficiency or severe hyper/hypoglycemia can cause movement disorders, so checking B12/HbA1C could be considered. If movement disorder is acute onset, then should get imaging to look for a structural cause (stroke, tumor, etc), and preferred is magnetic resonance imaging (MRI). If onset is gradual/progressive, then imaging is less useful. Of all neurologists, movement disorder neurologists order the fewest tests as diagnosis is mostly clinical and not reliant on ancillary testing.

C. Criteria for Diagnosing Each Diagnosis in the Method Above.

As described above under physical exam, diagnosing the different types of movement disorders is a PURELY clinical diagnosis based on the phenomenology of the movement, or what the description of the movement is. Each movement disorder below, in turn, has its own differential diagnosis. For those that are seen commonly in the hospital, there may be a separate chapter delineating how to evaluate and treat that specific movement disorder, but many are too obscure to warrant such special treatment. A description of the movement and some simple associated facts for each movement are listed below. All acute onset movement disorders should have an investigation for a precipitating agent (medication, toxin, metabolic, etc).

  1. Hyperkinetic movement disorders (too much movement)

    • ◦ Ataxia

      • ▪Coordination problem: Limb ataxia is dysmetria on finger-to-nose or heel-to-shin. Truncal ataxia is wobbling movement of trunk when sitting/standing. Gait ataxia is wide-based gait, usually with staggering and inability to tandem walk.

      • ▪Usually from disorders of the cerebellum, affects balance/coordination. Extent of cerebellar involvement will determine if ataxia is in limbs, trunk, gait, or combinations of those.

      • ▪Pure gait ataxias are rare, and if you see it, you should make sure that it is not a sensory ataxia (pseudo-ataxia caused by severe sensory/proprioceptive loss in the legs from causes like diabetic neurop.

    • ◦ >horea

      • ▪Irregular dance-like movement, usually small amplitude, can be mistaken for nervousness/fidgeting.

      • ▪Differentiate from simple fidgeting by assessing ability to sustain posture of the part of body involved (ie hold tongue protruded for 5 seconds or hold hands outstretched without twitching for 10 seconds).

      • ▪Two main causes are Huntington's disease (family history) or Tardive dyskinesia (history of antiemetic or antipsychotic use).

      • ▪Remember tardive dyskinesia only means late effect of a drug and NOT where the movement is. It just happens that TD most commonly affects the oro-bucco-lingual area, but technically drugs like metoclopramide can cause chorea in any part of the body.

    • ◦ Dystonia

      • ▪Forced alteration of posture caused by involuntary (centrally mediated) muscle contraction.

      • ▪Though not meriting its own chapter here, this is one of the most common movement disorders seen in the hospital due to an acute side effect of dopamine blocking drugs, akaacute dystonic reaction.

      • ▪Focal (torticollis, blepharospasm, writer's cramp), segmental (contiguous body parts affected like head and neck), or generalized.

    • ◦ Myoclonus

      • ▪Lightning-fast jerk/twitch is positive myoclonus; fast loss of tone is negative myoclonus (asterixis).

      • ▪In inpatient setting, can see myoclonus from any severe metabolic disturbance or post-hypoxic/post-code (which incidentally is one of the strongest predictors for poor prognosis of neurologic recovery after a code event) and is commonly mistaken for seizure activity.

      • ▪Many causes, ranging from brain to spinal cord, from acquired to hereditary.

    • ◦ Tics

      • ▪Motor, vocal, or both (Tourette's is defined as motorand vocal tics for at least one year).

      • ▪Can be simple or complex, tend to be stereotyped.

      • ▪Tend to come in clusters, as patient can suppress them for a time then will come out in a burst.

    • ◦ Tremor

      • Rhythmic, oscillatory movement.

      • ▪Is either rest, postural, or action depending on what brings out the tremor.

      • ▪Drug-induced tremors are almost exclusively postural/action enhanced physiologic tremors (high frequency but low amplitude).

      • ▪Stress makes all forms of tremor worse .

  2. Hypokinetic movement disorders

    • ◦ Bradykinesia/ Parkinsonism

      • ▪Idiopathic Parkinson's Disease - see chapter for full details, but briefly, need 2 of 4 cardinal symptoms (rest tremor, rigidity, bradykinesia, gait imbalance). Therefore, rest tremor alone is insufficient, and 30% of Parkinson's patients never develop tremor anyway (why rigidity is the most critical determining factor). Gait imbalance (especially leading to falls) is a late feature. Symptoms almost always asymmetric at onset

      • ▪Parkinson's Plus Syndromes / Atypical parkinsonism - all forms are more rapidly progressive and less responsive to medications that idiopathic Parkinson's disease and almost all lack tremor. Suspect when there is parkinsonism but also has features unusual for idiopathic Parkinson's disease (key one for each bolded below).

        • ▪Corticobasal ganglionic degeneration (CBGD) - bradykinesia, rigidity but alsoprominent apraxia, often to the extent that one limb is completely out of volitional control (alien limb phenomenon).

        • ▪Progressive supranuclear palsy (PSP) - bradykinesia, rigidity butprominent eye movement problems: supranuclear gaze palsy (when patient cannot initiate movements on their own but oculocephalic maneuver shows that ability to make those movements is intact) and/or apraxia of eyelid opening (physically able to but cannot seem to keep eyelids open) which lead to early/frequent falls.

        • ▪Multiple Systems Atrophy (MSA) - bradykinesia, rigidity (usually extreme axial rigidity) butsevere dysautonomia and/or cerebellar dysfunction (ataxia described above), often also has early falls.

        • ▪Dementia with Lewy Bodies (DLB) - key is presence ofearly dementia. By definition, parkinsonism and dementia coincident in onset within one year of each other. This is different from Parkinson's disease with dementia, which is usually motor symptoms for 10 years then developing dementia. Also, frequently has associated visual hallucinations, prominent fluctuations, and neuroleptic sensitivity.

      • ▪Drug-induced parkinsonism - symmetric parkinsonian symptoms at onset, history of commonly offending drugs (metoclopramide, antipsychotics).

D. Over-utilized or “wasted” diagnostic tests associated with the evaluation of this problem.

Computed tomography (CT) is almost never useful. As above, even MRI is not useful if clinically points to progressive disease like Parkinson's.

III. Management while the Diagnostic Process is Proceeding.

A. Management of Clinical Problem Movement Disorders.

Since most causes in the inpatient setting are secondary, treatment should be aimed at removing offending drug or correcting underlying metabolic disturbance (i.e. treating the cause not just the symptom).

Drug-induced parkinsonism and parkinsonism caused by a structural problem (stroke/tumor) are typically not responsive to parkinsonian medications such as carbidopa/levodopa.

Most common inpatient movement disorders:

1. Acute dystonic reaction from an antipsychotic or antiemetic. Treat by stopping the offending medication and give Cogentin (benztropine) 1mg IV or Benadryl (diphenhydramine) 25-50mg IV.

2. Post-hypoxic myoclonus. Notoriously refractory to treatment. Tends to abate on its own as the injured hypoxic cortex either dies altogether or begins to heal. Can try levetiracetam, valproate, or clonazepam.

3. Enhanced physiologic tremor from stress or medications. If medication-induced, can withdraw the medication. Otherwise can just reassure the patient or possibly use an anxiolytic (ie benzo).

Otherwise, if this is a primary degenerative type movement disorder, you may want neurology consultation to guide initiation of symptomatic therapy. We will list some common treatment algorithms for each movement disorder below:

  • Ataxia. There is no available symptomatic treatment for ataxia. PT/OT for adaptive measures is the most helpful.

  • Chorea. Tardive dyskinesia may improve with cessation of dopamine blocking agent, though this often takes months and may not completely resolve. If residual/debilitating despite stopping the agent, can use tetrabenazine titrated to effect (average is 25-75mg/day). Chorea of Huntington's disease is also effectively treated by tetrabenazine. This is unlikely to be used in the hospital as tetrabenazine is not stocked in any commercial/retail/hospital pharmacy (only available direct from manufacturer).

  • Dystonia. If focal, unless you can give a patient Botox in the hospital, then pharmacotherapy primarily starts with Artane (trihexiphenidyl) titrated to 2mg TID.

  • Myoclonus. Try to treat the cause, but as above, symptomatically may respond to levetiracetam, valproate, or clonazepam.

  • Tics. Start with clonidine (dose limited by hypotension), next can try topiramate, next can try antipsychotics like risperidone/haloperidol.

  • Tremor. Postural/action tremor of essential tremor is treated with either primidone (25-250mg HS with slow titration) or propranolol (dose limited by bradycardia). Rest tremor, if part of parkinsonism, treated as below.

  • Parkinsonism. In a younger patient (under 65), start with a dopamine agonist, either ropinirole (slow titration to 4mg TID) or pramipexole (slow titration to 0.5 TID). In older patient or intolerant to dopamine agonist, can try Sinemet (preferred formulation is carbidopa/levodopa 25/100) titrating slowly to one tab TID-QID.

B. Common Pitfalls and Side-Effects of Management of this Clinical Problem.

Remember that Parkinson's patients in the hospital should have their medications continued. Stopping dopaminergic therapy suddenly can lead to a withdrawal that is identical to neuroleptic malignant syndrome, which is a neurologic emergency.

The most common side effect of Sinemet therapy is nausea, which should be treated with extra carbidopa added to each dose. Too rapid titration of dopaminergic therapy (agonists or Sinemet) can cause sedation/confusion, which is aided by slowing titration schedule. Dopamine agonists may cause pedal edema and are known for the rare side effect of causing obsessive-compulsive behaviors (gambling, sexual behavior, eating, etc).

Artane, if used for dystonia, has anticholinergic side effects.

Anticonvulsants used for treatment of myoclonus do not need levels checked as treatment is aimed at abating the movement (if possible) not reaching a certain blood level.

When treating essential tremor, the typical contraindications to propranolol apply (pulmonary dysfunction, diabetes, bradycardia), so if these are present, just use primidone. The only side effect of primidone is sedation, so can go slower if this occurs.

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