Hyper Kinetic

List the dif Classification of Movement Disorders and Give examples

Classification of Movement Disorders

• Hypokinetic 

  • Parkinsonism

• Hyperkinetic 

  • Tremor, Dystonia, Tics, Ballismus, Chorea, Myoclonus

• Mixed

  • Drug induced (e.g. tardive dyskinesia), Huntington’s disease, Wilson’s disease

Define:

• Tremor:

• Dystonia:

• Tics:

• Myoclonus:

• Chorea:

• Athetosis:

• Ballismus:

Definitions

• Tremor:

  • Rhythmic, sinusoidal (oscillatory) movement of a body part, caused by regular contractions of reciprocally innervated muscles

• Dystonia:

  • Unnatural spasmodic movement of posture that puts the limb/body part in a twisted posture

• Tics:

  • Involuntary, compulsive, rapid, repetitive, stereotyped movement or vocalization

• Myoclonus:

  • Very rapid, shock-like contractions of a group of muscles, irregular in rhythm and amplitude

• Chorea:

  • Involuntary irregular movements of a forcible, rapid, jerky type 

  • In Latin Chorea means DANCE

• Athetosis:

  • Inability to sustain the fingers and toes, tongue, or any other part of the body in one position

• Ballismus:

  • Uncontrollable, poorly patterned flinging movement of an entire limb

• 
  

[REVIEW] Basal Ganglia Pathways

Draw out the Basal Ganglia Pathway for:

• Normal

• PD

• Hunt.

• Hemiballism

Describe Huntington’s Disease

• Genetics

• Clinical Manifestations

• Prognosis

• Pathology

• DDx

• Treatment

• Genetics:

  • Chr 4 Defect

    • CAG repeat in huntingtin gene

  • Autosomal Dom

• Clinical Manifestations

  • Gradual onset + progression of chorea + dementia/cognitive change

    • Personality changes, impulsiveness, aggressive behavior, depression, and paranoid psychosis

---

• Prognosis:

  • Death in 15-20 years

• Pathology

  • Neuronal loss + inclusions/gliosis of:

    •  striatum (caudate and putamen) 

    • cerebral cortex

---

DDx:

• Medication effect, 

• infectious, 

• immune disorders (SLE, Sydenham chorea), 

• other genetic disorders (Wilson disease), 

• stroke, 

• metabolic disorders (hyperthyroidism), 

• etc.

---

Treatment (symptomatic)

• Chorea may respond to 

  • tetrabenazine, 

  • amantadine, 

  • riluzole

Describe Hemiballismus

• What is it?

• Patho

• Treatment

• What is it?

  • Only one side

    •  single limb or both upper and lower limbs

• Patho:

  • Acute Lesion:

    • contralateral subthalamic nucleus 

    • surrounding structures (putamen, thalamus, parietal cortex

  • Due to ischemia or hemorrhage

  • Can also  be seen with non-ketotic hyperglycemia

• Treatment

  • Dopamine blocker (haloperidol)

  • Dopamine depleter (tetrabenazine)

  • Neurosurgery for refractory, persistent symptoms

Describe Dystonia

• Patho

• Causes

Dystonia

• Patho:

  • Loss of inhibitory function @ 

    • spinal, 

    • Brainstem

    • cortical levels

• Causes:

  • Severe Generalized Dystonia:

    • Heritable diseases

    • Rare

  • Acute generalized dystonia

    • Antipsychotics

    • Metoclopramide

What does Generalized/Focal Dystonia look like?

1. Describe Idiopathic Torsion Dystonia

   • Clinical Features

   • Treatments

2. Describe Focal Dystonia

   • Difference from tics

   • DDx

   • Examples

   • Treatment

Idiopathic Torsion Dystonia

• Clinical Features:

  • Normal birth and developmental history

  • NO other neurologic signs besides dystonic movements or postures

• Treatment: Responds poorly

  • Medications

    • diazepam, 

    • baclofen, 

    • levodopa, 

    • Carbamazepine

  • Surgery

    • Brain stimulator in selected patients

---

Focal Dystonia

• Difference from Tics:

  • Longer duration

  • Exhibits directionality

  • Typically aggravated by voluntary movement

• DDx

  • Idiopathic

  • Form of Tardive Dyskinesia

  • Other neurologic diseases

  • Systemic diseases

• Examples:

  • Blepharospasm

  • Oromandibular dystonia

  • Torticollis

  • Writers cramp

• Treatment

  • Botulinum A toxin injection 

    • blepharospasm, torticollis; writers cramp

Describe the different types of Tremors

Types

• Resting

  • Parkinson disease, Parkinsonism syndromes

• Action

  • Postural: when trying to maintain a posture vs. gravity

  • Kinetic: with voluntary movement

  • Intention: Increases when approaching a target

    • Finger to Nose Test

• Mixed

When taking History, What would you look out for when discussing tremors:

• Medical History

• Meds

• FH

• SH

• ROS

What about for PE:

• Vitals

• Mental Status

• CNs

• Motor

• Gait

• Psychiatric

History

• Medical History

  • Liver disease,

  • Renal insufficiency,

  • Hyperthyroidism

• Medications

  • Beta agonists, 

  • thyroid medication, 

  • steroids,

  • neuroleptics, 

  • metoclopramide, SSRI/TCA, 

• Family History

  • Essential Tremor, Parkinson

• Social History:

  • Substance use, 

  • caffeine, 

  • psychological trauma

• ROS

  • Constipation, loss of smell, lightheadedness (think Parkinsonism)

  • Mood disorder (think Functional)

---

• Vitals: 

  • Orthostasis (PD)

• Mental Status: 

  • Impaired alertness (myoclonus)

• Cranial Nerves: 

  • Anosmia, hypophonia (PD)

• Motor: Rigidity, bradykinesia, type or tremor, etc.

  • Changes in tremor with entrainment/distraction 

    • Functional Tremor

• Gait:

  •  shuffling, asymmetric arm swing (PD)

• Psychiatric: 

  • Mood, insight

Describe Physiologic Tremor

• What is it?

• Exacerbation?

Physiologic Tremor

• What is it?

  • 7- to 12-Hz detectable in everyone w/ electrophysiologic recording

• Symptomatic when exacerbated by:

  • Fatigue

  • Anxiety/excitement

  • Medications

  • Caffeine

  • Systemic processes (illness)

Describe Essential Tremor

• STATs

• Forms

• Genetics

• Key Characteristic

• Treatment

Essential Tremor

• STATs

  •  5% of the population > 60 years

• Forms:

  • Postural tremor of hands,

  • head tremor

  • voice tremor

• Genetics:

  • Family history common

  • Autosomal dominant

• Key Characteristic:

  • May improve temporarily with alcohol

NOTE:

• No abnormal findings other than tremor

---

Treatment (symptomatic):

• Beta blockers

  •  (propranolol)

• Anticonvulsants 

  • (primidone, topiramate, gabapentin)

• Benzodiazepines 

  • (clonazepam, alprazolam)

• Botulinum toxin A

• Surgical

  •  (Deep brain stimulator, thalamotomy)

Describe Functional (Psychogenic) Tremor

• Disease Course

• Tremor Characteristics

Functional (Psychogenic) Tremor

• Disease Course:

  • Acute onset

  • Spontaneous remissions

• Tremor characteristics:

  • Usually restricted to a single limb 

    • (often dominant hand)

  • Gross tremor

  • Irregular

  • Diminishes in amplitude or disappear if distracted

    • Observe tremor while patient is doing finger tapping on unaffected side, or while patient calculating serial 7s

Describe Tardive Dyskinesia

• Causes

• Clinical features

• Diagnosis Criteria

• Treatment

Tardive Dyskinesia

Causes:

• Delayed onset after prolonged use of dopamine receptor blocking agents

  • Drug Classes responsible

    • Neuroleptics

      • Seen with older and newer atypical antipsychotics, but less often

    • Anti-emetics

      • Metoclopramide

---

Clinical features

• Hyper (usually) or Hypokinetic

• Orofacial dyskinesia, 

• athetosis, 

• dystonia, 

• chorea, 

• tics, 

• facial grimacing

---

Diagnosis Criteria:

• Presence of dyskinetic or dystonic involuntary movements

• History of at least one month of antipsychotic drug treatment

• Exclusion of other causes of abnormal movements

---

Treatment

• Stop the offending drug

  • switch to a second gen antipsychotic if needed 

• Educate: 

  • Often reversible but may take months to a few years

• Pharmacotherapy:

  • Clonazepam

  • Trihexyphenidyl 

    • (Anti-Cholinergic)

  • Dopamine depletors 

    • (tetrabenazine, deutetetrabenzine*, valbenazine*)

  • Botulinum toxin 

    • for severe dystonia

Describe TICs

• Characteristics

• Classifications

  • Primary vs Secondary

Characteristics

• Associated with a premonitory sensation

• Urge to perform the tic

• Can be suppressed

---

Primary or Idiopathic (most common)

• Transient motor or vocal tics 

  • (< 1 year)

• Chronic motor or vocal tics 

  • (>1 year)

• Tourette’s syndrome

  • Motor tic + one (at least) vocal tic

---

Secondary

• Genetic/inherited 

  • (Wilson disease, Huntington disease, neuroacanthosis)

• Developmental disorders

• Drugs 

  • (cocaine, amphetamines)

• Medications 

  • (phenobarbital, phenytoin, methylphenidate, levodopa)

• Carbon monoxide poisoning

• Infections

• Stroke

• Head trauma

Describe Tourette’s Syndrome

• Diagnosis Criteria

• Timing

• Other Symptoms

• Treatment

Tourette’s Syndrome

• Diagnosis Criteria:

  • Multiple motor and phonic tics

• Timing:

  • Onset before age 21 years (usually between 2-15 years)

  • Tics wax and wane

• Other Symptoms:

  • OCD

  • ADD

  • Impulse control probs

  • Other behavioral disturbances

NOTE:

• Absence of other explanatory conditions

---

Treatment:

• Education/Reassurance

  • Behavioral therapy; 

  • address accompanying behavioral disorder

• Medications :

  • First line (symptoms interfere with function):

    • Clonidine, guanfacine, clonazepam

  • Second line (disabling tics):

    • Dopamine blockers: 

      • Risperidone, haloperidol, pimozide, fluphenazine, aripiprazole

  • Botulinum toxin injections

• Deep brain stimulation (clinical trials)

Describe Myoclonus

• Negative vs Positive

• List the Different Classifications and Characteristics

• List the causes of Secondary

Negative vs Positive

• Positive 

  • Active muscle contraction

• Negative

  • Brief inhibition of ongoing muscle activity 

---

Classification

• Physiologic myoclonus (types)

  • Sleep jerks

  • Anxiety-induced

  • Hiccups

  • Exercise-induced

• Essential myoclonus (features):

  • Hereditary or sporadic

  • Accompanied by postural tremor or dystonia

  • Non-progressive history

• Epileptic myoclonus

  • Associated with chronic seizure disorder or syndrome

---

Secondary myoclonus

• Neurodegenerative disorders

• Metabolic disorders 

  • (Wilson’s disease, storage diseases, electrolyte abnormalities, endocrine disorders)

• Infections or post-infectious

• Toxins, medications

• Paraneoplastic syndromes, tumors

• Physical encephalopathies (Anoxia, trauma)

• Psychogenic

Describe Cerebellar Tremor(Ataxia)

• Tremor characteristics

• Accompanying symptoms

• What do you always do? Common Causes

• Tremor Characteristics

  • Unilateral, <5hz

• Accompanying symptoms

  • Cerebellar dysfunction

    • Ataxia

    • Dysmetria

    • Disdiadochokinesia

    • Dysarthria

• ALWAYS GET IMAGING

  • Common Cause

    •  Stroke

    • Multiple sclerosis

    • Cerebellar injury

    • Others: Alcohol, drugs, heritable diseases, mass, infections