movement disorders

• Resting tremor


  Develops while a body part is at rest, is gravity dependent, disappears with voluntary movement
• Action tremor


  Simple is uniform in voluntary movement; intentional is worse as the body part approaches the target, associated with cerebellum, ataxia/dysmetria; task-specific occurs in a task; Postural shows as asterixis (flapping tremor) which indicates hepatic encephalopathy, uremic encephalopathy and CO2 retention in lung disease
• Cerebellar tremor


  Can be postural, simple kinetic or intention and is due to disturbances alone outflow projection from dentate nucleus of cerebellum→ its termination in ventral lateral nucleus of thalamus
• Causes of cerebellar tremor

  MS, midbrain trauma or stroke
• Essential tremor


  Postural tremor of hand, head or voice. Is autosomal dominant with incomplete penetrance
• Essential tremor


  • Unilateral/bilateral postural and kinetic tremors in arms/face/hands. Is worse with stress, hunger or caffeine and alcohol relieves symptoms
• Treatment for essential tremor

  Propranolol, primidone. Refer if refractory to first line pharmaceutical treatment
• Pill rolling tremor


  Patient rolls pointer finger and thumb like they are rolling a pill, occurs in parkinsonism
• Medications used to treat movement disorders

  • Dopamine
  • Haldol
  • Levodopa
  • Metoclopramide
  • Anticholinergic agents
• Parkinson's disease


  Slowly progressing, onset after 50 years old, loss of dopaminergic neurons in substantia nigra
• Etiology of Parkinson's disease

  • Genetic but not on a familial basis, several gene mutations, can be postencephalitic
• Pathophysiology of Parkinson's disease

  • Pars reticulata or pars compacta (pigmented dopaminergic neurons gradually disappear & this depigmented region can be seen on autopsy)
  • Degeneration in nigrostriatal pathway → ↓ projections to cortex → bradykinesia
  • Lewy bodies in substantia nigra, or imbalance between dopamine and acetylcholine, DOPAMINERGIC NEURON LOSS→ less movement/speech/swallow difficulties
• Symptoms of Parkinson's disease

  • TRAP= Tremor (pill rolling, resting tremor), Rigidity (cogwheel rigidity, lead-pipe rigidity), Akinesia (absence of movement, as narrow-based shuffling gait, masked facies), Postural instability (stooped posture, balance problems, increased fall frequency)
  • Dementia, depression, sleep disturbances, loss of smell, seborrheic dermatitis (oily skin rash on scalp/face/chest/axilla
• Parkinson's disease symptoms are asymmetric besides if medication induced
• Treatment for Parkinson's disease


  Motor symptom medications (amantadine, dopamine antagonist, levodopa, deep brain stimulation), manage psychosis with clozapine, physical/speech therapy, brain stimulation/ablative therapy
• Medication-induced parkinsonism


  Caused by medications that block dopamine receptors (haloperidol), maybe from MPTP from opioids
• Huntington's disease


  Autosomal dominant genetic, gene on chromosome 4
• Pathophysiology of Huntington's disease

  • CAG (glutamine) repeat causes an abnormal protein and that creates abnormal movement and cognitive problem
  • CAG makes polyglutamine disease
  • Mutated protein aggregates in neuronal cells of caudate/putamen of basal ganglia causing neuronal death
• Symptoms of Huntington's disease


  • Gradual onset of chorea, dementia or behavioral change, earlier symptom onset for each generation, onset around 40 years old, athetosis, abnormal eye movement, poor coordination, personality changes, depression
  • Early symptoms are abnormal movements or intellectual changes like irritable, moody, antisocial, psychiatric disturbance, fidget, restless
  • Late symptoms are dementia and choreiform movements and dystonic posturing
• Diagnosis of Huntington's disease


  Genetic testing and counseling, CT/MRI showing cerebral atrophy and atrophy of caudate nucleus, PET shows reduced striatal metabolic rate
• Treatment for Huntington's disease

  Neuroleptics (dopamine receptor antagonists) thioridazine, or tetrabenazine (depletes dopamine)
• Death in Huntington's disease is 10-20 years post diagnosis due to aspiration pneumonia or suicide
• Myasthenia gravis

  Autoimmune disorder with variable degree of block of neuromuscular transmission from auto-antibodies binding to acetylcholine receptors and reduce the amount of functioning receptors
• Etiologies of myasthenia gravis


  • Thymic tumor/thyrotoxicosis, rheumatoid arthritis, lupus erythematous, young women with HLA-DR3 gene, older men with thymoma
• Symptoms of myasthenia gravis


  • Fluctuating weakness of voluntary muscles throughout day with symptoms such as diplopia, ptosis, difficulty swallowing, activity increases weakness of affected muscles, short acting anticholinesterases improve weakness
  • Ptosis, diplopia, difficulty in chewing or swallowing, respiratory difficulties, limb weakness, or some combination of these problems
  • Spontaneous relapses/remissions that last for weeks
• Physical exam findings in myasthenia gravis

  • Asymmetric ocular palsies/ptosis, weak with sustained activity, respiratory distress
• Diagnosis of myasthenia gravis

  Antibodies to acetylcholine receptors, serum antibodies to MuSK, low density LRP4, argin, electrophysiologic studies (showing neuromuscular transmission disturbance, CT scan for thymoma
• Treatment for myasthenia gravis


  Short acting anticholinesterases improve weakness, stop exacerbating medications, thymectomy, severe (IVIG, plasmapheresis)
• Bell's palsy


  Sudden onset unilateral facial paralysis due to facial nerve swelling→ compression of CN VII
• Symptoms of Bell's palsy


  • Lose taste on anterior two-thirds of tongue, Eyebrow sagging, Inability to close eye, Disappearance of nasolabial folds, Drooping of mouth, Decreased tearing, Hyperacusis
• Diagnosis of Bell's palsy

  Determine if peripheral or central lesion? Wrinkling remains in forehead→ central. EMG, CT, MRI, imaging to assess brain, temporal bone and parotid gland
• Treatment for Bell's palsy

  Oral corticosteroids (60-80 mg x 7 days), oral antivirals (7-10 days), eye care to prevent corneal injury, give teardrops, tape shut
• Course of Bell's palsy


  • Worst 1-2 days, 3 weeks of symptoms then 2-3 months to improve
  • Recovery risk= severe, >40 years old, diabetes, uncontrolled hypertension
• Cerebral palsy


  Brain disease, genetic or secondary (preterm, CNS injury, hypoxia causing things with birth association, or drowning), damage to brain causing loss of muscle control
• Cerebral palsy does NOT get worse over time
• Diagnosis of cerebral palsy

  CT, MRI, ultrasound
• Treatment for cerebral palsy

  Rehabilitation, occupational therapy, speech therapy, physical therapy, benzodiazepines, spasmolytics, anticonvulsants, pain medications, botox
• Types of cerebral palsy

  • Spastic cerebral palsy= jerky/stiff movements due to lesion in upper motor neuron and impaired GABA receptors, scissor gait/toe walk
  • Dyskinetic/athetoid CP= damage to basal ganglia causing involuntary movement, dystonia, chorea
  • Ataxic CP= damage to cerebellum causing shaky/uncoordinated fine, precise movements, clumsy, unstable movement, trouble picking things up. Pain, insomnia, learning/vision/speaking/communication problems
• Tourette syndrome


  Tic disorder, worst during puberty, increase in anxiety, excitement of exhaustion
• Diagnostic criteria for Tourette syndrome

  • MUST HAVE: multiple motor/vocal tics, persist for 1 year, start before 18 years old
  • Simple tics= short, milliseconds→ eye blink and throat clearing
  • Complex tics= last longer,>1 second and a combo of simple tics, motor-schopraxia, copropraxia, verbal-echolalia, palilalia, coprolalia