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Cerebellar syndromes

Last updated: February 18, 2021

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The cerebellum is the region of the brain responsible for controlling stance, gait, and balance, as well as the coordination of complex and goal-directed movements. The acute onset of cerebellar symptoms is considered a medical emergency and is usually due to stroke, hemorrhage, or cerebral edema. Chronic cerebellar syndromes are either acquired (e.g., alcoholism, tumors, paraneoplastic) or genetic. Cerebellar injury is characterized by impaired cerebellar function, resulting in ataxia, imbalance, uncoordinated movements (dysmetria), speech (dysarthria), and oculomotor disorders (nystagmus). Vertigo may also occur if the vestibulocerebellar system is affected. The diagnosis is based on the evaluation of these symptoms and is confirmed by detection of the underlying cause in imaging or laboratory or genetic tests. As treatment of these causes is often not possible, management is focused on supportive measures such as physiotherapy and psychological support groups.

References:[1][2]

The clinical features vary depending on the underlying cause and severity of cerebellar injury. Symptoms manifest ipsilaterally to the lesion site.

  • Cerebellar ataxia
    • Gait ataxia: abnormal wide-based and unsteady gait; irregular, uncoordinated activity of the muscles of pelvic girdle and/or lower limbs
    • Truncal ataxia
      • Inability to sit upright and/or stand without support; most apparent in the sitting position
      • Occurs due to damage to the cerebellar vermis
    • Limb ataxia
      • Uncoordinated movements of the upper and lower extremities
      • Occurs due to damage to the cerebellar hemispheres
  • Dysmetria and tremor (postural, action, intention tremor)
    • Finger-to-nose test: patients with dysmetria are unable to touch the tip of their nose with their index finger ; patients with tremor perform the test with shaking fingers
    • Heel-knee-shin test: inability to slide the heel of one foot down the shin of the opposite leg; the heel will deviate to alternate sides
  • Dysdiadochokinesia
    • Inability to perform rapidly alternating agonistic-antagonistic movements
    • Rapid alternating movement test: the patient is unable to rapidly "screw" in an imaginary light bulb simultaneously with both hands (slow, uncoordinated movements)
  • Rebound phenomenon (Stewart-Holmes sign)
    • The patient bends their arm at the elbow, resisting the examiner's pull on the forearm; sudden release of the arm by the examiner results in an overshooting movement.
    • Indicates impaired coordination between muscular agonists and antagonists
  • Pendular knee jerk
    • Abnormally increased patellar reflex
    • After triggering of the initial reflex, the leg keeps moving multiple times
  • Cerebellar drift: Patients stretch supinated arms out in front of them at shoulder level; the arm ipsilateral to the lesion will pronate and drift upwards. [3]
  • Dysarthria (scanning speech): words are broken down into separate syllables and spoken with varying force
  • Oculomotor dysfunction (including nystagmus)
  • Acute cerebellar hemorrhage
  • Other

The localization of symptoms offers important diagnostic clues. Unilateral abnormalities in ocular movements, ataxia, and posture indicate a cerebellar lesion on the ipsilateral side.

Cerebellar syndromes are primarily a clinical diagnosis, based especially on the evaluation of posture, gait, and movements. Imaging tests and laboratory studies confirm the diagnosis.

  1. Neuroimaging (CT/MRI): indicated to rule out infarction, hemorrhage, tumors, edema
  2. Laboratory testing: complete blood cell count; electrolytes, vitamin B12 levels, vitamin B1 levels
  3. Genetic testing: if other diagnostic tests are negative or inconclusive

References:[1]

Differential diagnoses of ataxia
Cerebellar ataxia Sensory (spinal) ataxia Vestibular ataxia
Clinical features
  • See “Clinical features” above.
Romberg test (tests proprioception and vestibular function)
  • Unable to perform
  • Positive
  • Positive
Unterberger test (tests vestibular and cerebellar function)
  • Positive
  • Negative
  • Positive

The differential diagnoses listed here are not exhaustive.

  1. Gilbert, D. Acute Cerebellar Ataxia in Children. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/acute-cerebellar-ataxia-in-children?search=varicella%20ataxia&topicRef=8284&source=see_link.Last updated: January 10, 2018. Accessed: February 5, 2019.
  2. Todd PK, Shakkottai VG. Overview of Cerebellar Ataxia in Adults. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/overview-of-cerebellar-ataxia-in-adults.Last updated: January 20, 2017. Accessed: February 14, 2017.
  3. Ataxia. http://www.mayoclinic.org/diseases-conditions/ataxia/basics/causes/con-20030428. Updated: March 29, 2014. Accessed: March 2, 2017.
  4. Pronator Drift (Barre’s Sign) : Neurological Examination. http://epomedicine.com/clinical-medicine/pronator-drift-neurological-examination. Updated: June 18, 2018. Accessed: October 31, 2018.