Ataxia differential diagnosis

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TYPE CAUSE PROGRESSION IMAGING FEATURES and OTHER TESTS SYMPTOMS
Acute cerebellitis
  • Primary infectious, postinfectious or postvaccination disorder [1].
  • Epstein-Barr virus, influenza A and B, mumps, varicella-zoster virus, coxsackie virus, rotavirus, echovirus, Mycoplasma pneumoniae and immunization
  • Postinfectious cerebellitis typically occurs between one and six weeks after varicella or measles, but also can follow Epstein-Barr or other viral infections and vaccinations in teenagers and young adults.
From self-limited to fatal, depending on the amount of cerebellar swelling
  • Normal or abnormal brain magnetic resonance imaging (MRI) at onset
  • Bilateral hemispheric cerebellar swelling with cortical and white matter T2 hyperintensities; leptomenigeal enhancement may be present.
  • CSF: Elevated protein and leukocytes, with lymphocytic predominance, normal glucose
  • Blood/CSF: Antibodies anti- HSV, EBV, VZV, mumps, rubella, Lyme disease
  • Trunk and limbs ataxia, fever, abnormal eye movements, dysarthria, headache, nausea, vomiting and decreased level of consciousness
Bacterial infection
  • Mycoplasma pneumoniae, Listeria monocytogenes
  • Usually sudden and progressive
  • Lumbar puncture for examination of the cerebrospinal fluid (CSF) and microbiologic testing
  • Fever
  • instability when walking
  • changes in coordination that primarily affect the trunk or head and not the limbs
  • nodding or other unusual head movements
  • unusual eye movements, such as involuntarily darting from side to side
  • slow or slurred speech
  • changes in mood, behavior, or personality
  • headaches
  • nausea or vomiting
Acquired immunodeficiency syndromes
  • Related to M. pneumonia, Epstein-Barr virus, herpes simplex virus, and toxoplasmosis
Subacute ataxia which progress in months
  • Positive serologic test for HIV
  • Cerebellar atrophy
Chronic alcohol use/Alcoholic cerebellar degeneration
  • Toxic effects on the central and peripheral nervous systems
  • Direct toxic alcoholic effect on the Purkinje cells
Rapid progression (weeks or months)
  • Vermis atrophy
  • Severe ataxia of gait and lower limbs with relatively mild involvement of the upper limbs.
  • Speech and ocular motility are usually preserved
Antibiotic-induced acute ataxia
  • Interaction of polymyxins with neurons has been associated with the occurrence of several neurotoxic events
Weeks after initiation
  • Brain MRI abnormalities
  • Characteristic reversible MRI signal abnormalities in the cerebellar dentate nuclei, dorsal brainstem, or splenium of the corpus callosum
  • Non-specific EEG abnormalities
  • Ataxia may also occur in isolation or combined with dizziness, generalized muscle weakness, partial deafness, visual disturbances, vertigo, confusion, hallucinations, seizures, and neuromuscular blockade
Toxic ingestions
  • Alcohol, benzodiazepines, or other anticonvulsant drugs or exposure to environmental toxins such as mercury or lead
  • Usually sudden and progressive
  • Toxicology testing
  • Elevated plasma levels of substances like lithium, and phenytoin
  • Other imaging unremarkable; Cerebellar atrophy in late stages
  • Additional findings that suggest occult ingestion (eg, depressed consciousness)
Atypical infections
  • Progressive multifocal leukoencephalopathy
  • Caused by reactivation of the JC virus in immunocompromised hosts.
  • Progressive and multifocal
  • Magnetic resonance imaging (MRI) reveals a multifocal process limited to the white matter
  • Presents with subacute neurologic deficits like altered mental status, motor deficits (hemiparesis or monoparesis), limb ataxia, gait ataxia, and visual symptoms such as hemianopia and diplopia
Brain tumors
  • Nonmalignant and malignant tumors of the brain and spinal cord.
  • Usually progressive over weeks to months
  • MRI
  • Biopsy
  • Surgery
  • Symptoms and signs of tumor local invasion,
  • Adjacent structures compression,
  • Raised intracranial pressure
Stroke
  • Ischemic stroke (part of the brain loses blood flow)
  • Hemorrhagic stroke (bleeding occurs within the brain)
  • Risk factors
    • high blood pressure (hypertension),
    • high cholesterol,
    • diabetes, and
    • smoking.
  • Progressive or Sudden
  • CT Scan: look for bleeding or masses in the brain.
  • CT perfusion scan: see how much brain is at risk to check brain blood supply (perfusion).
  • MRI of the brain
Symptoms of ataxia with
  • numbness,
  • weakness,
  • tingling, or
  • vision loss or changes.
  • Confusion,
  • Changes in the level of consciousness,
  • Trouble speaking,
  • Trouble understanding speech, vertigo,
  • Balance problems
Vestibular neuritis
  • Problem in the inner ear or the brain.
  • Inflammation of the vestibular nerve caused by a virus
  • Symptoms may come and go over short periods of time, or last for longer periods of time
  • Hearing examination,
  • Blood tests,
  • Electronystagmo-gram
  • Imaging studies of the head and brain
  • Dizziness or vertigo
  • Falling or a feeling as if you are going to fall
  • Lightheadedness, fainting, or a floating sensation
  • Blurred vision
  • Confusion or disorientation
  • Nausea and vomiting
  • Diarrhea
  • Changes in blood pressure and heart rate
  • Fear
  • Anxiety
  • Panic

References

  1. Marsden JF (2018). "Cerebellar ataxia". Handb Clin Neurol. 159: 261–281. doi:10.1016/B978-0-444-63916-5.00017-3. PMID 30482319.


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