Ataxia and gait disorders Flashcards
Classification of ataxias: Acute or subacute onset with resolution or episodic course.
Post infectious and infectious cerebellitis; cerebellar hemorrhage or infarction;
drugs (e.g., phenytoin, barbitruates, antineoplastic agents)
multiple sclerosis
hydrocephalus
posterior fossa mass
foramen magnum compression
dominantly inherited episodic ataxias
childhood metabolic disorders (eg disorders of pyruvate or lactate metabolis,
Classification of ataxias: acute or subacute onset with progressive course
Paraneoplastic cerebellar degeneration
alcoholic or nutritional cerebellar degeneration
Classification of ataxias: Chronic onset and progressive course
autosomal dominant spinocerebellar degenerations
autosomal recessive cerebellar degenerative disorders
Infectious (eg, CJD)
Vitamin E deficiency
Hypothyroidism
childhood metabolic disroders.
Alcoholic cerebellar degeneration
The vermis bears the brunt of the damage. Presentation is usually w/ progressive gait and truncal ataxia that evolves over a period of weeks or months.
Post-infectious cerebellitis
Typically affects children 2-7, following varicella or other viral infection. Presents with acute onset of limb and gait ataxia as well as dysarthria. Illness lasts a few weeks and recovery is usually complete.
Paraneoplastic cerebellar degeneration
Typically presents with acute or subacute onset. With truncal, gait and limb ataxia, dysarthria, and disturbances of ocular motility (ocular dymetria, nystagmus). Usually assc’d with an underlying gynecologic or small cell lung cancer and may become manifest prior to the diagnosis of the tumor. May see anti-Yo or anti-Hu antibodies in CSF.
Friedrich’s ataxia
Autosomal recessive disorder characterized by a progressive ataxia that usualyl affects the amrs more than the legs, as well as severe dysarthria. Usually onset is in childhood. Classic findings include loss of reflexes, spasticity and extensor plantar responses, and impaired vibration and position sense.
Inherited episodic ataxia
Characterized by brief episodes of ataxia, vertigo, nausea and vomiting.
Autosomal dominant spinocerebellar degenerations
Dx based on the occurrence of cerebellar ataxia, w/ or w/out add’l neurologic signs, and a family hx consistent w/ autosomal dominant inheritance. Typical presentaiton is the insiduous onset of progressive impairment of gait and dysarthria in early adult life. Caused by trinucleotide expansions.
Miller-Fisher syndrome
A disorder characterized by ataxia, areflexia and opyhalmoplegia. Ataxia is due to proprioceptive loss rather than to cerebellar dysfunction. Most likely mediated by a postinfectious immune response. Usually self-limiting, with a relatively good prognosis for full recovery.
Etiology of abnormal gaits: hemiplegic
Brainstem, cerebral hemisphere– stroke, tumor, trauma.
Etiology of abnormal gaits: paraplegic
Spinal cord– demylination (eg MS), transverse myelitis, compressive myelopathy.
Bihemispheral– diffuse anoxic injury; parkinson’s disease, other parkinsonia syndromes.
Etiology of abnormal gaits: akinetic-rigid
Basal ganglia–parkinson’s disease, other parkinsonia syndromes.
Etiology of abnormal gaits: frontal
Frontal lobes– hydrocephalus, tumor, stroke, neurodegenerative disorder.
Subcortical– binswangers diseae.
Etiology of abnormal gaits: waddling
hip girdle weakness. Muscular dystrophy, spinal muscular atrophy, acquired proximal myopathy.