Ataxia is a term derived from Greek referring to impairment and lack of ability to coordinate or perform voluntary movements.1 Ataxia can present and be noted in children of all age groups ranging from young infants to adolescents. Both genders, plus all racial and ethnic groups, may be affected. However, since there are many conditions and disorders that may cause ataxia in children, there is great variability in its underlying pathogenesis, risk factors, and presentation.
The cerebellum is the structure that, its dysfunction, is commonly attributed to the presentation of ataxia. On the other hand, disturbance to the structures or function of other sites such as the peripheral nerves, dorsal columns of the spinal cord, brainstem, or frontal cerebral cortex, which provide connections or pathways into and out of the cerebellum, may also play contributing roles in ataxia.2,3
Most children presenting with ataxia typically present with abnormal gait.2 Some parents usually describe their children as clumsy, with poor coordination and balance, and unable to perform motor tasks smoothly4,5; whereas some are reported to walk as if under the influence of alcohol.6 The gait is usually wide-based and staggering, with elevation of the feet and then slapping the soles on the ground.2 The impaired balance and coordination may be worsened by closing both eyes (Romberg sign). Dysfunctions in the sensory and proprioceptive cerebellar inputs from the peripheral nerves and the posterior spinal columns may result in children looking at their feet during ambulation.2,3 Frequent falls and fears of injury may urge parents to seek advice from health care professionals. Lesions affecting the cerebellar vermis may lead into ataxia affecting the trunk with difficulties in maintaining axial posture and balance in an upright position. In addition, titubation (bobbing of the head forward and backward)4 may be noted with vermal lesions. The lesions affecting the cerebellar hemispheres may lead into hypotonia and ataxia affecting the limbs ipsilaterally.4
Associated findings in children presenting with ataxia may also include impaired speech, ocular movements, fine motor skills, and intentional tremors.4 The speech may be described as slow, scanning, with uneven volume and separation of syllables.2,4 Nystagmus and ocular dysmetria, overshooting and an inability to keep the moving eyes towards a targeted point, may be noted.1,6 The smooth and accurate performance of rapid alternating movements (diadochokinesia) and complex motor activities may be impaired.4Tables 7-1 summarizes some of the common and rare presentations of signs and symptoms of ataxia in children.
Table 7–1. Signs and Symptoms Associated with Ataxia |Favorite Table|Download (.pdf)
Table 7–1. Signs and Symptoms Associated with Ataxia
|Common Signs||Common Symptoms||Rare Signs||Rare Symptoms|
|Wide-based gait||Impaired walking||Opsoclonus||Headache|
|Slapping gait||Frequent falls||Myoclonus||Nausea|
|Abnormal speech||Poor balance||Papilledema||Vomiting|
|Hypotonia||Impaired motor coordination||Cranial nerve palsies||Visual impairment|