What is Ataxia?

Ataxia (Greek for “lack of order”) refers to the inability of distinct muscles or groups of muscles to cooperate or coordinate in order to perform a certain task. Perhaps the most prevalent symptom of ataxia is an uncoordinated drunken gait.

The origins of the problem should be investigated. Was it preceded by a rash, drug intake, an infectious sickness, or a sensorium-related neurologic disorder producing, among other things, convulsions?

A certain kind of ataxia (cerebellar or sensory) should be determined by physical examination. Romberg sign (inability to maintain an upright posture while standing on tiptoes with knees bent and eyes closed) is frequently negligible in cerebellar ataxia, but cerebellar symptoms such as nystagmus, dysarthria, hypotonia, and pendular jerks might be affirmative. The child’s motions become sluggish, uncoordinated, and unfinished (adiadokinesia).

The Romberg sign is positive in sensory ataxia owing to a posterior column lesion, although there are other indications of sensory impairments.

Acute Cerebellar Ataxia

Following a viral infection such as chickenpox, poliovirus type 1, influenza A and B, ECHO virus, and coxsackie type B, or as a result of an autoimmune reaction to a range of substances, the illness mainly affects children aged 1 to 5.

The emergence of ataxia has always been acute. A nonspecific infection occurs around 3 weeks before the particular illness in half of the cases. The most prominent component of the clinical presentation is severe ataxia, which leads to fast gait impairment.

Ataxia clears up after a week or so in moderate instances and roughly two months in the vast majority of full-blown cases.

Drug Toxicity

Piperazine, diphenylhydantoin, antihistaminics, chlordiazepoxide, diazepam, colistin, streptomycin, indomethacin, ethanol, vincristine, and meprobamate are examples of drugs that might cause ataxia as a side effect. Even in our nation, solvent sniffing is becoming a major cause of ataxia among schoolchildren and teenagers.

Hypoglycemia

Pallor, vertigo, diaphoresis, tachycardia, tachypnea, convulsions, and coma are some of the symptoms associated with hypoglycemia-induced ataxia.

Acute Labyrinthitis

Abrupt labyrinthitis following mumps is a common but uncommon cause of acute ataxia. Vertigo is usually present in such situations.

Encephalitis

In certain cases, ataxia is a symptom of encephalitis. Sensorium changes (ranging from lethargy to coma), fever, vomiting, convulsions, odd behavior, changed speech, headache, irritability, and feeding difficulties are some of the concomitant signs. Some meningeal symptoms can be caused by a meningeal inflammatory response. Hour-to-hour variations in the clinical manifestation are visible. Except for the fact that it flows at high pressure, CSF is generally normal.

Brain Tumors

Ataxia is a symptom of brain tumors that appears gradually. Vomiting, headaches, diplopia, personality changes, speech difficulties, impatience, and intellectual deterioration are all possible side effects. Common manifestations include papilledema and hydrocephalus.

Astrocytoma is cancer that affects children between the ages of three and eight. Unilateral cerebellar symptoms are the hallmarks of this condition. In addition to ataxia, the youngster may exhibit nystagmus, hypotonia, flexia, and head tilting to the side of the injury.

Medulloblastoma mainly affects male child between the ages of three and five. In most cases, ataxia is severe.

Differential Diagnosis in Pediatrics

The pontine gloima of the brainstem affects children aged 6 to 8. In addition to ataxia, the kid may have bilateral multiple cranial nerve involvement (typically the 6th and 7th), pyramidal symptoms, and no or very minor indicators of elevated intracranial pressure.

Friedreich Ataxia

A progressive gait deficiency is the first symptom of this spinocerebellar degenerative condition, which generally appears in late childhood or adolescence. Incoordination of the upper limbs develops all too rapidly. Ataxia, positive Babinski’s sign and absent ankle jerks form a typical triad that is more or less pathognomonic.

High arched feet (pes cavus), hammertoes, and scoliosis are some of the skeletal malformations seen with this disorder. Cardiomegaly, arrhythmias, and congestive heart failure are all examples of CVS abnormalities.

Ataxia-Telangiectasia

Another spinocerebellar degenerative condition that manifests in childhood, this one commences with ataxia and delayed walking. Other symptoms develop later, such as increasing dysarthria, intention tremors, nystagmus, and chorioathetosis. It is normal to have involuntary eye movements, but control over voluntary motions is lost. Tendon reflexes are slow or non-existent. By the age of five, telangiectasia has developed across the bulbar conjunctiva and skin (ears, nasolabial folds, flexor creases of the limbs). Immune deficits of varying severity are common. The patient may acquire dementia and cancer by the time he or she reaches adolescence.

Refsum’s Syndrome

Progressive cerebellar ataxia, ichthyosis, retinitis pigmentosa, polyneuritis, and deafness are all symptoms of this inborn metabolic defect, which generally appears in late childhood or adolescence (Fig. 5.1).

Abetalipoproteinemia (Acanthocytosis, Bassen-Kornzweiasyndrome)

Malabsorption (which appears in infancy), ataxia (which appears later in childhood), retinal degeneration (which appears in adolescence), and red cells with numerous spiny projections are all characteristics of this illness.

Fig. 5.1:Refsum syndrome Note ichthyosis in this girl with progressiveataxia (cerebellar). She also suffered from progressive deafness andpolyneuritis. Fundoscopy showed bilateral retinitis pigmentosa.

Remaining Causes of Ataxia

Hypothyroidism, Hartnup disease, maple syrup urine disease, disseminated sclerosis, mumps, migraine, cerebral abscess, Guillain-Barré syndrome, cerebral palsy, hysteria, head injury, and other conditions can induce ataxia.