Whereas neurologic problems are common in pediatric practice, pediatric neurologic emergencies are relatively uncommon. It is essential for the pediatrician to be able to identify these emergencies, and the pediatrician’s comfort level in addressing these problems is directly related to practice experience, often post residency. However, only about 20% of pediatricians report being “very comfortable” managing childhood neurologic problems, and many practicing pediatricians seek additional education post residency.1 Given workforce issues within pediatric neurology, the pediatrician may not have immediate access to pediatric neurology consultation.2
This paper will review 3 conditions that can present in pediatric practice and identify an appropriate differential diagnosis and workup for each.
CASE 1: 2-year-old with ataxia
A 2-year-old child presents with a several-day history of low-grade fevers, irritability, jerking of extremities, abnormal eye movements, and an unsteady gait. Exam reveals opsoclonus, myoclonus, and impressive ataxia. Head circumference is 46.0 cm (10%).
Acute ataxia is a relatively uncommon presentation to the general pediatrician. Whereas most children have a benign, self-limited problem, the pediatrician must ensure that life-threatening conditions such as a brain tumor or central nervous system (CNS) infection are not present.
Acute ataxia may be defined as an unsteadiness of walking or other fine motor movement that is less than 72 hours old in a previously well child. Most children with acute ataxia present with a refusal to walk or development of a wide-based, drunk-like gait.3
The differential diagnosis for acute ataxia is wide and includes acute infectious processes, postinfectious inflammatory conditions, drug ingestions, and other toxins, tumors, and trauma (Table 1).
However, a recent study looking at 11 years of referrals to a Division of Neurology at a large, urban pediatric medical center revealed cases of acute ataxia were primarily attributed to 3 diagnoses:4
- Postinfectious cerebellar ataxia
- Drug intoxication
- Opsoclonus myoclonus (ataxia) syndrome (OMS/OMAS)
Acute postinfectious cerebellar ataxia is most common in children aged 1 to 6 years and is thought to be triggered by antecedent viral illnesses. Gait ataxia is universal. Twitching of the trunk and head (titubation), action tremor, and end-gaze nystagmus are also common. If alteration of consciousness or multifocal neurologic abnormalities are present, alternative diagnoses such as acute disseminated encephalomyelitis (ADEM) or brainstem encephalitis should be considered.3 The possible alternative diagnosis of opsoclonus myoclonus (ataxia) syndrome (OMS/OMAS) should always be considered.
Ataxia is common after an ingestion of anticonvulsants, benzodiazepines, alcohol, or antihistamines. Accidental poisoning is common in children aged younger than 6 years and in adolescents for whom it is possibly a sign of substance abuse. Alterations of consciousness such as lethargy, slurred speech, or confusion are also common after an accidental ingestion.3
In OMS/OMAS, children will present with extreme irritability, rapid muscle twitches (myoclonus) that worsen with action, and ataxic gait. The characteristic eye movement problem of opsoclonus (ie, rapid, multidirectional saccades) may occur only intermittently, causing the diagnosis to be missed. This syndrome can be idiopathic or paraneoplastic, a presenting sign of neuroblastoma.5