A Toddler with Recurrent Episodes of Unexplained Irritability: Will you Consider Neuro Imaging?

Abstract

A two-and a half-year-old boy, presented with intermittent episodes of irritability for the past six months. A typical episode was characterized by extreme fussiness and intractable crying, which lasted for only 5 to 10 min. His parents tried to pacify him by feeding, rocking and burping but his irritability did not improve despite all attempts during these episodes. Each episode subsided by its own and occurred both in sleep and awake. He had 8 to 10 episodes/day to one episode/ month. There were no precipitating or relieving factors. He was being treated as colic by multiple practitioners without any improvement in his symptoms. There was no history of unresponsiveness, seizures, abnormal movements, vomiting and recurrent diarrhea. Systemic and neurological examination was unremarkable. As the child had recurrent brief intractable irritability, a possibility of central nervous system pathology was considered. His electroencephalogram was normal, and a cranial magnetic resonance imaging showed Chairi-1 malformation (Fig. 1). Surgical decompression was planned but parents refused for surgical intervention. The cardinal feature of Chairi-1 malformation is caudal displacement of the cerebellar tonsils through the foramen magnum and it effects around around 1–3% of the general population [1]. Nowadays, it is considered as a deformation of normal hindbrain rather than true malformation secondary to hydrodynamic and local mechanical factors. Most of the patients are asymptomatic or they may present with recurrent headache, cyclic vomiting and raised intracranial pressure [2]. A subset of the patients present with dysphagia, dysphonia, visual disturbance, sleep apnea, incoordination and sensory disturbances due to brainstem and cerebellar dysfunction. Headache secondary to Chairi-1 malformation is brief, usually lasting for less than 5–10 min and usually precipitated by Valsalva maneuver or cough [3]. The common causes of recurrent irritability in pre-school children are adjustment disorder, phobic disorder, attentiondeficit/hyperactivity disorders, psychosis [4], and intracranial pathology [5]. The index child had brief duration, intense headache, which was expressed as intractable irritability and sleep disturbance, which made authors think of intracranial pathology which was confirmed with cranial imaging. Early diagnosis of this condition by neuroimaging can avoid unnecessary investigations and over-the-counter anti-colic medications.