Fatal status migrainosus in Chiari 1 malformation.

BACKGROUND: Headaches are common in Chiari Type 1 malformation (CM-1). The prevalence of migraine headaches in CM-1 is similar to that of the general population. However, when migraine headaches occur with CM-1, they tend to have an earlier age of onset, are more frequent and certainly more severe than when they occur without CM-1 association. The exact role or impact of CM-1 in migraine headaches has not been fully elucidated. CASE DESCRIPTION: We report a fatal case of status migrainosus in 7 years old with CM-1 and review the literature on the possible associations.
CONCLUSION: Migraines occurring in association with CM-1 pose a management challenge and can be potentially fatal especially if associated with autonomic symptoms. The exact pathophysiological interaction between these two conditions when they occur simultaneously needs to be further elucidated. Copyright:

INTRODUCTION

Fatalities directly due to a migrainosus attack are scarce in the literature. Furthermore, few reports have focused on the association of migraine with Chiari Type 1 malformations (CM-1) in adults and even fewer if any in children. Here, we report an unusual fatal case of 7 years old with status migrainosus (SM) and CM-1.[2,3,5,11,13]

CASE DESCRIPTION

A 7-year-old boy presented with a 3-week history of severe intractable headaches characterized by visual aura, photophobia, phonophobia, nausea, and an unusual sensation in the abdomen. Other symptoms reflected autonomic dysfunction, e.g., pallor, diaphoresis, pulse ranging from 29 to 140, variable blood pressure (70–156 systolic/diastolic 45–120 mmHg), episodic pupillary dilatation, constipation, and had two syncopal episodes. His blood counts, infectious markers, and cerebral spinal fluid studies were all normal. A magnetic resonance imaging scan of the brain revealed multiple punctate areas of restricted diffusion in both heads of the caudate and lentiform nuclei of the basal ganglia consistent with microinfarcts [Figure 1]. In addition, there was an 8 mm descent of the cerebellar tonsils below the foramen magnum consistent with the diagnosis of a CM-1. Cerebral angiography and echocardiography were normal. The electrocardiogram revealed only bradycardia. The diagnosis of SM was established, and he was treated with prochlorperazine 0.15 mg/kg intravenous after preloading with normal saline; the headaches resolved within 15 min, and the child was symptom free for almost 48 h. However, subsequently, the autonomic dysfunction recurred accompanied by severe bradycardia and cardiopulmonary arrest, resulting in the patient’s expiration.

Figure 1:

(a-c) From left to right; sagittal T2-weighted magnetic resonance image (MRI) scan showing caudal descent of cerebellar tonsils below the foramen magnum, second image showing axial T2-weighted MRI scan with discrete hyperintense lesions on the head of the caudate lobe and the putamen, third image showing corresponding T2 hypointense areas.

DISCUSSION

According to the new International Classification of Headache Disorders 3rd edition 2018, the 7 years old discussed here had 1.2 migraine with aura headaches secondary to Chiari malformation.[13] This patient exhibited as a SM patient, defined as a debilitating migraine attack lasting more than 72 h.[7,13] Of all migraine sufferers, 23– 73% exhibit autonomic dysfunction which is characterized by; bradycardia, syncope attacks, diaphoresis, constipation, and blood pressure fluctuations.[9] Basal ganglia infarcts as seen in this 7-years-old are not unusual in patients with migraines.[1,4,6] Although sudden death in an adult patient with intractable migraine and CM-1 has been described, few if any of such cases have been reported in the pediatric population.[8,10,14-16]

CONCLUSION

The combination of pediatric CM-1 with migraines can be lethal, notably more so with autonomic dysfunction.[12]