Chiari malformation type 1-related headache: the importance of a multidisciplinary study

Abstract

Chiari type 1 Malformation (CM 1) is a structural defect consisting of a displacement of the cerebellar tonsils through the foramen magnum causing obstruction of cerebrospinal fluid (CSF) outflow. CM 1 has a variety of presentation with headache being the most common symptom. The evaluation and treatment of headache related to CM 1 are often difficult, because the pain in the occipital-suboccipital region or headache that is of cough-type suggests symptomatic CM 1, but patients suffering from CM 1 can also report migraine or tension-type headache. In 2015 we started a collaborative project in which our group of neurologists, neurosurgeons and neuroradiologists contribute to create a Chiari Special Outpatient Service; this was set up to provide a multidisciplinary evaluation, treatment and follow-up of patient suffering from CM 1. 201 patients (58 males, 143 females) suffering from CM 1 were multidisciplinary evaluated. Headache characteristics, clinical features, and treatment of patients are discussed. Further progress in multidisciplinary care of headache and CM 1 should be performed to define guidelines.

“Motion-specific Headache”: A Predictor for Diagnosis and Favorable Prognosis after Surgery in Young Patients with Chiari Malformation Type 1

“…9) Although headache is a common symptom and CM1 patients often show other types of headaches such as migraine and tension-type headaches, it is important to distinguish between CM1-specific and other types of headaches because of the possible favorable outcomes of surgical treatment. 10,11) Although this type of headache is typically induced by the difference of pressure gradients, created between the intracranial space and spinal canal by a Valsalva-like maneuver, here we report on six CM1 patients with a unique headache induced by specific movements, which are not Valsalva-like maneuvers. We have termed this headache as "motion-specific headache" and herein provide its characteristics and the surgical treatment followed in these patients.…”

Utilizing group acceptance and commitment therapy (ACT) to address chronic pain, coping, and functioning for patients with Chiari malformation: A case example.

“…medication pain management (Curone et al, 2017). However, this approach also has its disadvantages in the potential risk of medication overuse or dependence.…”

“…CM is a neurological condition in which the cerebellar tonsils (part of the brain) extend below the foramen magnum (the boundary between the brain and spinal cord) by 3–5 mm, or more, and block the flow of cerebrospinal fluid, resulting in headache pain (Curone et al, 2017; Doberstein et al, 2017). Most is known about CM Type I, which is the most prevalent of the four types of CM (Fischbein et al, 2015; the other three types of CM are very rare and have life-threatening symptoms—such as the inability to regulate breathing).…”

“…Specifically, decompression surgery is not always effective and can lead to a number of complications that may even result in the need for an additional surgery (Tisell, Wallskog, & Linde, 2009). Additionally, use of opiates for medication management of pain can result in misuse and dependence while often not sufficiently alleviating pain (Curone et al, 2017). Consequently, it is imperative that other treatment options be explored.…”

Sports and Headaches

“…Symptoms from CM1 usually start in the second or third decade of life. Similar to case 2, in a series of 201 patients with CM1, 13.4% of headaches had onset only during Valsalva maneuver, effort, cough, sneezing, and laughing . Of the entire cohort, the headaches were suboccipital‐occipital in 29%, diffuse/nonpulsating in 74%, and pulsating in 23% and were worsened by Valsalva maneuver in 85%.…”