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When a Headache Isn’t a Migraine: A Case of Chiari Malformation
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When a Headache Isn’t a Migraine: A Case of Chiari Malformation
Chiari malformation is a rare cause of adult headaches due to tonsillar herniation.
Ms B, a 33-year-old white woman, presents with a complaint of severe daily headaches for the past 3 weeks. The patient has a 15-year history of hypertension and intermittent headache. She describes feeling the headaches in the back of her head (suboccipital region), and the pain radiates forward behind her eyes. She describes the headaches as continuous pressure that causes her to have a “fuzzy” feeling in her head. She also reports chronic neck and shoulder pain, upper and lower back pain, and fatigue.
Additional symptoms include dizziness, numbness and tingling in her hands, and difficulty with visual focusing, concentration, and memory. The headaches are intensified with coughing, laughing, and bending over, and they prevent her from participating in her yoga practice.
The patient reports that the headaches are unresponsive to triptans, nonsteroidal anti-inflammatory drugs, acetaminophen, tramadol, and steroids. Her blood pressure is controlled with oral losartan 50 mg/d. The patient reports taking daily supplements of magnesium, fish oil, and vitamin D.
Examination
Ms B’s vital signs are normal, and her blood pressure is well controlled at 120/72 mm Hg. Musculoskeletal examination reveals a slightly unsteady gait with dysmetria and reduced lateral range of motion of the neck to the left and right with muscle spasm of the cervical spine.
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CHIARI MALFORMATION Chiari malformations develop in utero when the tonsils of the hindbrain extend into the spinal column, crowding the foramen magnum.
Abnormal findings on neurologic examination include a diminished gag reflex with protruding tongue deviation to the right. Hoffman sign is positive bilaterally. Deep tendon reflexes are symmetrically increased 4+ with 2-beat clonus bilaterally. Physical examination is otherwise unremarkable.
Testing and Diagnosis
Considering Ms B’s headache symptoms and her neurologic examination showing signs of spinal cord compression, magnetic resonance imaging (MRI) of the brain and cervical spine are ordered. MRI reveals cerebellar tonsils extending 5 mm below the foramen magnum and a few small nerve root sleeve cysts bilaterally at C6-C7.
Treatment
The patient was referred to a neurosurgeon who confirmed the diagnosis of Chiari malformation type I. Ms B subsequently underwent MRI of the thoracic and lumbar spine, which was negative for syrinx (syringomyelia) or tethered spinal cord. The neurosurgeon recommended that Ms B undergo decompression surgery.
Because of unrelenting symptoms and severely decreased quality of life, Ms B decided to move forward with surgery. Chiari decompression with microdissection was performed approximately 4 months after her initial symptoms began. The surgery included suboccipital craniectomy with duraplasty and C1 laminectomy. During surgery the cerebellar tonsils were found to be completely occluding the fourth ventricle. After resection, an arachnoid veil was discovered overlying the fourth ventricle, which was also resected allowing for normal flow of cerebrospinal fluid (CSF).
Discussion
Chiari malformation is a medical condition characterized by cerebellar tonsillar herniation of ≥5 mm below the level of the foramen magnum and is a known rare cause of adult-onset headaches. 1 Hans Chiari, an Austrian professor and pathologist, first identified hindbrain herniation while performing autopsies in the 1890s. 2,3 He classified his findings into 4 categories based on severity of herniation (Table), with type IV being the most severe. 4 While types II, III, and IV are typically diagnosed in utero or early childhood due to significant neurologic and physical deviations, Chiari malformation type I, the mildest type, is often not diagnosed until symptom development in early adulthood. 4 Some patients go undiagnosed for many years and suffer from headaches and additional symptoms during this time.
Chiari I malformation may cause severe headaches that resemble Valsalva-induced headaches. Chiari I malformation has been increasingly detected, with a prevalence in some studies of 0.1% to 0.5% percent. 5,6 Chiari malformations develop in utero when the tonsils of the hindbrain extend downward out of the skull into the spinal column, crowding the foramen
TABLE. Classifications of Chiari Malformation 4
Chiari Malformation Description
Type I
Type II
Type III
Type IV
• Occurs when the cerebellar tonsils extend into the foramen magnum • Usually first noticed in adolescence or adulthood • Asymptomatic adolescents and adults may develop signs of the disorder later in life
• Symptoms are generally more severe than in type 1 and usually appear during childhood • Both the cerebellum and brain stem tissue protrude into the foramen magnum • Usually accompanied by a myelomeningocele, resulting in partial or complete paralysis of the area below the spinal opening • Term Arnold-Chiari malformation is specific to type II malformations
• Rarest and most serious form • Some of the cerebellum and the brain stem herniate through an abnormal opening in the back of the skull • Appears in infancy and can cause debilitating and life-threatening complications • Infants with type III can have many of the same symptoms as those with type II but can also have additional severe neurologic defects such as mental delays, physical delays, and seizures
• Involves an incomplete or underdeveloped cerebellum (cerebellar hypoplasia) • In this rare form, the cerebellum is located in its normal position but parts of it are missing, and portions of the skull and spinal cord may be visible
