Because of the complexity of the disorder, the symptoms experienced by persons with Chiari malformation can be as varied as the individual him/herself. The initial symptoms may be vague and variable. Subjective symptoms may be slowly progressive, leading to misdiagnoses such as multiple sclerosis, myasthenia gravis, or psychiatric disorders. Often, the symptoms are present for months to several years prior to diagnosis. Most symptoms are related to compression of the lower cranial nerves or brainstem structures. The following are the most common symptoms:
Headache
The headache which is associated with Chiari malformation generally begins in the neck, or the skull base area. The pain is generally described as sharp, throbbing or pulsating. Pain radiates throughout the back of the head, into the frontal areas and often around or behind the eyes. The headache may be brought on by cough, sneeze, bending forward or straining. Duration of the headache can last from minutes to hours. The headache may be associated with nausea, but usually not vomiting.
Dysphagia (dis-fa-jia)
Persons may report difficulty swallowing either liquids or solid foods, or a feeling of "catching" in the throat when attempting to swallow. The progression of this symptom may be rapid, suggesting the possibility of aspiration if undetected. If you have this symptom, your health care provider should be notified as soon as possible.
Pain
Persons may complain of neck or upper extremity (arm) pain. Often, the pain is worse with exertion, fatigue, or lifting. Pain can be described as dull and aching, or shooting and stabbing. Pain on one side is more commonly reported than pain on both sides equally. Along with the pain, some patients experience weakness of the hand or arm, or incoordination of fine movements of the hand.
Hoarseness
Changes in voice character and timbre is a common complaint. Hoarseness is often noted first by the person's friends or family members. Some persons will report inability to modulate the voice for singing or speaking loudly. Slurred speech has been reported, however, this is a less common complaint.
Numbness
Tingling, numbness or pins and needles (proprioceptive disturbances)sensation can also accompany the pain, usually in the same areas as the pain. Again, the numbness is generally on one side only. Numbness can progress over months to years to involve the lower extremities and trunk.
Visual disturbance
Persons may complain of various visual disturbances, from blurred vision to double vision (diplopia). Nystagmus (nis-tag-mus), or bobbing of the eyes is a common finding. This is often described by the person as "bobbing" of the eyes when looking downward or to the side . Difficulty driving or reading may be reported by the person, due to "blind spots" in the eyes. Some persons do not report any visual changes, and the nystagmus is noted on clinical evaluation.
Ataxia (a-tax-ea)
Gait disturbance can be described as unsteadiness, or listing to one side. Persons may report falling, or bumping into walls or doors when walking. Infrequently, patients may present with spasticity (stiffness) of the lower extremities, which results in gait disturbance.
Syncope (sin-co-pe)
Episodes of passing out or loss of consciousness are reported in the literature, however, this is a less common complaint. The episodes are unpredictable and brief, lasting from 30 seconds to 2 minutes, associated with loss of consciousness, and prompt, complete recovery. Drop attacks have been also been reported in the literature, however, less commonly in the adult population. There are generally no symptoms of seizure and no state of confusion after the event. Persons may also complain of occasional vertigo, without loss of consciousness.
Various other symptoms may occur, which may include- Nausea, vomiting, dizziness, dysequilibrium, nosebleeds, fast or slow heart beat, high or low blood pressure, ringing in the ears, hearing loss, depression, shortness of breath, and fatigue.
