Clinical AdvisorClinical Challenge: Headache With Transient Drooling

Navigate the complex world of diagnosing Chiari malformation in this insightful review of a clinical case.

To understand Chiari malformation, let’s explore a clinical case. A mid-30s patient with a migraine history came to the ED with a sudden posterior headache. Accompanied by transient total body numbness, loss of coordination, and an inability to swallow with drooling, these symptoms started abruptly after violent sneezing. Although neurological symptoms faded after 20 seconds, the headache persisted.

Regarding vital signs and physical examination, everything was normal. The patient had a head CT ordered. Interpreting the clinical picture and CT images with care is crucial in these cases. Even though a sudden onset headache could signify a ruptured berry aneurysm, no blood was detected on the CT cuts, minimizing that concern. Remember, an LP is seldom recommended if the CT is performed within 6 hours and hemoglobin exceeds 10 g/dL.

However, the clinical presentation in this case, characterized by headache and drooling, didn’t match a typical migraine. Be mindful that many patients might loosely use the term “migraine” for any severe headache. The CT cut revealed a brainstem compressed by the cerebellum, indicative of a Chiari malformation type 1.

Chiari malformation, characterized by an underdeveloped posterior cranial fossa, can cause the cerebellum and brainstem to crowd, possibly leading to compression. It’s important to note that symptoms can vary in severity, from absent to intermittent. Cerebellum compression might result in ataxia or nystagmus, while brainstem compression can lead to lower cranial nerve issues such as dysarthria or dysphagia. These symptoms can be triggered or intensified by certain head motions or Valsalva maneuver equivalents, like straining, coughing, or sneezing.

When you spot multiple symptoms or signs listed above, consider Chiari malformation. The preferred diagnostic tests are non-contrast head CT or brain MRI. If imaging suggests Chiari malformation, seek consultation from diagnostic neurology and/or neurosurgery for appropriate follow-up.

Most Chiari malformations are type 1, developing in adulthood, whereas type 2 is present at birth and associated with spina bifida. Treatment typically involves conservative measures like minimizing triggers and as-needed analgesics. However, for severe cases, skull base surgery can be curative, even though symptoms may reappear. This case demonstrates the need for a deep understanding of Chiari malformation.