Neurologic symptoms of mast cell disease

Mast cells are known to closely associate with nerve endings and to be important in neurotransmission.  This can translate into a variety of neurologic symptoms.

In 2011, a retrospective study on the neurologic symptoms of mast cell patients (171 SM patients, 52 CM patients, all adult) was published.  The following is a summary of the results.

Syncope (fainting) is a well-defined complication of mastocytosis, reported here in 14.3% of patients .  In these patients, evaluation revealed that the likelihood of epileptic involvement was likely low.  About 2/3 of patients who had fainting episodes also had loose stool, cramping, nausea, sweating and flushing accompanying the episode.  Prostaglandin D2 and histamine are known to cause low blood pressure and fainting in addition to GI symptoms.  Aspirin is thought to protect against acute vascular collapse and fainting, and sees use in tolerant patients for these purposes.   

16.6% of mastocytosis patients complained of back pain.  In all but one patient, the cause was determined to be multifocal compression fractures throughout the spine, including thoracic region.  Vertebroplasty, a procedure in which special bone cement is applied to the fractured vertebrae, has been suggested for symptom relief of these patients.  One patient was found to have back pain due to dense mast cell infiltration of the vertebrae.  In this patient, radiation therapy provided symptom relief.

35% of patients reported headaches.  Several of these patients met the criteria for migraines.  Mast cells have been implicated repeatedly in migraine pathology, and mastocytosis patients are more likely to suffer from them than the general population.  In response to mast cell degranulation, reactive changes have been noted in trigeminal nerve, the structure responsible for sensation in the face and activities like chewing.  Trigeminal neuralgia has been noted in some patients with mast cell disease.

This paper was also the first to find a link between mastocytosis and multiple sclerosis.  Two adults with ISM developed relapsing remitting MS, and a patient with isolated UP developed primary progressive MS.  Mast cells are known to associate with MS lesions, and mast cell activation can be detected in cerebrospinal fluid of MS patients.  This study found an MS frequency of 1.3% among mastocytosis patients, compared to 0.1% in the general population.

Lastly, an association has been found between overall mast cell burden and susceptibility to experimental autoimmune encephalitis (EAE.)

Reference:

Smith, Jonathan H, Butterfield, Joseph H, Pardanini, Animesh, DeLuca, Gabriele, Cutrer, F Michael.  Neurologic symptoms and diagnosis in adults with mast cell disease.  Clinical Neurology and Neurosurgery 113 (2011) 570-574.