SUNCT syndrome

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By Anna S Cohen PhD, Giorgio Lambru MD, and Manjit S Matharu PhD MRCP

SUNCT syndrome is also known as and serves as an acronym for Short-lasting Unilateral Neuralgiform headache attacks with  C onjunctival injection and Tearing. -ed.

SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) is one of the rarest of all primary headache syndromes. It is one of the trigeminal autonomic cephalalgias, along with cluster headache and paroxysmal hemicrania. It is marked by frequent, short-lasting attacks of 1-sided headache with associated cranial autonomic symptoms. SUNCT forms the majority subset of SUNA (short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms), in which the combination of conjunctival injection and tearing are not required for diagnosis. Historically, this disorder was considered difficult to treat, but recent reports and series have shown some therapeutic benefits with medical treatments. Structural imaging studies report an association with aberrant vessel in contact with the trigeminal nerve, and functional imaging studies report hypothalamic activation. Microvascular decompression, occipital nerve stimulation, and posterior hypothalamic region deep brain stimulation have all been reported to be effective in medically intractable cases.

Key points

  • SUNCT is a relatively rare but very severe form of primary headache syndrome that belongs to a family of trigeminal autonomic cephalalgias.
  • Functional neuroimaging studies in trigeminal autonomic cephalalgias, including SUNCT, report specific activation of the hypothalamus in association with the headaches.
  • A recent structural imaging study reports that a significant proportion of patients have an aberrant vascular loop in contact with the ipsilateral trigeminal nerve, akin to the situation in trigeminal neuralgia.
  • Medical treatment options include lamotrigine, topiramate, gabapentin, and intravenous lidocaine.
  • In patients who are intractable to medical treatments, the surgical options include trigeminal microvascular decompression, occipital nerve stimulation, and posterior hypothalamic region deep brain stimulation.