Hemicrania continua

Abstract

Hemicrania continua (HC) is an indomethacin responsive primary chronic headache disorder which is currently classified as a subtype of trigeminal autonomic cephalalgias (TACs). It is not very uncommon. There are >1000 cases of HC in the literature, and it constitutes 1.7% of total headache in the clinic settings. Misdiagnosis for HC is very common at all clinical settings. A diagnosis of HC is missed even by neurologists and headache specialists. It is characterized by a continuous strictly unilateral headache with superimposed exacerbations. Just like other TACs, exacerbations are associated with cranial autonomic symptoms and restlessness. A large number of patients may have migrainous features (nausea, vomiting, photophobia, and phonophobia) during exacerbations phase. The “key” feature of HC is persistent featureless background headaches. However, patients and physicians may focus only on the exacerbation part. As durations, frequency and associated symptoms of exacerbations are highly variables; it may mimic a large number of primary and secondary headache disorders. Migraine and cluster headache are two most common misdiagnosed conditions. Another specific feature of HC is remarkable repose to indomethacin. A “complete” response to indomethacin is as “sine qua non” for HC. However, a few other medications may also be effective in a subset of HC patients. Various surgical procedures have been tried with mixed results in patients who were intolerant to indomethacin or other drugs.

Herpes Zoster Ophthalmicus Initially Diagnosed As Cluster Headache, Complicated by Delayed Eruption

“…Manifestations typified by persistent cephalalgia and ipsilateral autonomic symptoms, confined unilaterally, necessitate differentiation from hemicrania continua (HC), as delineated in ICHD-3 under code 3.4 [5]. Given that many conditions can simulate HC symptoms, a comprehensive and meticulous diagnostic exclusion procedure is imperative [6]. It is pertinent to note that the diagnosis of HC hinges on a favorable response to indomethacin.…”

Hemicrania continua associated with an unruptured anterior communicating artery aneurysm: first case report

“…Hemicrania continua has been reported in association with a variety of intracranial pathologies especially traumatic brain injury, sinusitis, primary and secondary brain tumours, internal carotid artery dissection, dental, orbital and temporomandibular joint problems [ 3 , 4 ]. Rarer secondary causes of hemicrania continua include carotid cavernous fistula and cerebral vein thrombosis (CVT) [ 5 , 6 ].…”

Continuous hemicrania as the initial manifestation of Ramsay Hunt syndrome: a case report

“…9,13 Some disorders may mimic HC, such as intracranial space-occupying lesions, posttraumatic headache, postcraniotomy headache, post-stroke headache, internal carotid artery (ICA) dissection, or aneurysm, analgesic rebound headache, idiopathic intracranial hypertension, venous malformation, cerebral venous sinus thrombosis, paraneoplastic syndrome, sinus pathologies, dental lesions, and temporomandibular joint diseases. 14 Two other differential diagnoses one can speculate in this clinical scenario are the "New Daily Persistent Headache" (NDPH) 15 and Cervicogenic Headache (CGH).9 NDPH characteristically presents with a sudden onset headache, which starts and remains without remission, but a bilateral headache typifies this condition. Accordingly, the diagnosis of NDPH was made in none of the patients evaluated by Ramón et al 11 and in only 3% by Prakash et al 10 CGH presents as unilateral pain that starts in the neck, and its prevalence was 8.1% among the side-locked headaches.…”