Abstract text (incl. figure legends and references)
Question: Neck-tongue syndrome (NTS) is a rare and underrecognized headache disorder, characterized by paroxysms of neck and/or occipital pain brought out by abrupt head-turning and accompanied by ipsilateral tongue symptoms.
Methods: Case-report
Results: A 31-year-old woman, hotel receptionist, with a history of alopecia and active smoking, was referred to the Neurology consultation due to monthly episodes of sharp and shooting headache since age 15, characterized as an explosive headache, intensity 10/10, lasting 3-5 seconds, in the occipital region (right predominance), precipitated by ipsilateral neck rotation. During these paroxysms, she used to have a concomitant posterior "tongue-pulling" movement, followed by numbness of the hemi-tongue ipsilateral to the headache, usually lasting less than 1 minute. Episodes of involuntary tongue movements caused discomfort and anxiety due to their social impact. She had a history of minor trauma, after headache debut; no positive family history. On general examination, she showed signs of ligamentous hyperlaxity; normal neurological examination, with no trigger points. The referred episodes were not possible to induce. The blood cell count, erythrocyte sedimentation rate, and chemistry profile were normal. Rheumatoid factor and antinuclear antibody were negative. Cerebral and cervical MRI, and skull base X-ray, were normal. Her clinical features fulfilled the ICHD-3 criteria for a diagnosis of NTS.
Conclusions: Our case illustrates the diagnostic delay of this underrecognized condition and the possible etiological link with ligamentous laxity leading to transient subluxation of the atlanto-axis joint. There are currently no consensus treatment guidelines; conservative management, including physiotherapy and minor cervical adjustment, is the preferred initial treatment.