Dodick, DW

Hypnic headache syndrome is a rare, sleep-related, benign headache disorder. We report 19 new cases (84% females) with follow-up data. The mean age at headache onset was 60.5 ± 9 years (range 40–73 years). Headache awakened the patients from the night's sleep at a consistent time, usually between 1.00 and 3.00 a.m. (63%); three patients (16%) reported that identical headaches could occur also during daytime naps. Headache frequency was high, occurring more than 4 nights/week in 68% of the patients. Headache resolution occurred within 2 h in 68% of patients. Neurologic examination, laboratory studies, and brain imaging were unrevealing at the time of diagnosis. Headache severity largely remains unchanged or attenuates over time, but frequency may vary in either direction. Only one patient had spontaneous relief from headache. Four patients (24%) achieved permanent suppression of headache with medication, and two were able to abort individual headache attacks. Caffeine in a tablet or beverage was helpful in four patients. Lithium carbonate therapy caused side effects requiring cessation of treatment in four patients.

We describe a 63-year-old smoker who suffered from intractable facial pain secondary to an underlying lung neoplasm. Data from 30 previously reported and similar cases are also summarized. The clinical triad of a smoker suffering from periauricular pain and an elevated ESR should alert the clinician to the possibility of an occult lung mass. In these cases a computed tomography of the chest should always be obtained. Previously refractory pain typically responds to surgical resection of the mass and/or radiation therapy.

The syndrome of orthostatic (low pressure) headaches is well described and most commonly occurs following deliberate violation of the dura (e.g. lumbar puncture). This syndrome can also occur spontaneously and results from the leakage of CSF. We describe three patients who suffered from spontaneous CSF leaks secondary to bony pathology of the cervical spine, and propose that this may be a more common aetiology than originally thought. Often these patients are difficult to manage medically, and surgery may be necessary for symptomatic relief.

We report two cases of SUNCT that demonstrate the medically and surgically refractory nature of this disorder and support the hypothesis that the causative ‘lesion’ lies within the central nervous system. After both patients had failed medical therapies, the first underwent a glycerol rhizotomy, gammaknife radiosurgery and microvascular decompression of the trigeminal nerve. The second patient underwent gammaknife radiosurgery of the trigeminal root exit zone and two microvascular decompression surgeries. Neither patient benefited from these procedures. Currently, the first patient suffers from anaesthesia dolorosa and the second patient from unilateral deafness, chronic vertigo and dysequilibrium as a result of surgical trauma. These cases of SUNCT highlight the uncertainty regarding the role of surgery given the potential for significant morbidity. These cases also suggest that SUNCT originates and may be maintained from within the CNS and this central locus explains why SUNCT is not typically amenable to interventions aimed at the peripheral portion of the trigeminal nerve.

Cluster headache is a stereotypic, primary headache disorder that is marked by repeated short-lasting attacks of severe, unilateral head pain and associated autonomic symptoms. Cluster headache is probably due to an abnormality in the circadian hypothalamic generator with subsequent trigeminovascular activation. We have reviewed the clinical manifestations, pathophysiology, gender differences, and treatment options in cluster headache.

The clinical syndrome of hypertension, headache, palpitation, diaphoresis, flushing, and emotional lability is classically associated with pheochromocytoma. Two patients are presented with this constellation of symptoms in whom investigations for pheochromocytoma were unrevealing. Headache was the presenting and most prominent complaint, with daily episodes of short-lasting, intermittent, and paroxysmal attacks. Each paroxysm of headache was associated with a dramatic increase in systolic and diastolic blood pressure. After failure to control the labile fluctuations in blood pressure and headache with several classes of anti-hypertensive medications used in combination, a prompt and persistent response occurred after the administration of clonidine. The pathophysiology of this syndrome and the mechanism of clonidine action are reviewed in the context of a possible failure of the baroreceptor reflex.