Short-lasting unilateral neuralgiform headache attacks with ispilateral facial flushing is a new variant of paroxysmal extreme pain disorder.

BACKGROUND: We encountered a 5-year-old girl who had short-lasting, severe, unilateral temporal headaches with ipsilateral lacrimation, nasal congestion and rhinorrhoea, and facial flushing after severe attacks. Family history revealed similar short-lasting, severe headaches in an older brother, younger sister, mother, maternal aunt, and maternal grandfather's brother. METHODS: We performed routine laboratory examinations and electrophysiological and radiological studies for three children, and whole-exome sequencing to determine the genetic causality in this family. RESULTS: Focal hyperperfusion of the right trigeminal root entry zone was seen during a right-sided attack in one child, while left-sided temporal headache attacks were provoked by bilateral electrical stimulation of the upper extremities in another. We identified a novel SCN9A mutation (NM_002977: c.5218G>C, p.Val1740Leu) in all affected family members, but not in any of the unaffected members. SCN9A encodes the voltage-gated sodium-channel type IX alpha subunit known as Na(v)1.7. CONCLUSIONS: Gain-of-function mutations in Na(v)1.7 are well known to cause paroxysmal extreme pain disorder (PEPD), a painful Na-channelopathy characterized by attacks of excruciating deep burning pain in the rectal, ocular, or jaw areas. The SCN9A mutation suggests that our patients had a phenotype of PEPD with a predominant symptom of short-lasting, severe, unilateral headache.

[Cephalic hypersensitivity syndrome].

The number of patients suffering from chronic headache accompanied by dizziness and cephalic ringing is gradually increasing. Pathophysiology of migraine has been commonly explained by trigeminovascular theory, although recent studies have suggested that the cause of the migraine stems from cortical hyperexcitability. We measured EEG in 1,000 patients suffering from daily headache accompanied by dizziness and cephalic ringing. Here we defined a new syndrome,"cephalic hypersensitivity syndrome" as a subliminal cortical hyperexcitability which itself is invisible but apparently seen as some symptoms such as dizziness and cephalic ringing. The cephalic hypersensitivity syndrome should be treated to attenuate the excitability by an appropriate triptan medication during attacks so as to exhibit its recurrence.

A case of SUNCT syndrome responsive to zonisamide.

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is a rare headache syndrome that represents a subtype of trigeminal autonomic cephalalgia thought to be highly refractory to treatment. More recently, numerous anticonvulsant agents including lamotrigine, topiramate, gabapentin, and carbamazepine have been reported to be partially or completely effective for treating SUNCT. We report the case of a patient with SUNCT in whom symptoms were completely relieved with carbamazepine at 600 mg/day. However, carbamazepine had to be discontinued due to severe rash. Zonisamide was selected for continued treatment, as a Na-channel blocker like carbamazepine but with lower risk of producing skin rashes as caused by carbamazepine. Attacks ceased completely with 300 mg/day of zonisamide achieving a blood serum level of 19 µg/ml. This is the first case report to describe zonisamide alone completely eliminating SUNCT symptoms. Zonisamide should be considered a viable candidate drug for the treatment of SUNCT.

[An overview of the revised international classification of headache disorders (ICHD -II)].

The International Headache Society (IHS) revised The International Classification of Headache published in 1988. Old version has facilitated epidemiological and multinational clinical trials. Fifteen years after its original publication, a revised International Classification of Headache Disorders 2nd Edition(ICHD-II) has been unveiled in 2004. Modifications are small but significant. A lot of knowledge and evidences were added to the new version. A revised classification classifies headaches in 14 groups and categorized headache groups into four parts; part 1: primary headache, part 2: secondary headache, part 3: neuralgias and facial pain, and appendix. Physicians should continue to consult the IHS criteria to ensure accurate diagnosis and management for headache.

[Coexistance of cluster headache and hemicrania continua: a case report].

We reported a 36-year-old man, who suffered from cluster headache (CH) associated with hemicrania continua (HC). The continuous, dull or pressure-type headache appeared on the same side of the CH during the third month of a prolonged cluster period, and fluctuated in the severity of pain. This headache was aggravated when the CH was ameliorated by the administration of lithium carbonate. This converse relationship between CH and HC persisted during an on-off trial of the lithium carbonate, and the HC was exacerbated again after the complete cessation of CH. Retrobulbar pain and nasal congestion were present as components of HC similarly to CH, but they subsided gradually and the pressure-type vascular headache over the temporal area predominated later. The continuous headache lasted more than 3 months, and responded significantly to the indomethacin at a dose of 75mg/d. The clinical course of this patient suggests that HC and CH have a common pathomechanism including hyperactivation of the trigemino-vascular reflex, and may be different in the involvement of other central pathway of pain generation. Indomethacin may deserve consideration for the treatment of continuous headache that appears during an atypical course of other primary headaches.

[Cluster headache and other trigeminal autonomic cephalalgias: diagnosis and treatment].

Cluster headaches are characterized by strictly unilateral paroxysmal attacks of severe pain with associated autonomic sign and symptom. Prevalence is 5 times higher in men than in women in our cases. About 10-15% of patients have chronic symptoms without remissions, but we estimated less frequent in Japanese (6.6% in our series). Pain almost invariably recurs on the same side, but in some patients (16.4%) the affected site switches. Cluster headache may be inherited in about 5% of our cases. Attacks frequently occur at night (60.7%). The patients (64.8%) are restless or agitated during an attack. Recent PET studies elucidated that acute attacks causes activation of the posterior hypothalamic grey matter. The excitement of the area might be responsible for peculiar clinical characteristics of agitation. Some patients (66.0%) have also have symptoms (especially a visual aura) usually attributed to migraine. Treatment of cluster headache includes both acute therapy aimed at aborting individual attacks and prophylactic therapy aimed at preventing recurrent attacks during the cluster period. There are many choices using for both therapies. Based on our clinical experience, we recommended the combination of nasal sumatriptan for acute attacks and verapamil 240 mg/day for prophylaxis.

Postoperative seizures: epidemiology, pathology, and prophylaxis.

The risk of epileptic seizures after craniotomy is extremely important but the incidence of postoperative epilepsy varies greatly, depending on the patient's conditions such as primary diseases, severity of surgical insult, and pre-existing epilepsy. Animal studies suggest that neurosurgical insults lead to seizures by two different mechanisms: One mechanism is mediated by free radical generation and the other by impaired ion balance across the cell membrane caused by ischemia or hypoxia. Conventional antiepileptic agents such as phenytoin, phenobarbital, carbamazepine, and valproic acid are promising for the prevention of early seizures, but the effect in preventing postoperative epilepsy is still controversial. Studies on the prophylactic effect of newer antiepileptic agents in craniotomized patients were very limited. Zonisamide, an antiepileptic agent with antiepileptogenic, free radical scavenging and neuroprotective actions in experimental animals, showed promising effects against postoperative epilepsy in a randomized double blind controlled trial. Prophylactic treatment for craniotomized patients significantly prevented the development of partial seizures during the follow-up period. Most recent studies have not supported the prophylactic use of antiepileptic agents in craniotomized patients, but further studies are required.