Neurosurgical treatment of short-lasting, unilateral, neuralgiform hemicrania with conjunctival injection and tearing.

Trigeminal neuralgia solely involving the upper trigeminal nerve branch is rare. The SUNCT syndrome (short-lasting, unilateral, neuralgiform hemicrania with conjunctival injection and tearing) in which the periorbital pain lasts for 60-120 s, and is accompanied by conjunctival injection and tearing is even less common. Unlike trigeminal neuralgia, SUNCT is usually not relieved by medication. Three patients with SUNCT were treated with retrogasserian glycerol rhizolysis, two of them twice. All five treatments provided complete pain relief and the duration of the effects was 2 to more than 4 years. One of these three patients also had a third treatment with compression of retroganglionic fibres with a Fogarthy balloon, according to Mullan, of the upper trigeminal nerve with excellent results.

Decrease of intracranial pressure and weight with digoxin in obesity.

Fourteen obese patients (body mass index = 34-47 kg/m2; mean = 40 kg/m2) with lumbar cerebrospinal fluid pressure (Pcsf) above 20 cm water in 10 of the 14 patients were treated with digoxin with a serum concentration of at least 1.0 nmol/L (0.8 ng/ml) for 6 months. Pcsf decreased significantly during digoxin medication (p < 0.005). Although there were no diet restrictions, all patients decreased in weight (range: 3-25 kg; mean = 10.6 kg) during the 6 months (p < 0.001). When digoxin medication was stopped in 3 patients, prompt weight increase occurred. Most patients needed progressively increased digoxin doses to attain stabilized serum concentrations at the stipulated level, in 5 patients more than 0.5 mg a day. Five of 13 patients developed diabetes mellitus during the digoxin medication. The larger the dose of digoxin, the greater the risk for diabetes mellitus to occur.

Chronic bilateral headache responding to indomethacin.

Three patients with bilateral chronic tension-type headache (meeting IHS diagnostic criteria) responded with complete control of the headache during the more than 2 years they were treated with indomethacin. The headache recurred within 12 to 26 hours after indomethacin was stopped. Fifty milligrams of intravenous indomethacin resulted in complete relief of headache for 6.5 to 25 hours, similar to results found earlier in patients with hemicrania continua. It is concluded that there may be a subgroup of patients with bilateral chronic headache who respond to indomethacin in the group of patients otherwise diagnosed as having chronic tension-type headache.

MR imaging with gadolinium in patients with and without post-lumbar puncture headache.

PURPOSE: To study frequency and extent of meningeal enhancement in patients with and without post-lumbar puncture headache (PLPH) with cerebral MR with Gd-DTPA. MATERIAL AND METHODS: Ten consecutive patients with PLPH and 9 consecutive patients without PLPH were included in the study. Nine of the PLPH patients were reinvestigated when the PLPH was over and all non-PLPH patients during the week after the lumbar puncture. RESULTS: Eight of the 9 patients with PLPH showed more enhancement of meningeal structures during PLPH than when PLPH had disappeared. The differences were slight in 5, more marked in 2, and pronounced in 1 patient. The 9th patient showed no change at all. In the 9 patients without PLPH, there was mainly slight enhancement before lumbar puncture and no change in enhancement after lumbar puncture as compared with before. CONCLUSION: PLPH is related to increased Gd-DTPA enhancement of the meninges, although the increase in enhancement during PLPH is minor in most cases. Pronounced enhancement of the meninges after lumbar puncture may predict long duration of the PLPH.

Recurrent Tolosa-Hunt syndrome: a report of ten new cases.

Ten patients (6F, 4M) with recurrent Tolosa-Hunt syndrome are reported. Besides ocular motor symptoms, one patient had trigeminal nerve involvement, one had ipsilateral ocular sympathicoplegia with miosis and ptosis, and one tinnitus during an episode of Tolosa-Hunt syndrome, ipsilateral to the pain side. One patient had Bell's palsy, one had a possible Raeder's syndrome, and one had a period of tinnitus between the Tolosa-Hunt syndrome episodes. Three of the 10 patients reported periods of periocular pain without ophthalmoplegia between the Tolosa-Hunt episodes, the pain located ipsilateral to the ophthalmoplegic side in the Tolosa-Hunt episodes. Systemic symptoms associated with Tolosa-Hunt syndrome, e.g., back pain, chronic fatigue, arthralgia, gut problems among others, occurred with the same frequency in these 10 patients as in an earlier report. Seventy per cent of the patients had signs of inflammation in serum during a period of Tolosa-Hunt syndrome. Orbital phlebograms showed pathologic signs in four of the five patients investigated during a Tolosa-Hunt period. One phlebogram was normal in a sixth patient when performed during a period of unilateral periocular pain without ophthalmolegia. Magnetic resonance imaging of the head (with contrast) was only performed in three patients during the Tolosa-Hunt period: one showed signs of inflammation in the middle fossa and two were normal. In one of the patients with normal magnetic resonance imaging, the orbital phlebogram was pathologic. Steroid treatment promptly relieved the pain in all patients.

Is chronic tension-type headache a vascular headache? The relation between chronic tension-type headache and cranial hemodynamics.

Twenty-seven patients with chronic tension-type headache were studied as to end-tidal PCO2, heart rate, mean blood pressure, diameter and blood flow of the common carotid arteries, cranial vascular resistance, and headache intensity at supine rest, after administration of nitroglycerin, and at head down tilt. The results were compared to the results of nitroglycerin and head down tilt provocations in age- and sex-matched controls. During supine rest, no change in chronic tension-type headache occurred. Nitroglycerin and tilting induced significant increase of the headache intensity compared to baseline in patients with chronic tension-type headache (P=0.01 and P<0.05, respectively) in contradistinction to controls who did not develop significant headache. Common carotid artery blood flow changes were similar during nitroglycerin provocations in the two groups, but greater (P<0.05) during head down tilt in patients than in controls. Lumbar cerebrospinal fluid pressure was found to be greater than 20 but less than 26 cm H2O in 45% of the 22 patients studied with chronic tension-type headache. The results indicate that the pain in chronic tension-type headache is related to cranial hemodynamics, presumably to distention of intracranial veins.

Symptoms and diseases and smoking habits in female episodic cluster headache and migraine patients.

Twenty-seven episodic female cluster headache patients were compared to 27 age-matched female migraine patients with regard to occurrence of symptoms and diseases other than headache, and also with regard to tobacco consumption. Some symptoms and diseases were found to occur significantly or almost significantly more often in the cluster headache patients than in the migraine patients; Chronic fatigue (p < 0.01), vertigo (p < 0.05), arthralgia (p < 0.05), back pain (p = 0.05), spontaneous ecchymoses (p = 0.05) and constipation and/or periodic diarrhea (p = 0.09). There were significantly fewer persons who had never smoked in the cluster headache group than in the migraine group (p < 0.01). The extent of smoking was significantly greater in the cluster headache group than in the migraine group, both as to the number of cigarettes smoked per day (p < 0.001) and as to smoking years (p < 0.001).

Coping style and social support in women suffering from cluster headache or migraine.

BACKGROUND: Many clinical neurologists have considered cluster headache patients to differ from migraine patients as to behavioral patterns. There is, however, little empirical validation of such a differentiation. METHODS: Coping profiles and social networks were studied in patients suffering from two kinds of recurrent headache. Twenty-four female patients with cluster headache, aged 23-72 years, and 24 age-matched migraine patients with and without aura participated in the study. All female cluster patients treated at the neurologic clinic of the hospital were included, and consecutive outpatients, who had been referred to the policlinics for diagnosis and treatment, whose symptoms agreed with the IHS criteria for migraine and who had ages matching the cluster headache patients, participated in the study. RESULTS: In the semiprojective coping tests the cluster headache patients were found to be statistically significant more 'positive' as to their anticipated activities in the future compared to the migraine patients (p < 0.04). No other statistical differences were found between the two groups. Compared to randomly selected and age-matched referents in the population. cluster headache patients reported significantly poorer social support (p < 0.01), while no other difference was found when the migraine patients were compared with controls. CONCLUSIONS: The findings indicate that there are differences in perception of anticipated activities and social support between patients with cluster headache and migraine.

Postlumbar puncture headache and its relation to chronic tension-type headache.

One hundred consecutive patients, the majority suffering from bilateral chronic tension-type headache, investigated with lumbar puncture, were studied as to age, sex, body mass index, diagnosis, lumbar cerebrospinal fluid pressure, and signs of inflammation in the serum in relation to postlumbar puncture headache. Patients younger than 40 years of age were significantly more prone to develop postlumbar puncture headache than patients older than 40 years of age (P = 0.01). Sex, body mass index, cerebrospinal fluid pressure, and signs of inflammation in the serum were not related to the frequency of postlumbar puncture headache in the present study. Postlumbar puncture headache occurred significantly more often in patients with bilateral chronic tension-type headache than in patients with unilateral headache (P = 0.02) and in patients without headache (P < 0.01). In a regression analysis with age, sex, and chronic tension-type headaches, only bilateral headache contributed significantly to the prediction of postlumbar puncture headache (P < 0.01). Age did not contribute apart from the common variance with chronic tension-type headache/no chronic tension-type headache. The results may indicate that postlumbar puncture headache and chronic tension-type headache have etiologic mechanisms in common, mechanisms presumably localized intracranially rather than extracranially.

A proposed model of cerebrospinal fluid circulation: observations with radionuclide cisternography.

PURPOSE: To determine the mechanisms of the tracer distribution at radionuclide cisternography (RC). METHODS: Ten patients with venous vasculitis were studied with RC. Flow phantom studies were performed mimicking cerebrospinal fluid (CSF) circulation with and without a main outlet comparable to the pacchionian granulations. RESULTS: Nine of the 10 patients had normal findings on RC images, including a maximum uptake over the vertex at 24 hours. In all patients, a second maximum occurred in the lumbosacral area. The flow phantom studies showed no tracer accumulation at an open outlet corresponding to the pacchionian granulations. On the contrary, a maximum arose without such an outlet. A maximum always arose at the closed dead ends of the phantom, including the lumbosacral area. CONCLUSION: The commonly accepted flow model for CSF circulation needs to be revised. The pattern of the normal RC cannot be explained by a bulk flow transport of the tracer to an outlet at the pacchionian granulations but rather by a primary mixing caused by pulsatile flow with a secondary dilution by newly formed CSF from the ventricular system. We suggest that the main absorption of the CSF is through the central nervous system to the blood.

Chronic cluster headache: provocation with carbon dioxide breathing and nitroglycerin.

Nine patients with chronic cluster headache were studied as to end-tidal PCO2, heart rate, blood pressure, common carotid artery blood flow, vascular resistance, and intensity and duration of pain before, during, and after breathing 6% CO2 in air for 6 minutes and before and after administration of 1 mg nitroglycerin sublingually. End-tidal PCO2 was low at rest without provocation indicating that chronic cluster headache patients hyperventilate. Carbon dioxide provocation induced an increase in common carotid artery blood flow. This provocation, previously shown to induce pain in episodic cluster headache patients, did not result in unilateral pain in chronic cluster headache patients. Nitroglycerin did not provoke any pain in 4 of 5 chronic cluster headache patients in contrast to the effects in episodic cluster headache patients in a cluster period. In one chronic cluster headache patient, a short-lasting attack of moderate pain intensity was provoked. The results agree with the hypothesis that chronic cluster headache patients have changed vascular reactivity due to permanent sympathicoplegia unilaterally in the middle fossa in contrast to episodic cluster headache patients who it has been suggested have a nonpermanent sympathicoplegia unilaterally in the same region.

Effects of increasing the intracranial blood volume in cluster headache patients and controls.

Eleven patients with episodic cluster headache in period, five patients out of period and six controls were studied concerning the effects of an increase of the intracranial blood volume by tilting. Common carotid artery (CCA) blood flow was similar in all three groups at baseline and during tilting. CCA diameters were similar at baseline and increased during tilting in all three groups, indicating that tilting caused an increase in the extra- and intracranial blood volume. Unilateral pain or sympathetic dysfunction did not appear during tilting in the patients out of period or in the controls. In four of eight studied patients with cluster headache in period, unilateral miosis and ptosis appeared during tilting. Two of these four patients developed intense unilateral pain, while the other two did not report any pain. Four other patients developed slight unilateral pain but no sympathetic dysfunction during tilting.

Is there a relationship between obesity and intracranial hypertension?

BACKGROUND: Intracranial hypertension and obesity have been reported in recent studies of patients with periorbital venous vasculitis. These findings indicate that obese patients should be investigated for signs of inflammation in serum and lumbar cerebrospinal fluid (CSF) pressure. PATIENTS AND METHODS: Twenty obese females, aged 27-68 years participated in the study of associated symptoms, signs of inflammation in serum, intracranial hypertension and magnetic resonance imaging of the brain (MR). Twenty randomly selected age- and sex-matched females were also investigated for associated symptoms and MR as controls. RESULTS: There were no statistically significant differences in associated symptoms and diseases except for infertility (P < 0.05) between the two groups. The values for orosomucoid, haptoglobin, IgG, IgM and tests for rheumatic and antinuclear factors were significantly increased in the obese group compared with normal values at the hospital. The lumbar CSF pressure was increased above 20 cm water in 79% and above 25 cm water in 42% in the obese patients. MR showed that the subarachnoidal space in the obese patients were significantly smaller than in the controls. CONCLUSIONS: Signs of inflammation in serum, intracranial hypertension and decreased subarachnoidal space were statistically significantly more common in patients with obesity, than in controls.

Provocation of unilateral pain in cluster headache patients by breathing CO2.

Ten patients with cluster headache in an active period and 6 controls were studied as to heart rate, blood pressure, blood flow in the common carotid arteries (CCA), end-tidal PCO2 and pain before, during and after 6 minutes of breathing 6% CO2 in air. Heart rate increased significantly during CO2 breathing in controls but not in patients. The cluster headache patients had significantly lower baseline end-tidal PCO2 than controls. CCA blood flow increased significantly during CO2 breathing in both groups. Vascular resistance decreased during CO2 provocation and increased above baseline levels 5 minutes after provocation in both groups and related to the end-tidal PCO2. Six of eight cluster headache patients, who had an increase of blood flow at provocation, reported slight to moderate unilateral pain in relation to the CO2 provocation in contrast to controls. One patient treated with 6 mg sumatriptan 2.5 hours before the provocation had an end-tidal PCO2 within the range of the controls, and did not get an increase of CCA blood flow or pain at provocation. Six of the cluster headache patients were restudied when out of the active period. There was still no heart rate increase during CO2 breathing and end-tidal PCO2 was still lower than in the controls. Unilateral headache was not provoked.

MR imaging of cerebrospinal fluid dynamics in health and disease. On the vascular pathogenesis of communicating hydrocephalus and benign intracranial hypertension.

The CSF flows in the aqueduct and at the foramen magnum were examined in 5 patients with communicating hydrocephalus (HC) and in 10 with benign intracranial hypertension (BIH) as well as in 5 healthy volunteers. As compared to normal individuals, the aqueductal flow in HC was about 10 times larger and the cervical flow was half as large. In BIH the CSF flows were not different from those of normal volunteers. The decreased arterial expansion as reflected in the reduced cervical flow in HC may be due to pathologic changes in the arteries and paravascular spaces. The large aqueductal flow in HC reflects a large brain expansion, causing increased transcerebral mantle pressure gradient and ventricular dilatation. In BIH there is a normal brain expansion (aqueductal flow) and consequently no ventricular dilatation. It is argued that BIH be caused by an obstruction on the venous side, as opposed to the vascular alterations in HC, which are on the arterial side.

Periorbital venous vasculitis, intracranial hypertension and empty sella.

Sixteen patients with chronic periorbital venous vasculitis without nerve involvement and 9 patients with active episodic cluster headache were studied as to cerebrospinal fluid (CSF) pressure. Eighty-one percent of the patients with chronic and 33% with episodic symptoms had pathologically increased CSF pressure. Magnetic resonance imaging of the brains in the chronic group showed empty sella in 60%, cerebral atrophy in 21% and white matter lesions with high signal intensity on T2 weighted sequences in 29%. Abnormal obesity was found in 31% of the patients with chronic periorbital venous vasculitis under 60 years of age. Venous vasculitis is suggested as a cause of intracranial hypertension, empty sella, and endocrinologic dysfunctions.

Trigeminal neuralgia with chronic paroxysmal hemicrania: the CPH-tic syndrome.

A case of chronic paroxysmal hemicrania-tic syndrome is described. The chronic paroxysmal hemicrania (CPH) was controlled with indomethacin and the trigeminal neuralgia with a glycerol blockade. The trigeminal neuralgia reappeared four years after the blockade and was then treated successfully with corticosteroids. Six months later, when indomethacin was stopped, the trigeminal neuralgia recurred and resolved again with corticosteroids. We suggest that the CHP-tic syndrome is due to periorbital venous vasculitis. To our knowledge this is the first reported case of this disorder.