Search

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The recruitment order of motor units was studied with an electromyographic technique for secure identification of single motor unit potentials. It has been shown in previous studies of normal subjects that the recruitment order in sustained voluntary contraction is predominantly stable, and that motor units which increase slowly in discharge rate with increasing contraction strength and which already attain regular discharge intervals at low frequencies are always recruited before motor units which increase more rapidly in discharge rate and which do not attain regular discharge intervals until at higher frequencies. In this study 15 patients with severe cerebellar ataxia were examined. It was shown that the recruitment order in sustained voluntary contraction in attaxia is unstable and that low- and high-frequency motor units may alternate as the unit of lowest threshold. [ABSTRACT FROM AUTHOR]

The recruitment of motor units is studied with an electromyographic technique for secure identification of single motor unit potentials. It has been shown in previous studies that the recruitment order is different in tonic and in phasic activities; in tonic activity the recruitment order is stable and low frequency units are always recruited before high frequency units; in phasic activity, however, the recruitment order is unstable and units with a higher frequency range may be recruited before units with lower frequency range. In this investigation the shifts between tonic and phasic recruitment order in voluntary contraction were compared in normal subjects and in patients with severe bradykinesia of Parkinsonism. Upon initiation of a voluntary contraction in a normal subject, phasic recruitment order may be used for a few 100 msec but tonic recruitment order then takes over. In bradykinetic patients, however, this shift from phasic to tonic recruitment order is delayed. After termination of tonic voluntary contraction in a normal subject, phasic recruitment order can again be used after a few seconds. In bradykinetic patients, however, the shift back from tonic to phasic recruitment order is also delayed. In favourable experimental situations the shift from phasic to tonic recruitment order can be normalized by passive stretch of the muscle and the shift from tonic to phasic recruitment pattern by unloading the muscle. It is discussed whether the pathological recruitment in bradykinesia might be due to disturbed gamma loop function. [ABSTRACT FROM AUTHOR]

The recruitment order of motor units in the tibialis anterior muscle upon fatigue of tonic voluntary contraction was studied in 20 patients with severe spastic paraparesis. An electromyographic technique for secure identification of single motor units was used. Before fatigue the recruitment order is stable and low-frequency units are recruited before high-frequency units; this recruitment pattern agrees with that in normal voluntary activity. When fatigue appears, however, the recruitment order becomes indefinite and high-frequency units can be recruited before low-frequency units; this recruitment pattern agrees with that in normal phasic voluntary activity. Finally, all voluntary activation power disappears, even in the case of units which have never been active. Contraction ability and original recruitment order are restored upon rest but also upon tonic reflex support of the voluntary drive. Whether the fatigue reaction may be due to insufficient gamma motoneurone innervation and whether it is related to spasticity are discussed. The practical physiotherapeutic implications are reviewed. [ABSTRACT FROM AUTHOR]

It has been shown in previous investigations that the recruitment order of motor units is different in tonic and in phasic voluntary activity. The significance of the pre-existing state of facilitation in the motoneurone pool for the recruitment of units is studied, using the phasic flexion reflex in the anterior tibial muscle as test reflex. It is shown that the recruitment order of units in a series of reflexes (1) is unstable if the subject does not expect the stimulus; (2) is stable and identical with that in tonic activity if the subject subliminally facilitates the motoneurone pool before the reflex activation; (3) is stable and almost identical with that in tonic activity if the subject expects the stimulus and therefore involuntarily influences the motoneurone pool; (4) is stable and similar to that in phasic voluntary activity if the subject inhibits the motoneurone pool before the activation and the stimulus strength thus consequentially is increased; and (5) is influenced by blockade of the proprioceptive afferent impulses from the muscle. It is concluded that normal man can select in advance the recruitment order of motor units most appropriate for the work intended. [ABSTRACT FROM AUTHOR]

The recruitment order of motoneurones in muscle contractions has been held to be largely constant and determined by the size of the cell. However, as shown in a previous investigation using electromyographic techniques, the order in which different motor units are activated during voluntary muscle contractions changes in normal human subjects on shifts from phasic to tonic contraction. In order to investigate these two types of activity also in cases in which the cerebral influence on the motoneurone pool is blocked, an analysis was made of the recruitment order in phasic and tonic flexion reflexes in 10 patients with total interruption of the spinal cord. The following four principles were found to apply and presumed to be generally valid for the isolated human spinal cord: (1) in the phasic exteroceptive reflex, the order of recruitment varies despite application of a standardized stimulus; (2) in the tonic reflex, the first unit to be recruited is usually the same even with widely different types of stimuli; (3) a shift from phasic to tonic reflex activation may result in considerable changes in recruitment order; (4) after facilitation by a subliminal long-lasting stimulus, the first unit to be recruited in the phasic reflex is also the first to be recruited in the tonic reflex. It is suggested that a tonic influence on the motoneurone pool is required for the presupposed constancy of the recruitment order. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Presents a letter to the editor responding to comments made on the author's article about the craniovascular and hemodynamic responses to head-down tilt in patients felt to have a diagnosis of chronic tension type headache.

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 andor 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). [ABSTRACT FROM AUTHOR]

Br J Neurosurg. 2002;16:55-58. 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. Comment: Although lamotrigine has shown promise, no pharmacologic treatment has been found to work consistently for SUNCT. Are we really at the surgical juncture? SJT. [ABSTRACT FROM AUTHOR]

Discusses two cases of trigeminal neuralgia and chronic paroxysmal hemicrania-tic syndrome occurring ipsilaterally. Medical condition of both patients; Medical management given; Implication of both cases.

Background: Pathologic signs in orbital phlebographies have been reported in various neurological diseases., Purpose: To study if pathologic signs in orbital phlebography may be markers of inflammation primarily affecting intracranial capillaries, which would cause intracranial hypertension., Material and Methods: Two groups with different intracranial cerebrospinal fluid pressures (Pcsf) were compared as to inflammatory markers in serum and pathologic signs in orbital phlebographies. Nine consecutive patients with idiopathic intracranial hypertension (IIH) with bilateral papilledema and eight consecutive patients with chronic tension-type headache (CTTH) were investigated prospectively with fibrinogen, orosomucoid, haptoglobin in serum, and invasive orbital phlebograms. The angiograms were evaluated by two skilled neuroradiologists, independent of each other and without knowledge of the diagnoses or aim of the study, as to the following pathologic signs: (i) narrowing of superior ophthalmic veins; (ii) caliber changes of intraorbital veins; (iii) collaterals of intraorbital veins; (iv) flow to cavernous sinus; and (v) asymmetric drainage of cavernous sinus., Results: Mean body mass index was >30 kg/m(2) in both groups. Pcsf was >200 < 250 mm H2O in 50% of the CTTH and >350 mm H2O in all IIH patients. No difference in inflammatory markers in blood was found. The phlebographies of the IIH patients had more pathologic signs and were considered pathologic significantly more often than the ones of the CTTH patients (P < 0.001)., Conclusion: The difference as to phlebographic pathologic signs between the IIH and the CTTH patients with different Pcsf supports the hypothesis that such phlebographic signs are markers of inflammation primarily affecting intracranial capillaries, which would disturb cerebrospinal fluid regulation causing intracranial hypertension.

Objective: Idiopathic intracranial hypertension (IIH) is usually considered to result from deficient intracranial absorption of cerebrospinal fluid, but has also been suggested to be caused by decreased cranial venous flow because of increased intrathoracic pressure resulting from intra-abdominal obesity. To test this hypothesis, cerebrospinal fluid pressure (Pcsf), extracranial venous pressure (Pvf), intracranial venous pressure, and body mass index (BMI) were studied in patients with IIH with papilledema compared with patients with chronic tension-type headache (CTTH)., Design and Subjects: The Pcsf and the pressures in frontal veins without (Pvf) and with bilateral compression of the supraorbital branch of the frontal veins and the superficial facial veins (Pvfc), the latter considered to be about equal to Pvfc, were studied in 10 consecutive patients with IIH with papilledema. Ten consecutive CTTH patients were used for controls. Orbital phlebography was performed to confirm that the compression of facial veins other than the frontal veins resulted in adequate communication between the frontal vein used for the studies and the cavernous sinus., Results: Cerebrospinal fluid pressure was between 200 and 250 mm water in 5 of the CTTH patients and above 350 mm water in all IIH patients. Body mass index was >25 in all CTTH patients and similar in the 2 groups. Cerebrospinal fluid pressure was similar to Pvfc in all 10 CTTH patients but significantly greater in 6 of the 10 IIH patients. Pvf was similar in the 2 groups and related to BMI., Conclusions: Chronic tension-type headache patients may be prone to have Pcsf > 200 mm water and BMI > 25. Papilledema because of intracranial hypertension occurred in the present study at Pcsf > 350 mm water. The findings of Pvfc and Pcsf being similar in all CTTH patients support the suggestion that the techniques used for measuring intracranial venous pressure are adequate. The findings of similar BMI in the CTTH and the IIH patients who differed significantly as to Pcsf refute the hypothesis that obesity precedes, and is the cause of, intracranial hypertension in IIH. The difference between Pcsf and Pvfc in 6 of the IIH patients also does not support such a hypothesis but may indicate that IIH is due to deficient intracranial cerebrospinal fluid absorption. Since a relationship between intracranial hypertension and obesity is established and obesity is not found to cause intracranial hypertension in IIH, intracranial hypertension may be suggested to be the primary cause of weight increase in IIH. Obesity, however, may secondarily increase the preexistent IIH.

Methods: Twelve consecutive patients with clinical symptoms and testing results compatible with a diagnosis of idiopathic intracranial hypotension (IIH), but no identifiable site of cerebrospinal fluid (CSF) leakage, were treated with a cervicothoracic or lumbar epidural "blood patch" (EBP) or orally administered steroids., Results: Prompt and complete relief from headache persisting for at least 4 months was attained in 3 of 4 treatments with cervicothoracic EBP, 2 of 15 with lumbar EBP, and 4 of 8 with steroids., Conclusion: These results suggest that in patients who presumably suffer from IIH and yet have no identifiable site of CSF leakage, the presumed leakage more often occurs at the cervicothoracic level than the lumbar. In addition, our experience suggests that some IIH patients may be treated effectively with oral steroids and a trial of such therapy may be considered as an alternative to EBP.

After dissection with complete occlusion of the internal carotid artery, a 58-year-old man started to suffer from intense cluster headache-like attacks. Magnetic imaging showed signs of nonsymptomatic cerebral emboli, which could be dated to have occurred in temporal relation to the start of the attacks, all on the right side. This case and two similar ones indicate that peripheral postganglionic sympathicoplegia can cause attacks with similar pain characteristics, accompanying symptoms, duration, and regularity as in cluster headache.

T cell Ig- and mucin-domain-containing molecules (TIMs) comprise a recently described family of molecules expressed on T cells. TIM-3 has been shown to be expressed on murine Th1 cell clones and has been implicated in the pathogenesis of Th1-driven experimental autoimmune encephalomyelitis. In contrast, association of TIM-1 polymorphisms to Th2-related airway hyperreactivity has been suggested in mice. The TIM molecules have not been investigated in human Th1- or Th2-mediated diseases. Using real-time (TaqMan) RT-PCR, we show that human Th1 lines expressed higher TIM-3 mRNA levels, while Th2 lines demonstrated a higher expression of TIM-1. Analysis of cerebrospinal fluid mononuclear cells obtained from patients with multiple sclerosis revealed significantly higher mRNA expression of TIM-1 compared with controls. Moreover, higher TIM-1 expression was associated with clinical remissions and low expression of IFN-gamma mRNA in cerebrospinal fluid mononuclear cells. In contrast, expression of TIM-3 correlated well with high expression of IFN-gamma and TNF-alpha. These data imply the differential expression of human TIM molecules by Th1 and Th2 cells and may suggest their differential involvement in different phases of a human autoimmune disease.

Objective: To investigate the mechanisms behind the increase of chronic tension-type headache during head-down tilt., Background: The pathophysiology of chronic tension-type headache is unknown., Design and Methods: Ten patients suffering from chronic tension-type headache and 10 age- and sex-matched controls were studied with respect to pain intensity and alterations in cranial blood volume using planar scintigraphy and radiolabeled autologous erythrocytes before, during, and after head-down tilt, a procedure known to increase chronic tension-type headache., Results: Four of 8 patients with chronic tension-type headache studied had increased cerebrospinal fluid pressure. During head-down tilt, the pain increased significantly in the group with chronic tension-type headache (P <.001) while the procedure did not cause headache in the controls. Blood volume significantly increased extracranially and decreased intracranially in both groups during head-down tilt. The extracranial nasal blood volume was significantly related to the pain experienced by the patients with chronic tension-type headache before and during head-down tilt., Conclusions: Although the changes in blood volume and, presumably, the increase of intracranial pressure were similar in the patients with chronic tension-type headache and the controls, only the patients experienced pain and pain increase during head-down tilt. This indicates that the pre-head-down tilt conditions must be different in the 2 groups and should be related to increased cerebrospinal fluid pressure/intracranial venous pressure in patients with chronic tension-type headache compared with controls. A difference in central mechanisms may, however, also be of importance for the difference in headache provocation in the 2 groups during head-down tilt.

Objective: To study the relationship between chronic tension-type headache, cranial hemodynamics, and cerebrospinal pressure., Background: Cerebrospinal pressure has been found to be above 200 mm in about 50% of patients with chronic tension-type headache., Methods: Heart rate, blood pressure, common carotid artery diameter and blood flow, and craniovascular resistance and pain at regular intervals before, during, and after head-down tilt-a procedure which increases cerebrospinal pressure, were recorded. After head-down tilt, subcutaneous injections of either placebo or 6 mg of sumatriptan were administered. Chronic tension-type headache intensity before and after withdrawal of 20 mL of cerebrospinal fluid was documented. Cerebrospinal pressure and chronic tension-type headache intensity were measured after subcutaneous injection of 6 mg of sumatriptan., Results: Head-down tilt provoked an increase of headache compared with baseline. Common carotid artery blood flow decreased and craniovascular resistance increased after sumatriptan injection, but not after placebo injection. The pain decreased after head-down tilt and placebo injection, but not after sumatriptan injection. Chronic tension-type headache intensity decreased in all 4 patients studied after withdrawal of 20 mL of cerebrospinal fluid. Cerebrospinal pressure increased in 5 patients with chronic tension-type headache after subcutaneous injection of 6 mg of sumatriptan with slight or no increase of pain., Conclusion: The results indicated that cerebrospinal pressure or intracranial venous pressure (or both) are related to chronic tension-type headache.

Objective: To investigate similarities and differences between patients with cluster headache and patients with migraine., Background: Patients with migraine and patients with cluster headache are considered, by many clinical neurologists, to be different psychologically and socially., Methods: Twenty-five age-matched pairs of men and 24 age-matched pairs of women with either migraine or episodic cluster headache (men aged 31 to 62 years; mean, 47 years; women aged 23 to 72 years; mean, 44 years) were compared with regard to coping profiles as reflected in two "coping wheels," one for the present situation and one for the future. In addition, availability of attachment and social interaction was assessed by means of the ISSI (Interview Schedule for Social Interaction)., Results: Women with cluster headache anticipated fewer activities for themselves than women with migraine, and findings were similar in the male pairs. The men with cluster headache also anticipated significantly fewer activities for themselves in the present and with others in the present and in the future than the men with migraine. There was no significant difference as to emotional loading between the two groups. A tendency to more optimistic anticipation was found in the women with cluster headache. There were highly significant differences between the two groups in the "future" wheel. The group with migraine expected more concrete activities and more activities with their families in the future, and they also described their present situation to involve more activities with others., Conclusions: Results from the present study differ from those from studies utilizing more conventional questionnaires. In particular, we found that patients with cluster headache have fewer close social contacts than patients with migraine.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

A female patient is described who had a four year long period of unilateral chronic paroxysmal hemicrania (CPH) which then became bilateral. For some years before the CPH started she suffered from periods of about one month with chronic hemicrania without nerve involvement. She also suffered from chronic fatigue, back pain, arthralgia, vertigo, chronic constipation and spontaneous ecchymoses. Blood tests showed chronic leukocytosis, low serum iron, and signs of inflammation in serum electrophoresis during the five years she was studied. CPH attacks could be provoked by breathing 6% carbon dioxide in air. Lumbar cerebrospinal fluid pressure was pathologically increased (30 cm water). The attacks decreased during indomethacin treatment but 275 mg was needed for satisfactory control of the attacks, i.e., more than the 150 mg which, according to the criteria for CPH, should be absolutely effective. Sumatriptan was found to suppress the CPH attacks as well as indomethacin. Due to these findings CPH is considered to be another manifestation of venous vasculitis. The beneficiary mechanism of indomethacin in CPH is considered to be due partly to its anti-inflammatory effects and partly to its reduction of the intracranial blood flow.

Eighteen cluster headache patients and five controls were studied using ultrasound duplex techniques to measure blood flow in the common carotid arteries after nitroglycerin and placebo administration. Vessel diameter and blood flow tended to be greater before nitroglycerin in patients in the cluster headache period than in patients out of period and controls. Nitroglycerin tended to increase blood flow only in patients not in the cluster period and in controls. There was a significant decrease in common carotid blood flow and increase in vascular resistance related to maximum pain in both nitroglycerin-induced and spontaneous cluster headache attacks. Blood flow did not reach the initial flow values after the attack was over. In one patient a hyperventilation attack only temporarily decreased the pain. We suggest that the decrease in blood flow and increase in vascular resistance may be due to constriction of intracranial arteries by reflex activation of sympathetic efferents, rather than to decrease of arterial CO2 tension.

Eight patients with episodic cluster headache, five in active episode, three out of episode, were investigated as to diameters of intracranial arteries before and after nitroglycerin (NG) administration. The diameter of all intracranial carotids were increased about 10 minutes after NG, although more in the patients in episode than in patients out of episode. The dilatation remained for the next 60 minutes in the patients who did not get a cluster headache attack. There was a normalization of the diameters of the internal carotid arteries compared to the initial values, at maximum pain in all patients who got a cluster headache attack. Similar changes were also found in the basilar arteries. The findings support the hypothesis of a constriction of intracranial arteries at maximum pain in cluster headache attacks to stop the pain.

Six patients with episodic cluster headache were investigated as to blood pressure, heart rate, cerebrospinal fluid pressure (Pcsf) and frontal vein pressure (Pvf) during five nitroglycerin (NG) provoked attacks and one spontaneous attack. In a seventh studied patient the NG failed to provoke an attack. The earlier reported decrease of systolic blood pressure and increase of diastolic blood pressure and heart rate after NG administration were also found in these patients. The "dynamite headache" was related to the start and duration of an increase of the cerebrospinal fluid pressure. There was no relationship between the start or the maximum pain of the cluster headache attack and changes in Pcsf or Pvf. On breathing oxygen during a cluster headache attack, there was a decrease of Pcsf but in some patients a temporary increase of Pvf was observed, which possibly indicates that oxygen simultaneously attains constriction of arteries and veins.

A patient with more than 20 years of SUNCT, i.e., long lasting periods with frequent attacks of intense orbital pain with a duration of about one minute, associated with ipsilateral conjunctival injection, lacrimation, rhinorrhea and facial sweating is described. Some attacks were possibly related to increased cerebral blood flow but could also be triggered from the oral area. Orbital phlebography showed pathologic changes on the side of the pain, changes which were normalized when these attacks ceased to appear. Due to these findings in conjunction with serum evidence of inflammation, associated systemic symptoms and susceptibility to steroids and azathioprine, venous vasculitis is suggested to be the cause of SUNCT in this patient. Carbamazepine and sumatriptan decreased the frequency, intensity and duration of attacks, although not completely.

Twenty consecutive patients with recurrent Tolosa-Hunt syndrome were studied. One had a parent who suffered from recurrent Tolosa-Hunt syndrome. Thirty-three percent of the patients had also recurrent periods of weeks to months of unilateral periorbital pain without ophthalmoplegia. One patient had cluster headache before the Tolosa-Hunt syndrome started. Some patients had involvement of cranial nerves outside the cavernous sinus region during Tolosa-Hunt syndrome and also between episodes. The same systemic symptoms, i.e. back pain, cold feet, arthralgia, gut problems, varices, vertigo, chronic fatigue, thrombophlebitis, memory deficiency and signs of inflammation in serum, occurred in Tolosa-Hunt syndrome as earlier found in patients with orbital venous vasculitis. Seventy-three percent of the patients had pathologic orbital phlebograms. All patients treated with steroids reacted promptly; four who developed chronic pain syndromes were treated satisfactorily with azathioprine.

One of our 7 patients (14%) with chronic cluster headache had an abnormal orbital phlebogram; this was significantly less than the 61% encountered in our 13 patients with active episodic cluster headache who had this test done. There were no pathologically increased values for serum haptoglobin or orosomucoid in our 9 patients with chronic cluster headache, again significantly less than in our 43 patients with active episodic cluster headache, 51 percent of whom had pathologically increased values of haptoglobin or orosomucoid. These inflammatory signs decreased after the episodic cluster headache was over. Episodic cluster headache we suggest to be due to temporary sympathicoplegia caused by venous vasculitis in the cavernous sinus region; chronic cluster headache we attribute to permanent post-inflammatory sympathicoplegia in the middle fossa.