[Posture-dependent headache caused by spontaneous intracranial hypotension]. / Houdingsafhankelijke hoofdpijn door spontane intracraniële hypotensie.

A 36-year-old woman presented with a 3-4 month history of severe, progressive headache. The headache was characterized by postural variation, with excruciating headache in the upright position and near-immediate relief upon recumbence. There was no history of trauma or lumbar puncture. Gadolineum-enhanced brain MRI revealed abnormalities characteristic intracranial hypotension. Spinal MRI showed a longitudinal extradural fluid collection; a localization of the dural defect was not found. The patient was treated with caffeine, bed rest and lumbar epidural blood patches; she recovered completely. Severe orthostatic headache which aggravates upon standing and is relieved by recumbence, can be caused by spontaneous intracranial hypotension. Recognition of its characteristic symptoms is needed for timely referral. Treatment is usually successful and can prevent life-threatening complications.

[Awakening ptosis]. / Kortdurende ptosis bij het ontwaken.

BACKGROUND: A variable ptosis may point towards serious neurological disorders and is presented to general practitioners, ophthalmologists and neurologists. CASE DESCRIPTION: Two patients presented at the neurology outpatient clinic with a ptosis confined to awakening from sleep. There were no other neurological complaints and neurological examination was normal. The diagnosis 'awakening ptosis' was made. CONCLUSION: Awakening ptosis is a benign, but rare disorder. The exact pathophysiology remains unclear. In the case of a classic clinical picture of awakening ptosis, additional examinations are not indicated.

Incidence of subarachnoid haemorrhage: a systematic review with emphasis on region, age, gender and time trends.

BACKGROUND AND AIM: To update our 1996 review on the incidence of subarachnoid haemorrhage (SAH) and assess the relation of incidence with region, age, gender and time period. METHODS: We searched for studies on the incidence of SAH published until October 2005. The overall incidences with corresponding 95% confidence intervals were calculated. We determined the relationship between the incidence of SAH and determinants by means of univariate Poisson regression. RESULTS: We included 51 studies (33 new), describing 58 study populations in 21 countries, observing 45,821,896 person-years. Incidences per 100,000 person-years were 22.7 (95% CI 21.9 to 23.5) in Japan, 19.7 (18.1 to 21.3) in Finland, 4.2 (3.1 to 5.7) in South and Central America, and 9.1 (8.8 to 9.5) in the other regions. With age category 45-55 years as the reference, incidence ratios increased from 0.10 (0.08 to 0.14) for age groups younger than 25 years to 1.61 (1.24 to 2.07) for age groups older than 85 years. The incidence in women was 1.24 (1.09 to 1.42) times higher than in men; this gender difference started at age 55 years and increased thereafter. Between 1950 and 2005, the incidence decreased by 0.6% (1.3% decrease to 0.1% increase) per year. CONCLUSIONS: The overall incidence of SAH is approximately 9 per 100,000 person-years. Rates are higher in Japan and Finland and increase with age. The preponderance of women starts only in the sixth decade. The decline in incidence of SAH over the past 45 years is relatively moderate compared with that for stroke in general.

Blended care; development of a day treatment program for medically unexplained physical symptoms (MUPS) in the Dutch Armed Forces.

BACKGROUND: A subgroup of servicemen can be identified that seek a disproportionally amount of health care in comparison to diagnostic and therapeutic perspectives. This group can be identified on the basis of an absence of a structural medical explanation for their symptoms. The symptoms manifest predominantly as fatigue and pain, and are often chronic. Patients with medical unexplained medical symptoms (MUPS) often have multiple and complex problems that would be best treated by a multidisciplinary team of medical specialists and paramedics. The military is characterized by high loyalty towards peers and leadership, leading to neglect for personal care. OBJECTIVE: While consensus on the biological basis for these complaints is lacking, awareness on the need for effective treatments for this patient group is high. METHOD: Based on reviews, expert recommendations and clinical demand, a specialized treatment program for soldiers with MUPS has recently been developed and implemented in the system of health care in the Netherlands Armed Forces. We developed a functional rehabilitation program with blended care elements of cognitive behavioral therapy (CBT), physical therapy, case management, and psychoeducation, embedded in a day treatment setting. RESULTS: The program received high scores on participant as well as team satisfaction. The program is illustrated by two clinical vignettes. CONCLUSION: The blended care program for MUPS that focused on allostatic load awareness offered a more holistic and preventive approach that contributed to a reduction of unnecessary medical consumption, and increased job participation. We recommend that the development of guidelines for diagnoses and treatment of these complaints in military settings will improve the quality of patient care, reduce disability, facilitate reintegration, and encourage scientific research.

Follow-up of idiopathic thunderclap headache in general practice.

Follow-up studies of idiopathic thunderclap headache (ITH) have found no subsequent subarachnoid hemorrhage (SAH) or other serious neurological disease, but the effect on life-style has not been studied. To assess the long-term outcome of patients with ITH in general practice we prospectively followed 93 patients with an episode of ITH during 1988-1993, of whom 77 were referred to hospital. ITH was defined as a sudden, unusually severe headache that started within 1 min, lasted at least 1 h, and for which no underlying cause was found. These patients were treated in 252 general practices. Outcome measures were subsequent SAH, subsequent headaches, absence from work, and diminished daily functioning. Patients were followed up by their general practitioner for a median of 5 years (range 1-10). Three patients died, all from non-neurological diseases. No subsequent SAH was diagnosed in any of the 93 patients. Recurrent attacks of ITH occurred in 8 patients, and 13 developed new tension headache or migraine. Absence from work because of headache was recorded in 11 patients, and in the overall group 6 patients were dependent on welfare. In only one-half of patients (n=52) did the general practitioner judge the level of daily functioning to be similar to that before the index episode of ITH. Thus, although no episodes of SAH occurred after ITH during long-term follow-up, one-half of patients with ITH had a lower level of daily functioning, and one-eighth had reduced working capacity, specifically because of headache.

Causes and management of thunderclap headache: a comprehensive review.

BACKGROUND: Thunderclap headache (or sudden severe headache) is an uncommon type of headache. Recognition and accurate diagnosis of this headache are important, because there is often a serious underlying brain disorder. SUMMARY: In this article, causes and management of thunderclap headache are discussed. In the primary care setting, there is a serious cause in one third of patients, but in the hospital setting, up to two thirds of patients have a serious underlying brain disorder. Clues in history and physical examination can point to a possible serious underlying cause of thunderclap headache, such as subarachnoid hemorrhage, intracranial hematoma, or cerebral venous thrombosis. The remaining patients with thunderclap headache, however, have a primary headache disorder, such as migraine or (less frequently) tension headache with an unusual sudden onset, exertional headache, coital headache, cough headache, or cluster headache. The concept of thunderclap headache as a distinct clinical entity is discussed, with implications for its evaluation. Present radiological techniques are reviewed with regard to their diagnostic utility in detecting a serious brain disorder. CONCLUSIONS: Thunderclap headache is an uncommon type of headache, and a serious underlying cause should be excluded.

Bilateral anterior inferior cerebellar artery territory infarcts.

Bilateral anterior inferior cerebellar artery (AICA) territory infarcts are rare. Their occurrence usually signifies severe intracranial vertebrobasilar disease. Unlike head computed tomography, magnetic resonance (MR) imaging reveals these infarcts clearly and MR angiography allows the intracranial vasculature to be defined noninvasively. We now report a patient with bilateral AICA territory infarcts.

Segmental fasciculations as a late sequel of spinal cord injury.

In two patients with a history of spinal cord injury many years ago fasciculations developed in muscles belonging to previously damaged segments. In both patients MRI of the cervical spine showed an area of abnormal signal intensity representing a cavity, near the level of the neurological abnormalities and the cord injury. During a 4-year follow-up period no new neurological or MRI abnormalities were detected. A hypothesis for the genesis of fasciculations following spinal cord injury is presented.

[Acute severe headache: a subarachnoidal hemorrhage?]. / Acute hevige hoofdpijn: subarachnoïdale bloeding?

Five patients, three women aged 87, 50, and 31 years, and two men aged 31 and 32 years, presented with severe headache of sudden onset. A sudden onset of unusually severe headache is suggestive of an intracranial haemorrhage or other serious disease, even in the absence of focal neurologic deficits. The diagnoses were subdural haematoma, cerebral venous sinus thrombosis, idiopathic thunderclap headache, subarachnoid haemorrhage, and viral meningitis, respectively. There are no characteristics from history or examination that accurately discriminate among all these causes; idiopathic thunderclap headache and subarachnoid haemorrhage are commonest. Consultation of a neurologist and further ancillary investigations are necessary for proper diagnosis and treatment.

Chance of aneurysm in patients suspected of SAH who have a 'negative' CT scan but a 'positive' lumbar puncture.

In patients with sudden severe headache and a negative computed tomography (CT) scan, a lumbar puncture (LP) is performed to rule in or out a subarachnoid haemorrhage (SAH), but this procedure is under debate. In a hospital-based series of 30 patients with sudden headache, a negative CT scan but a positive LP (defined as detection of bilirubin >0.05 at wavelength 458 nm), we studied the chance of harbouring an aneurysm and the clinical outcome. Aneurysms were found in none of both patients who presented within 3 days, in 8 of the 18 (44%) who presented within 4-7 days and in 5 of the 10 (50%) who presented within 8-14 days. Of the 13 patients with an aneurysm, 3 (23%) had poor outcome. In patients who present late after sudden headache, the yield in terms of aneurysms is high in those who have a positive lumbar puncture. In patients with an aneurysm as cause of the positive lumbar puncture, outcome is in the same range as in SAH patients admitted in good clinical condition.

Primary thunderclap headache.

Thunderclap headache is an uncommon type of headache, but recognition and diagnosis are important because of the possibility of a serious underlying brain disorder. In this chapter, primary thunderclap headache in relation to other primary headache disorders and secondary, symptomatic headache disorders are discussed. Most importantly, subarachnoid hemorrhage should be excluded. The first investigation is a computed tomography (CT) scan, and, if the CT scan is negative, investigation of the cerebrospinal fluid. Other symptomatic vascular causes are intracranial hemorrhage, cerebral venous sinus thrombosis, cervical artery dissection, or a reversible vasoconstriction syndrome. These and other serious underlying intracranial disorders should be detected by magnetic resonance imaging or the appropriate investigations. The remaining patients with thunderclap headache most likely represent a primary headache disorder, including migraine, primary cough headache, primary exertional headache, or primary headache associated with sexual activity. Within the group of primary headache disorders, primary thunderclap headache represents a distinct clinical entity; it is characterized by a sudden severe headache lasting from 1h up to 10 days and not attributed to another disorder. The pathogenesis of primary thunderclap headache is still not known, but the sympathetic nervous system may play an important role.

Visual inspection versus spectrophotometry in detecting bilirubin in cerebrospinal fluid.

OBJECTIVES: To compare the diagnostic accuracy of visual inspection and spectrophotometry for identifying the presence of bilirubin in the cerebrospinal fluid (CSF). METHODS: Clinicians and students assessed CSF specimens with seven degrees of extinction between 0.00 and 0.09 at 450-460 nm as "yellow," "doubtful," or "colourless" after random presentation under standard conditions. The assessments were compared with spectrophotometry, with 0.05 being taken as the cut off level for the presence of bilirubin. Results were compared between the two groups and explored by means of receiver operating characteristic (ROC) curves. RESULTS: All 51 clinicians and 50 of 51 students scored the tubes with extinction of 0.06 or higher as "yellow" or "doubtful." Tubes without any bilirubin were scored as "yellow" by three of the students only. The ROC curves confirmed that the diagnostic properties of the visual inspection versus spectrophotometry were slightly better for the clinicians than for the students. CONCLUSIONS: If CSF is considered colourless, the extinction of bilirubin is too low to be compatible with a diagnosis of recent subarachnoid haemorrhage. If CSF is not considered colourless, spectrophotometry should be carried out to determine the level of extinction of bilirubin.

Incidence of subarachnoid hemorrhage: role of region, year, and rate of computed tomography: a meta-analysis.

BACKGROUND AND PURPOSE: The incidence of subarachnoid hemorrhage (SAH) has been estimated for many years at 10 to 15 per 100 000 person-years, but the most recent studies yield lower figures, of 6 to 8 per 100 000 person-years. To investigate the cause of this apparent decline, we studied the influence of year to study, rate of CT,and region. RESULTS: Eighteen studies fulfilled predefined inclusion criteria. In three Finnish studies, the pooled incidence was 21.4 per 100 000 person-years (95% confidence interval [CI], 19.5 to 23.4); in 15 non-Finnish studies, it was 7.8 per 100 000 person-years (95% CI, 7.2 to 8.4). With univariate analysis, in non-Finnish studies the incidence decreased .96% for each percentage point increase of patients investigated with CT (rate ratio, -0.9904; 95% CI, 0.9878 to 0.9930). With 100% CT scanning, the incidence of SAH outside Finland is estimated at 6 per 100 000 person-years. The rate ratio for year of study was 0.952 (95% CI, 0.935 to 0.969) for each later year in the period 1960 to 1994. In multivariate analysis, only the use of CT was independently related to SAH incidence. For the Finnish studies, the rate ratios for use of CT and year to study were not statistically significant. We also found in six studies that incidence for women was 1.6 (95% CI, 1.1 to 2.3) times higher than that for men (7.1 [95% CI, 5.4 to 8.7] and 4.5 [95% CI, 3.1 to 5.8], respectively). CONCLUSIONS: The actual incidence of SAH has remained stable over the last three decades; the apparent decline in incidence is entirely explained by the greater proportion of patients investigated with CT. The incidence of SAH in Finland is almost three times as high as in other parts of the world.

The notion of "warning leaks" in subarachnoid haemorrhage: are such patients in fact admitted with a rebleed?

OBJECTIVE: Often patients with subarachnoid haemorrhage (SAH) recall a recent episode of acute severe headache, usually interpreted as a "warning headache" or first SAH. An alternative explanation is recall bias. The clinical and radiological features of patients with SAH were studied in relation to previous headaches or later rebleeding. METHODS: Patients with either a previous headache episode or a subsequent rebleed were selected from the SAH database in Utrecht within 1 month of the index SAH. The clinical condition was graded on the World Federation of Neurological Surgeons (WFNS) scale. The CT was reviewed and the amounts of subarachnoid blood, hydrocephalus, and intraventricular, intracerebral, and subdural blood were rated. Proportions were compared by unpaired or paired t test. RESULTS: Forty four of 390 patients (11%) had had a severe headache before their index SAH (11 of these had a subsequent rebleed); 31 other patients had a rebleed in hospital but no preceding headache. Patients with and without preceding headache did not differ in level of consciousness (14 of 44 v 11 of 31 were comatose), nor in any of the radiological features. After rebleeding (42 patients), 37 of 42 patients were comatose (v 11 of 42 before), and CT showed higher proportions of intracerebral haemorrhage (17%), intraventricular haemorrhage, (27%), and hydrocephalus (12%) than baseline scans. Intraventricular haemorrhage was twice as frequent after rebleeding than at baseline. CONCLUSIONS: The clinical and radiological features of patients admitted with SAH after a preceding bout of headache did not differ from those without such an episode, and are clearly dissimilar from those after documented rebleeds. The findings challenge the existence of minor "warning headaches".

Headache characteristics in subarachnoid haemorrhage and benign thunderclap headache.

One third of patients with aneurysmal subarachnoid haemorrhage (ASAH) present with headache only. A prompt diagnosis is crucial, but these patients must be distinguished from patients with non-haemorrhagic benign thunderclap headache (BTH). The headache characteristics and associated features at onset in subarachnoid haemorrhage and benign thunderclap headache were studied to delineate the range of early features in these conditions. In this prospective study, one of two observers interviewed 102 patients with acute severe headache by means of a standard questionnaire. The patients were alert on admission and had no focal deficits. ASAH was subsequently diagnosed in 42 patients, non-aneurysmal perimesencephalic haemorrhage (PMH) in 23 patients, and BTH in 37 patients. Headache developed almost instantaneously in 50% of patients with ASAH, 35% of patients with PMH, and 68% of patients with BTH and within 1 to 5 minutes in 19%, 35%, and 19%, respectively. Loss of consciousness was reported in 26% of patients with ASAH, 4% of patients with PMH and 16% of patients with BTH, and transient focal symptoms in 33%, 9%, and 22% respectively. Seizures and double vision had occurred only in ASAH. Vomiting and physical exertion preceding the onset of headache were more frequent in patients with ASAH (69% and 50%) and those with PMH (83% and 39%) than in those with BTH (43% and 22%). Headache developed almost instantaneously in only half the patients with aneurysmal rupture and in two thirds of patients with benign thunderclap headache. In patients with acute severe headache, female sex, the presence of seizures, a history of loss of consciousness or focal symptoms, vomiting, or exertion increases the probability of ASAH, but these characteristics are of limited value in distinguishing ASAH from BTH. Aneurysmal rupture should be considered even if focal signs are absent and the headache starts within minutes.

Prospective study of sentinel headache in aneurysmal subarachnoid haemorrhage.

Retrospective surveys of patients with subarachnoid haemorrhage suggest that minor episodes with sudden headache (warning leaks) may precede rupture of an aneurysm, and that early recognition and surgery might lead to improved outcome. We studied 148 patients with sudden and severe headache (possible sentinel headache) seen by 252 general practitioners in a 5-year period in the Netherlands. Subarachnoid haemorrhage was the cause in 37 patients (25%) (proven aneurysm in 21, negative angiogram in 6, no angiogram done in 6, sudden headache followed by death in 4). 103 patients had headache as the only symptom, 12 of whom proved to have subarachnoid haemorrhage (6 with a ruptured aneurysm). Previous bouts of sudden headache had occurred in only 2. Other serious neurological conditions were diagnosed in 18. In the remaining 93, no underlying cause of headache was found; follow-up over 1 year showed no subsequent subarachnoid haemorrhage or sudden death. In this cohort, acute, severe headache in general practice indicated a serious neurological disorder in 37% (95% CI 29-45%), and subarachnoid haemorrhage in 25% (18-32%). 12% (5-18%) of those with headache as the only symptom. The notion of warning leaks as a less serious variant of subarachnoid haemorrhage is not supported by this study. Early recognition of subarachnoid haemorrhage is important but will probably have only limited impact on the outcome in the general population.

Risk of primary sclerosing cholangitis is associated with nonsmoking behavior.

BACKGROUND & AIMS: Seventy percent of patients with primary sclerosing cholangitis (PSC) have concomitant ulcerative colitis. Smoking and previous appendectomy may protect against ulcerative colitis. The aim of this study was to examine these factors in patients with PSC. METHODS: Fifty-nine patients with PSC, 130 patients with ulcerative colitis and normal liver biochemistry, and 197 control subjects were interviewed about smoking behavior and history of appendectomy. RESULTS: There were less current smokers in the PSC and ulcerative colitis groups than in the control group (19%, 12%, and 38%, respectively). The resulting odds ratio for current smoking was 0.37 (95% confidence interval, (0.18-0.76) in the PSC group and 0.23 (95% confidence interval, 0.13-0.41) in the ulcerative colitis group. Percentage of persons who ever smoked was also significantly less in the PSC group (41% vs. 56% in the control group). Frequency of previous appendectomy in the PSC and ulcerative colitis groups was not significantly different from that of controls (19%, 9%, and 14%, respectively). CONCLUSIONS: Smoking but not previous appendectomy is associated with decreased risk of PSC.