Diagnostic and therapeutic trajectory of cluster headache patients in Flanders.

OBJECTIVE: A fraction of cluster headache (CH) patients face diagnostic delay, misdiagnosis, undertreatment and mismanagement. Specific data for Flanders are warranted. METHODS: Data on CH characteristics, diagnostic process and treatment history were gathered using a self-administered questionnaire with 90 items in CH patients that presented to 4 neurology outpatient clinics. RESULTS: Data for 85 patients (77 men) with a mean age of 44 years (range 23-69) were analysed. 79% suffered from episodic CH and 21% from chronic CH. A mean diagnostic delay of 44 months was reported. 31% of patients had to wait more than 4 years for the CH diagnosis. 52% of patients consulted at least 3 physicians prior to CH diagnosis. Most common misdiagnoses were migraine (45%), sinusitis (23%), tooth/jaw problems (23%), tension-type headache (16%) and trigeminal neuralgia (16%). A significant percentage of patients had never received access to injectable sumatriptan (26%) or oxygen (31%). Most prescribed preventative drugs after the CH diagnosis were verapamil (82%), lithium (35%), methysergide (31%) and topiramate (22%). Despite the CH diagnosis, ineffective preventatives were still used in some, including propranolol (12%), amitriptyline (9%) and carbamazepine (12%). 31% of patients had undergone invasive therapy prior to CH diagnosis, including dental procedures (21%) and sinus surgery (10%). CONCLUSION: Despite the obvious methodological limitations of this study, the need for better medical education on CH is evident to optimize CH management in Flanders.

Subcutaneous sumatriptan compared with usual acute treatments for migraine: clinical and pharmacoeconomic evaluation.

Cost-effectiveness and cost per successful treatment has been evaluated in 186 outpatients randomised to treat moderate to severe migraine attacks either with subcutaneous sumatriptan 6 mg (n = 97) or with their current therapy (n = 89) during an open, multicentre study of 3 months. Within 2 hours, headache severity decreased to none/mild in 86% of all attacks in the sumatriptan group (STG) compared to 25% in the customary group (CTG). Migraine was alleviated earlier in the STG than in the CTG (median 3.78 vs. 13.39 hours, p < 0.0001). The direct and total cost of treatment was 133 and 2012 BF, respectively, in the CTG and 1400 and 2522 BF, respectively, in the STG. Measuring the effectiveness of earlier pain relief with sumatriptan, the incremental cost-effective ratios for direct and total cost were 132 and 53 BF per hour of relieved pain, respectively. For this price, significantly more sumatriptan patients improved their quality of life by more than 20% (61.6 vs. 20.6% patients, p < 0.001) and less sumatriptan patient consulted a medical professional (11.3 vs. 29.2% patients, p < 0.01), used less medication for adverse events (6.2 vs. 22.5%, p < 0.001) and suffered less from associated migraine symptoms. The median number of hours of diminished work-efficiency (3 vs. 7 hours, p < 0.01) or of suspension of non-professional activity (10 vs. 24 hours, p < 0.001) was also significantly lower in the STG. The total cost per successfully treated patient was lower in the STG. Sumatriptan is more effective, provides a better quality of life, reduces health care resource utilisation, and improves work productivity as compared to the CTG, thereby resulting in a favourable cost-effectiveness ratio.

Positron emission tomography studies of changes in cerebral blood flow and oxygen metabolism in arteriovenous malformation of the brain.

Two patients with arteriovenous malformation were studied by position emission tomography, using the steady-state technique with 15O2. In the first patient, who had seizures, preceded by auditory hallucinations, an area of decreased cerebral blood flow and oxygen consumption was shown just behind the malformation. In the second patient, who had repeated attacks of right hemiparesis, an area of decreased blood flow and oxygen metabolism was shown at distance of the malformation. These areas of low flow and metabolism are most probably the result of a vascular steal phenomenon and the origin of the transient neurological symptoms in both patients.

Familial meningioma. Case report with cytogenetic study.

A family is presented of which two members were shown to have an intracranial meningioma. We performed a chromosome study in the affected living individual and several other relatives, with special attention for chromosome 22 anomalies. In neither of them a chromosomal abnormality could be detected. However, in view of the recent cytogenetic findings reported in meningioma, it is suggested that karyotyping should be performed in every family with two or more members affected by meningioma.

Free light chains in the cerebrospinal fluid: an indicator of recent immunological stimulation.

A group of 38 patients with immunoglobulin free light chains (kappa and/or lambda) in the cerebrospinal fluid was studied. Only five of them showed one band of kappa free light chains, 36 of them showed one or more bands of lambda free light chains. There were 33 multiple sclerosis patients among them and they were compared with 33 control multiple sclerosis patients who did not have free light chains but only showed an oligoclonal pattern of immunoglobulins in their CSF. Of several clinical and pathological parameters, only three were statistically significant: (A) for all patients, as well as for the multiple sclerosis patients alone, elevated numbers of free light chains of type lambda were associated with the elevated numbers of white blood cells in the CSF (p less than 0.003), (B) for multiple sclerosis patients, elevated numbers of free light chains of type lambda were associated with associated with shorter time intervals between the last relapse and the date of the lumbar puncture (p less than 0.004) and (C) also with shorter duration of the disease (p less than 0.01). The presence of lambda free light chains in the cerebrospinal fluid seems to indicate that recent antigenic stimulation has occurred within the central nervous system following exacerbation. The inverse association of the number of lambda free light chains with the duration of the disease is suggestive of a pathological expression of the "burning out" phenomenon of the multiple sclerosis process.

Intracranial bilateral symmetrical calcification on CT-scanning. A case report and a review of the literature.

The case of a 57-year-old woman with idiopathic hypoparathyroidism is presented. A CT-scan showed extensive bilateral symmetrical calcification in the region of the basal ganglia, nuclei of the cerebellum and the cerebral and cerebellar white matter. A review of the literature showed that bilateral symmetrical calcification detected by CT is usually small in extent and is most often confined to the globus pallidus. It is most commonly found in patients older than 50 years, who only rarely have symptoms associated with it. The finding is, though, non-specific and may occur in a variety of pathological conditions both with and without an aetiological relationship. Further study of the cerebral parathormone responsive adenylate cyclase enzyme proves hopeful to elucidate the aetiology of idiopathic bilateral symmetrical calcification.

Intracerebral osteoma: a clinicopathologic and neuropsychologic case study.

This case report deals with a 16-year-old girl, admitted for headache and Jacksonian seizures. The clinical examination showed no gross disturbances, but the neuropsychologic examination revealed difficulties in speech-linked abstract thinking. Extensive radiologic investigations revealed the presence of a frontal intracallosal bony tumor, protruding into the lateral ventricles. Histologic examination of the surgical specimen showed a large benign osteoma, without any connection with dura or skull. In view of the paucity of symptoms, a dysontogenetic etiology of the tumor seems obvious.