[Electrotherapy for headaches]. / De elektrische behandeling van hoofdpijn.

Neuromodulation is being applied increasingly for the treatment of drug resistant headache. Although these techniques are often considered high-tech, electrotherapy for headache has a long history; electric fish have been used for headache treatment since the first century CE. During the eighteenth and nineteenth century, static electricity was a treatment for a wide variety of neuropsychiatric disorders including headache. The efficacy of electrotherapy, however, has been disputed continuously, since opponents were of the opinion that the positive results could be attributed to suggestion. At the beginning of the twentieth century, the electric treatment of headache gradually disappeared. In recent years, there has been a resurgence in the use of electrotherapy, along with the efficacy debate. With this historical review we wish to emphasize the importance of placebo-controlled studies, not only in terms of electrotherapy of headache, but also for the evaluation of neuromodulation for other disorders.

High-flow oxygen therapy in cluster headache patients has no significant effect on nociception specific blink reflex parameters: a pilot study.

BACKGROUND: The exact pathophysiology of cluster headache is unclear. We examined the influence of interneurons on the trigemino-facial reflex arch and the effect of oxygen, by using the nociception specific blink reflex parameters. FINDINGS: There is no significant effect of oxygen, immediately and over time, on the nociception specific blink reflex parameters in ten male patients during the active phase of cluster headache, outside attacks. Also, there is no significant difference between the symptomatic and asymptomatic side. None of the subjects experienced a cluster headache attack during study participation. We therefore present the collected data as reference values of nociception specific trigeminal stimulation and the effect of oxygen on nociception specific blink reflex parameters. CONCLUSION: The nociception specific blink reflex seems not a suitable instrument for exploring the pathophysiology of cluster headache.

Cluster headache and oxygen: is it possible to predict which patients will be relieved? A prospective cross-sectional correlation study.

Response to 100 % oxygen as acute treatment for cluster headache is relative low considering certain subgroups or predictors. The primary purpose of the present study was to find prospectively which factors differ between responders and non-responders to oxygen therapy. The second goal was to find whether any of these differences would clarify the mechanism of pain reduction by oxygen and cluster headache pathophysiology. Patients diagnosed with cluster headache according to the ICHD-II criteria, who started on oxygen therapy (n = 193), were recruited from 51 outpatient clinics and via patient websites in The Netherlands. Patients had to return two questionnaires around the start of oxygen therapy (n = 120). Eventually, 94 patients were included. Clear non- plus moderate responders had ever used pizotifen more often (p = 0.03). Clear non-responders more often had photophobia or phonophobia during cluster headache attacks (p = 0.047) and more often had used triptans in the same active phase as the phase in which they had used oxygen for the first time (p = 0.02). Using correction for multiple testing, we could only confirm a statistically significant difference in triptan use. We were unable to locate the level of action of oxygen in the thalamus and cortex or confirm the sites of its action presently known, solely based on current knowledge of photophobia circuits. However, we conclude that particularly the higher frequency of photophobia or phonophobia in clear non-responders deserves further study to understand the mechanism of pain reduction by oxygen and cluster headache pathophysiology.

Is isolated aphasia a typical presentation of presumed cardioembolic transient ischemic attack or stroke?

BACKGROUND: Previous studies have suggested that patients with a transient ischemic attack (TIA) or minor ischemic stroke and isolated aphasia should be carefully screened for a potential cardiac source of embolism. Most of these publications, however, were case reports or small-series. The purpose of this study was to assess the relationship between isolated aphasia and atrial fibrillation (AF) as the cause of presumed cardioembolic TIA or stroke within the setting of 2 large multicenter trials. METHODS: The frequency of isolated aphasia was compared between patients with a TIA or minor ischemic stroke either with AF [European Atrial Fibrillation Trial (EAFT), n = 1,001] or without AF [Dutch TIA Trial (DTT), n = 3,150]. We analyzed data with univariable and multivariable logistic regression. Isolated aphasia was defined as aphasia without dysarthria, visual-field defects or motor or sensory deficits of the arm, leg or face. Because dysarthria can be difficult to detect in aphasic patients, a second analysis was done without excluding dysarthric patients. In a third analysis, we excluded patients with a symptomatic lacunar infarct from the DTT, as these patients were overrepresented due to the exclusion of patients with AF. Subgroup analysis was performed for patients presenting with TIA and minor stroke. RESULTS: Of 4,151 patients, 210 (5.1%) had isolated aphasia, 109 from the EAFT and 101 from the DTT, crude odds ratio (OR) 3.69, 95% confidence interval (CI) 2.79-4.89. Patients with isolated aphasia were older (mean age 70.3 vs. 66.8 years, p < 0.01), more often female (OR 1.87, 95% CI 1.41-2.46), and more often had diabetes (OR 1.73, 95% CI 1.16-2.59) and hypercholesterolemia (OR 1.83, 95% CI 1.11-3.03) than those without aphasia. After simultaneous adjustment for age, sex, diabetes and hypercholesterolemia, patients with isolated aphasia still had AF more often than patients without isolated aphasia (adjusted OR 2.94, 95% CI 2.16-4.01). Both after inclusion of patients with dysarthria in the group of patients with isolated aphasia and after exclusion of patients with a symptomatic lacunar infarct, essentially the results remained the same. Patients presenting with isolated aphasia due to a TIA tended to have AF more often than patients with a minor ischemic stroke. CONCLUSIONS: Isolated aphasia is an independent sign of AF in patients with a TIA or minor ischemic stroke. Careful cardiac screening seems warranted in patients with isolated aphasia, as secondary prevention is different in patients with a cardiac source of embolism.

Trigeminal autonomic cephalalgia sine headache.

Cluster headache without headache (CH-H) has been described several times. We add three new CH-H patients and a patient with (probable) paroxysmal hemicrania without headache (PH-H). We searched the literature and found some more cases of CH-H and PH-H. CH-H attacks may have a shorter minimal attack duration than CH attacks. We propose the term trigeminal autonomic cephalalgia without headache (TAC-H) for autonomic attacks and/or extracephalic pain or sensory symptoms with an attack duration and distribution and/or response to therapy suggesting one of the trigeminal autonomic cephalalgias, but without accompanying headache. Secondary TAC-H may develop after treatment for painful TAC attacks. We discuss pathophysiological issues, particularly the central role of the hypothalamus and the suggestion that the superior salivatory nucleus (SSN) might be triggered by the diencephalic pacemaker without nociceptive activation.

Cluster headache and oxygen: is it possible to predict which patients will be relieved? A retrospective cross-sectional correlation study.

Most cluster headache patients respond to oxygen therapy, but approximately 20% do not. The aim of the present study was to assess which factors differ between cluster headache patients who respond to oxygen therapy and those who do not. We included patients from the headache clinic of Atrium Medical Centre Heerlen (n = 53) and patients who responded to a cluster headache web-site (n = 62). Participants completed a questionnaire with questions on cluster headache and factors that might be of significance with respect to the response to oxygen. Non-responders had less often smoked in the past (p = 0.014), had longer cluster headache attacks (p = 0.049), and more often reported interictal headache (p = 0.02) than responders. Logistic regression analysis showed these variables to be independent risk factors for not responding to oxygen and a clinical prediction model is provided. The area under the receiver operating characteristic curve was 0.75. We conclude that cluster headache patients who smoked in the past, had shorter attacks and were pain-free interictally respond best to oxygen inhalation. The results did not provide clues for the mechanism of action of oxygen therapy.

The diagnostic value of provocative clinical tests in ulnar neuropathy at the elbow is marginal.

BACKGROUND: Provocative clinical tests are often performed in the diagnosis of ulnar neuropathy at the elbow (UNE) although the evidence for the usefulness of these tests is limited. The aim of this study was to determine the diagnostic value of provocative clinical tests in the diagnosis of UNE in a relevant spectrum of patients and controls. METHODS: A prospective cohort study was performed in consecutive patients clinically suspected of having UNE. All patients underwent a neurological examination and four commonly used provocative clinical tests (Tinel's test, flexion compression test, palpating for local ulnar nerve tenderness and nerve thickening). Subsequently, in all patients a reference standard test comprising electrophysiological studies and neurosonography was independently assessed. RESULTS: 192 eligible patients completed the study protocol. UNE was diagnosed in 137 and an alternative diagnosis was made in 55 patients. The sensitivity, specificity, and positive and negative predictive values were as follows: Tinel's test 62%, 53%, 77% and 30%; flexion compression test 61%, 40%, 72% and 29%; palpating for nerve thickening 28%, 87%, 84% and 33%; and palpating for nerve tenderness 32%, 80%, 80% and 32%. Logistic regression and receiver operating characteristic curves showed that the added value of one or more provocative tests over routine clinical examination is minimal. CONCLUSION: The diagnostic value of provocative clinical tests in UNE is poor.

[Success of carotid endarterectomy dependent on type of atherosclerotic plaque and gender, among others]. / Succes van carotisendarteriëctomie afhankelijk van onder meer het type atherosclerotische plaque en het geslacht.

Carotid endarterectomy (CEA) reduces the risk of stroke in both symptomatic and asymptomatic patients with a high-grade stenosis of the internal carotid artery. Surgery, however, is less beneficial for women than for men. Besides gender, other factors, like degree ofstenosis and plaque morphology, influence the risk of stroke and the beneficial effect of CEA. A recent study shows that women, asymptomatic women in particular, have more stable atherosclerotic carotid plaques than men. Increasing knowledge regarding local plaque characteristics should be carried through to clinical practice. Further studies, especially prospective studies, are needed to identify subgroups of patients that will benefit most from CEA. Low surgical morbidity and mortality remain a prerequisite to perform CEA in symptomatic carotid stenosis and even more so in asymptomatic carotid stenosis.

History of methysergide in migraine.

Harold Wolff's theory of vasodilation in migraine is well-known. Less known is his search for a perivascular factor that would damage local tissues and increase pain sensitivity during migraine attacks. Serotonin was found to be among the candidate agents to be included. In the same period, serotonin was isolated (1948) and, because of its actions, an anti-serotonin drug was needed. Methysergide was synthesized from lysergic acid (LSD) by adding a methyl group and a butanolamid group. This resulted in a compound with selectivity and high potency as a serotonin (5-HT) inhibitor. Based on the possible involvement of serotonin in migraine attacks, it was introduced in 1959 by Sicuteri as a preventive drug for migraine. The clinical effect was often excellent, but 5 years later it was found to cause retroperitoneal fibrosis after chronic intake. Consequently, the use of the drug in migraine declined considerably, but it was still used as a 5-HT antagonist in experimental studies. In 1974 Saxena showed that methysergide had a selective vasoconstrictor effect in the carotid bed and in 1984 he found an atypical receptor. This finding provided an incentive for the development of sumatriptan. Bredberg et al. showed that methysergide is probably a prodrug for its active metabolite methylergometrine. Whereas methysergide is 'a clean drug', methylergometrine is 'a relatively dirty drug' with additional dopaminergic activity. The mechanism for the preventive effect of methysergide (methylergometrine) in migraine remains elusive. We describe the rise, fall and subsequent use as a third-choice drug of the first effective migraine prophylactic, methysergide.

History of the use of ergotamine and dihydroergotamine in migraine from 1906 and onward.

Dale showed in 1906 in a seminal work that ergot inhibits the pressor effect of adrenaline. Stoll at Sandoz isolated ergotamine from ergot in 1918. Based on the belief that migraine was due to increased sympathetic activity, ergotamine was first used in the acute treatment of migraine by Maier in Switzerland in 1925. In 1938 Graham and Wolff demonstrated the parallel decrease of temporal pulsations and headache after ergotamine i.v. This inspired the vascular theory of Wolff: an initial cerebral vasoconstriction followed by an extracranial vasodilation. Dihydroergotamine (DHE) was introduced as an adrenolytic agent in 1943. It is still in use parenterally and by the nasal route. Before the triptan era ergotamine and DHE had widespread use as the only specific antimigraine drugs. From 1950 the world literature on ergotamine was dominated by two adverse events: ergotamine overuse headache and the relatively rare overt ergotism. Recently, oral ergotamine, which has an oral bioavailability of < 1%, has been inferior to oral triptans in randomized clinical trials. A European Consensus in 2000 concluded that ergotamine is not a drug of first choice. In an American review of 2003 it was suggested that ergotamine may be considered in the treatment of selected patients with moderate to severe migraine.

Vinken and Bruyn's Handbook of Clinical Neurology. A witness of late-twentieth century neurological progress.

Vinken and Bruyn's Handbook of Clinical Neurology (HCN) is best characterized as an encyclopedia. In this paper we describe the origin, production, and reception of HCN. Data were gathered from a literature search, by screening of HCN-volumes, interviewing key-role persons and a study of an HCN-archive. The initiative for HCN was taken by two Excerpta Medica staff members, the one a strategist with expertise in information systems, the other a gifted neurologist with an expert knowledge of who is who in the world of neurological literature. Within a period of 38 years, 2799 authors, 28 volume editors, the two initiators, and a third chief editor for the American continent described the whole of neurology in 1909 chapters on all together 46,025 pages (excluding index volumes). HCN was sold mainly to medical institutes in affluent countries. A digital version of the revised edition was proposed by the editors but refused by the publisher for commercial reasons. HCN was in general well received by book reviewers. The main criticisms concerned the price of the volumes, lack of editorial control, inadequacy of indexes, and lack of cross references. HCN offers unrivalled information on the state of the art of the clinical neurosciences in the second half of the twentieth century. In addition, it contains extensive reviews of the history of neurological diseases in the volumes of the original edition.

[The South-American eel. Two early letters on animal electricity from the Dutch colonies]. / De Zuid-Amerikaanse aal. Twee vroege brieven uit de Nederlandse koloniën over dierlijke elektriciteit.

Speculations about the nature of nerve action, including animal spirits, date back to antiquity. Only during the 18th century, when it became possible to store electricity in Leyden jars, did natural philosophers begin to realise that the sensations caused by electric fish are like those produced by these primitive capacitors. The important roles played by amateur observers in the Dutch colonies of South-America, and how they communicated with more established Dutch scientists in The Netherlands, are often relatively overlooked in histories of how the nerves became electrical. In this article we examine two mid-18th-century letters from South-America to the Netherlands. Both dealt with what would soon be called the electric eel, and were published in the proceedings of the first Dutch Society of Science (in Haarlem). The discovery of the electric nature of the shocks produced by these fish in particular proved to be a crucial step in understanding animal electricity and the true nature of neurophysiology.

[Familial schwannomatosis, a new entity distinct from neurofibromatosis type 1 and 2]. / Familiaire schwannomatose, een nieuwe entiteit te onderscheiden van neurofibromatose 1 en 2.

A Dutch family was diagnosed with familial schwannomatosis, a disorder that is distinct from neurofibromatosis (NF) type 1 and 2. The proband and 4 relatives had schwannomas on spinal roots, cranial nerves, plexuses, and peripheral nerves; no vestibular schwannomas were found. One of the affected relatives was later diagnosed with intracerebral glioma; schwannomas were not found. None of the living affected relatives had genomic defects affecting the NF2 gene. Large deletions in the proximal region of chromosome 22 were found in all resected schwannomas. Schwannomatosis can occur sporadically or be inherited. Pain is often the clinical manifestation of schwannomas. Resection should be reserved for tumours that are symptomatic or threaten to cause spinal cord compression.

[Two patients with syringomyelia and Charcot's arthropathy]. / Twee patiënten met syringomyelie en een charcot-gewricht.

Two patients, a man aged 45 years and a woman aged 61 years, were diagnosed with syringomyelia. They later developed Charcot's arthropathy of the elbow and shoulder, respectively. The second patient was misdiagnosed with multiple sclerosis during the pre-MRI era. The 3 hallmarks of syringomyelia are impairment of vital or non-vital sensory perception, muscle weakness with atrophy and areflexia of the arms. Syringomyelia often occurs in association with other disorders, such as Chiari's malformation type I or tumours of the spinal column. Diagnosis should include scanning of the entire spinal column and the region surrounding the foramen magnum. Various treatment options exist: watchful waiting is possible or surgery, including decompression of the foramen magnum or placement of a syringosubarachnoidal or syringoperitoneal shunt. In the first patient, the elbow became infected, necessitating surgery. The joint later became non-functional. In the second patient, a conservative approach was followed.

[Diagnostic image (284). A man with Horner's syndrome, ipsilateral headache and dysphasia]. / Diagnose in beeld (284). Een man met het syndroom van Horner, ipsilaterale hoofdpijn en dysfasie.

A 56-year-old man presented with sudden dysphasia and dyspraxia. He had noticed a small left pupil and had suffered from left-sided headache two weeks previously. MRI revealed dissection of the left inner carotid artery.

[From gene to disease; the PABN1 gene and oculopharyngeal muscular dystrophy]. / Van gen naar ziekte; het PABN1-gen en oculofaryngeale spierdystrofie.

Oculopharyngeal muscular dystrophy is a rare disease, presenting with bilateral ptosis and dysphagia, followed by slow progressive muscle weakness. The pathological hallmark of the disease is the presence of intranuclear inclusions in muscle cells. Inheritance is autosomal dominant in almost all cases. The mutation responsible is a short guanine-cytosine-guanine (GCG) expansion in the 'poly adenylate binding nuclear I protein' (PABN1) gene. This expansion is stable in subsequent generations and is translated into a polyalanine tract. The aberrant protein is found within the intranuclear inclusions and interferes with normal mRNA function.

[Diagnostic image (270). A confused and restless woman]. / Diagnose in beeld (270). Een verwarde, onrustige vrouw.

A 63-year-old woman suffering from confusion, restlessness, vertigo and nausea had multiple old cortical and subcortical microhaemorrhages on MR gradient echo imaging, consistent with amyloid angiopathy.

[The practice guideline 'Headache' from the Dutch College of General Practitioners; a response from the perspective of neurology]. / De standaard 'Hoofdpijn' van het Nederlands Huisartsen Genootschap; reactie vanuit de neurologie.

In comparison with the previous version, the Dutch College of General Practitioners' practice guideline 'Headache' has been improved in several respects. The addition of information on cluster headache, tension headache and substance-induced headache may benefit the daily practice of the general practitioner. The list of alarm symptoms is useful for distinguishing between secondary and primary types of headache. With respect to substance-induced headache, there is a difference from the international criteria: according to the latter, the diagnosis may also be made if the headache does not completely disappear but reverts back to the previous pattern. There is a need for studies into the non-medicinal treatment of tension headache. In these practice guidelines, the medicinal treatment of migraine is largely consistent with the guidelines of The Netherlands Society of Neurology.

[The significance of Bernard Brouwer's contribution to Dutch neurology and neurosurgery. On the 125th anniversary of the year of his birth]. / Bij Bernard Brouwers 125e geboortejaar; zijn betekenis voor de Nederlandse neurologie en neurochirurgie.

Following his training under Winkler and carrying out research at the Central Institute for Brain Research in Amsterdam, in 1923 Bernard Brouwer (1881-1949) became the firstprofessor ofneurologyindependent from psychiatry, in the Netherlands. His most important scientific work included comparative anatomical studies of the cerebellum and the projection of retinal fibres on the geniculate body and occipital cortex. He gained international fame and in 1926, during one of his American lecture tours, he was invited to fill the new chair of experimental neurology at Johns Hopkins University. He refused this offer and instead started a new university clinic for neurology in Amsterdam, which included neurosurgery. He did however send a surgeon--Dr. Oljenick--to America to be trained by Cushing. The Amsterdam neurology clinic became an international exchange centre for students of neuroscience. The difficult decisions he had to make during his rectorate in the Second World War, were criticised in 1945. During the last years of his life, he was director of the Central Institute for Brain Research.