J Haan

Leiden University, Leyden, South Holland, Netherlands

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Publications (264)1392.68 Total impact

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    ABSTRACT: Aim: In the revised criteria of the International Classification of Headache Disorders (ICHD-III beta) the following items are added to the diagnostic criteria of cluster headache: ipsilateral sensation of fullness in the ear and ipsilateral forehead/facial flushing. We evaluated the possible additional value of these symptoms for diagnosing cluster headache. Methods: In this cross-sectional cohort study of (potential) cluster headache patients we investigated these additional symptoms using a Web-based questionnaire. Patients not fulfilling the ICHD-II criteria for cluster headache but fulfilling the ICHD-III beta criteria were interviewed. Results: Response rate was 916/1138 (80.5%). Of all 573 patients with cluster headache according to ICHD-II criteria, 192 (33.5%) reported ipsilateral ear fullness and 113 (19.7%) facial flushing during attacks. There was no difference in reporting ipsilateral ear fullness and facial flushing between patients who received a diagnosis of cluster headache and patients who did not. None of the patients who did not fulfill all ICHD-II criteria could be categorized as cluster headache according to the ICHD-III beta criteria. Conclusion: The results of this study do not support the addition of ear fullness and facial flushing to the new ICHD-III beta criteria.
    No preview · Article · Sep 2015 · Cephalalgia
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    ABSTRACT: Trigeminal autonomic cephalalgias (TACs) are primary headache syndromes that share some clinical features such as a trigeminal distribution of the pain and accompanying ipsilateral autonomic symptoms. By definition, no underlying structural lesion for the phenotype is found. There are, however, many descriptions in the literature of patients with structural lesions causing symptoms that are indistinguishable from those of idiopathic TACs. In this article, we review the recent insights in symptomatic TACs by comparing and categorizing newly published cases. We confirm that symptomatic TACs can have typical phenotypes. It is of crucial importance to identify symptomatic TACs, as the underlying cause will influence treatment and outcome. Our update focuses on when a structural lesion should be sought.
    Full-text · Article · Aug 2015 · Current Pain and Headache Reports

  • No preview · Article · Jun 2015

  • No preview · Article · Apr 2015 · Cephalalgia
  • Chapter: Headache
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    ABSTRACT: This patient was a 42-year-old man who had suffered from headache attacks since he was 24 years old. Most of the attacks started with a gray dot in the corner of the right visual field, increasing to a right hemianopsia within 20 min, which quickly disappeared. There never were left-sided visual disturbances. During some of the attacks there were right-sided paresthesias in the arm and face; once his wife noticed slurred speech. Left-sided throbbing headache normally occurred shortly after the disappearance of these symptoms, with some nausea and photophobia and lasting for 1 or 2 days. One or two tablets of paracetamol would sufficiently decrease the intensity of the pain and enable continuance of daily activities. Frequency of the attacks varied from only one per year to once per month. A diagnosis of migraine with aura was made, with no need for further analysis or adjustment of treatment.
    No preview · Chapter · Jan 2015
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    ABSTRACT: Our patient, a 27-year-old amateur soccer player, was hit hard against the head by a ball. Within a few minutes he developed visual and sensory symptoms followed by headache, nausea, vomiting, photophobia and phonophobia. After treatment with paracetamol and metoclopramide, he was free of symptoms within 24 hours. This picture is stereotypically associated with migraine provoked by minor head injury. TTM may also occur without aura. Trauma triggered migraine is seen more frequently in children, adolescents and young adults. The cause of trauma triggered migraine is unknown. Treatment of the headache and nausea with common analgesics and anti-emetic drugs might be effective. Proper explanation and reassurance are most important.
    No preview · Article · Nov 2014 · Nederlands tijdschrift voor geneeskunde
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    ABSTRACT: Background: Cluster headache is a severe neurological disorder with a complex genetic background. A missense single nucleotide polymorphism (rs2653349; p.Ile308Val) in the HCRTR2 gene that encodes the hypocretin receptor 2 is the only genetic factor that is reported to be associated with cluster headache in different studies. However, as there are conflicting results between studies, we re-evaluated its role in cluster headache. Methods: We performed a genetic association analysis for rs2653349 in our large Leiden University Cluster headache Analysis (LUCA) program study population. Systematic selection of the literature yielded three additional studies comprising five study populations, which were included in our meta-analysis. Data were extracted according to predefined criteria. Results: A total of 575 cluster headache patients from our LUCA study and 874 controls were genotyped for HCRTR2 SNP rs2653349 but no significant association with cluster headache was found (odds ratio 0.91 (95% confidence intervals 0.75-1.10), p = 0.319). In contrast, the meta-analysis that included in total 1167 cluster headache cases and 1618 controls from the six study populations, which were part of four different studies, showed association of the single nucleotide polymorphism with cluster headache (random effect odds ratio 0.69 (95% confidence intervals 0.53-0.90), p = 0.006). The association became weaker, as the odds ratio increased to 0.80, when the meta-analysis was repeated without the initial single South European study with the largest effect size. Conclusions: Although we did not find evidence for association of rs2653349 in our LUCA study, which is the largest investigated study population thus far, our meta-analysis provides genetic evidence for a role of HCRTR2 in cluster headache. Regardless, we feel that the association should be interpreted with caution as meta-analyses with individual populations that have limited power have diminished validity.
    No preview · Article · Nov 2014 · Cephalalgia
  • Joost Haan · Peter J Koehler
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    ABSTRACT: There have been many descriptions of presumed 'hysterics' in fiction, many appearing in French literature, but also in a number of other languages. It is clear that contemporary medical ideas and insights about hysteria had a major influence on its depiction in novels. This is particularly true for naturalistic literature, which has been the subject of previous reviews. Here, we focus on a more recent novel: Human Traces by Sebastian Faulks (2005). What is special about the depiction of hysteria in this work is that the presumed 'hysteric' turns out not to be hysteric at all. In the novel, as well as in this chapter, the diagnosis of hysteria is discussed in the light of theories about hysteria of around 1900. For comparison, we present some examples of true 'hysterics' as they occur in fiction. Since it has become clear that severe nonpsychiatric diseases such as an ovarian teratoma can lead to bizarre phenotypes, the association of 'hysteria' with the womb has to be seen in another light. © 2014 S. Karger AG, Basel.
    No preview · Article · Oct 2014 · Frontiers of neurology and neuroscience

  • No preview · Article · Jun 2014 · Neurology
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    ABSTRACT: CADASIL is an autosomal dominant inherited disease, characterized by mid-adult onset of cerebrovascular disease and dementia. CADASIL is caused by mutations in the NOTCH3 gene, which encodes the NOTCH3 protein. Pathogenic mutations in CADASIL are highly distinctive in the sense that they lead to the loss or gain of a cysteine residue in 1 of the 34 EGFr domains of the NOTCH3 protein. The majority are missense mutations, but small deletions, insertions and splice-site mutations have been reported, which typically also lead to a numerical cysteine alteration. Whether numerical cysteine-altering mutations are a rule in CADASIL remains subject of debate, as there are reports suggesting pathogenicity of other types of mutations. However, for most of these the association with CADASIL was later revoked or is questionable. Here, we discuss and provide recommendations for the interpretation of NOTCH3 mutations in the diagnosis of CADASIL.
    No preview · Article · Jun 2014 · Expert Review of Molecular Diagnostics
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    ABSTRACT: Abstract Hereditary cerebral hemorrhage with amyloidosis - Dutch type is an autosomal dominant hereditary disease caused by a point mutation in the amyloid precursor protein gene on chromosome 21. The mutation causes an amino acid substitution at codon 693 (E22Q), the 'Dutch mutation'. Amyloid beta, the product after cleavage of the amyloid precursor protein, is secreted into the extracellular space. The Dutch mutation leads to altered amyloid beta cleavage and secretion, enhanced aggregation properties, higher proteolysis resistance, lowered brain efflux transporter affinity, and enhanced cell surfaces binding. All these result in amyloid beta accumulation in cerebral vessel walls, causing cell death and vessel wall integrity loss, making cerebral vessel walls in hereditary cerebral hemorrhage with amyloidosis-Dutch type more prone to rupture and obstruction, leading to hemorrhages and infarcts. Studying the effects of altered amyloid beta metabolism due to mutations like the 'Dutch' provides us with
    Full-text · Article · May 2014 · Reviews in the neurosciences
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    ABSTRACT: Hemiplegic migraine (HM) and alternating hemiplegia of childhood (AHC) are rare episodic neurological brain disorders with partial clinical and genetic overlap. Recently, ATP1A3 mutations were shown to account for the majority of AHC patients. In addition, a mutation in the SLC2A1 gene was reported in a patient with atypical AHC. We therefore investigated whether mutations in these genes may also be involved in HM. Furthermore, we studied the role of SLC2A1 mutations in a small set of AHC patients without ATP1A3 mutations. We screened 42 HM patients (21 familial and 21 sporadic patients) for ATP1A3 and SLC2A1 mutations. In addition, four typical AHC patients and one atypical patient with overlapping symptoms of both disorders were screened for SLC2A1 mutations. A pathogenic de novo SLC2A1 mutation (p.Gly18Arg) was found in the atypical patient with overlapping symptoms of AHC and hemiplegic migraine. No mutations were found in the HM and the other AHC patients. Screening for a mutation in the SLC2A1 gene should be considered in patients with a complex phenotype with overlapping symptoms of hemiplegic migraine and AHC.
    No preview · Article · May 2014 · Cephalalgia
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    ABSTRACT: Familial hemiplegic migraine (FHM) is a rare monogenic subtype of migraine with aura, characterized by motor auras. The majority of FHM families have mutations in the CACNA1A and ATP1A2 genes; less than 5% of FHM families are explained by mutations in the SCN1A gene. Here we screened two Spanish FHM families for mutations in the FHM genes. We assessed the clinical features of both FHM families and performed direct sequencing of all coding exons (and adjacent sequences) of the CACNA1A , ATP1A2 , PRRT2 and SCN1A genes. FHM patients in both families had pure hemiplegic migraine with highly variable severity and frequency of attacks. We identified a novel SCN1A missense mutation p.Ile1498Met in all three tested hemiplegic migraine patients of one family. In the other family, novel SCN1A missense mutation p.Phe1661Leu was identified in six out of eight tested hemiplegic migraine patients. Both mutations affect amino acid residues that either reside in an important functional domain (in the case of Ile(1498)) or are known to be important for kinetic properties of the NaV1.1 channel (in the case of Phe(1661)). We identified two mutations in families with FHM. SCN1A mutations are an infrequent but important cause of FHM. Genetic testing is indicated in families when no mutations are found in other FHM genes.
    No preview · Article · Apr 2014 · Cephalalgia
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    ABSTRACT: Familial hemiplegic migraine (FHM) is a rare monogenic subtype of migraine with aura that includes motor auras. Prophylactic treatment of FHM often has marginal effects and involves a trial-and-error strategy based on therapeutic guidelines for non-hemiplegic migraine and on case reports in FHM. We assessed the response to prophylactic medication in an FHM family and sequenced the FHM2 ATP1A2 gene in all available relatives. A novel p.Met731Val ATP1A2 mutation was identified. Attack frequency was reduced significantly with sodium valproate monotherapy ( N = 1) and attacks ceased completely with a combination of sodium valproate and lamotrigine ( N = 2). We report dramatic prophylactic effects of sodium valproate and lamotrigine in an FHM2 family, making these drugs worth considering in the treatment of other FHM patients.
    Full-text · Article · Jan 2014 · Cephalalgia
  • Joost Haan
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    ABSTRACT: Protagonists who are locked-in can be found throughout fiction, probably because being locked-in serves as a strong philosophical metaphor for human existence. In this chapter, three protagonists who are locked-in due to physical/medical reasons will be described. The fictitious stories of Noirtier from The Count of Monte Cristo by Alexandre Dumas and that of Madame Raquin from Thérèse Raquin by Émile Zola are followed by the real-life story of Jean-Dominique Bauby's The Diving Bell and the Butterfly. Dumas' Noirtier is considered the first description of the locked-in syndrome in literature, and Madame Raquin appears to be locked-in also, but her description leaves many questions. Bauby's autopathography is chosen from many several similar discourses as it contains cross-references to Noirtier, Dumas' prototype of the syndrome, but also because it is of high literary value. The similarities and remarkable differences between these three case reports of this existentialistically important state of being will be highlighted, with an emphasis on the focus of their narratives.
    No preview · Article · Dec 2013 · Progress in brain research
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    ABSTRACT: Headache and epilepsy often co-occur. Epidemiologic studies conducted in the past few years reinforce the notion of a bi-directional association between migraine and epilepsy. Data on an association between headache (in general) and epilepsy, however, are less clear. Peri-ictal headache often presents with migraine-like symptoms and can be severe. A correct diagnosis and management are paramount. It was demonstrated that cortical hyperexcitability may underlie both epilepsy and migraine. A recent study linked spreading depolarisation, the supposed underlying pathophysiological mechanism of migraine with aura, to epilepsy. Although this study was carried out in patients who had suffered a subarachnoid haemorrhage, the finding may shed light on pathophysiological mechanisms common to epilepsy and migraine.
    Full-text · Article · Aug 2013 · Current Pain and Headache Reports

  • No preview · Article · Jul 2013 · Journal of Neurology

  • No preview · Conference Paper · Jun 2013
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    ABSTRACT: Background: About 10% of cluster headache patients have the chronic form. At least 10% of this chronic group is intractable to or cannot tolerate medical treatment. Open pilot studies suggest that occipital nerve stimulation (ONS) might offer effective prevention in these patients. Controlled neuromodulation studies in treatments inducing paraesthesias have a general problem in blinding. We have introduced a new design in pain neuromodulation by which we think we can overcome this problem. Methods/design: We propose a prospective, randomised, double-blind, parallel-group international clinical study in medically intractable, chronic cluster headache patients of high- versus low-amplitude ONS. Primary outcome measure is the mean number of attacks over the last four weeks. After a study period of six months there is an open extension phase of six months. Alongside the randomised trial an economic evaluation study is performed. Discussion: The ICON study will show if ONS is an effective preventive therapy for patients suffering medically intractable chronic cluster headache and if there is a difference between high- and low-amplitude stimulation. The innovative design of the study will, for the first time, assess efficacy of ONS in a blinded way.
    Full-text · Article · May 2013 · Cephalalgia
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    ABSTRACT: To determine the prevalence and nature of trigeminal neuralgia in a large group of cluster headache patients. Cluster-tic syndrome is a rare headache syndrome in which trigeminal neuralgia and cluster headache co-occur. The existence of cluster-tic syndrome as a separate entity is questioned, and figures on prevalence of simultaneous existence of cluster headache and trigeminal neuralgia are not available. As part of a nationwide study on headache mechanisms in cluster headache (Leiden University Medical Centre Cluster headache Neuro Analysis programme), we collected clinical data of 244 cluster headache patients using a semistructured telephone interview in a cross-sectional design. In 11 (4.5%) cluster headache patients, attacks fulfilling International Headache Society criteria for trigeminal neuralgia were also present. In all cases, trigeminal neuralgia occurred ipsilateral to cluster headache and in the majority (82%) in the ophthalmic branch. In 8 of these 11 patients (73%), the frequency and time pattern of trigeminal neuralgia seemed to parallel cluster headache and was likely a part of the cluster headache spectrum. In the 3 remaining patients, cluster headache and trigeminal neuralgia were unrelated in time and appeared to occur independently. Trigeminal neuralgia co-occurred in 11/244 (4.5%) of cluster headache patients. In only 3 (1.2%) patients, trigeminal neuralgia seemed to occur independently from cluster headache episodes. Trigeminal neuralgia (-like) attacks in cluster headache patients are most of the time part of the cluster headache spectrum and should then probably not be treated separately. A shared underlying pathophysiological mechanism of cluster headache and trigeminal neuralgia is not supported by this study.
    No preview · Article · May 2013 · Headache The Journal of Head and Face Pain

Publication Stats

9k Citations
1,392.68 Total Impact Points


  • 1988-2015
    • Leiden University
      Leyden, South Holland, Netherlands
  • 1994-2014
    • Rijnland Hospital, Leiderdorp
      Лейдердорпе, South Holland, Netherlands
  • 1988-2013
    • Leiden University Medical Centre
      • Department of Neurology
      Leyden, South Holland, Netherlands
  • 2009
    • HagaZiekenhuis van Den Haag
      's-Gravenhage, South Holland, Netherlands
  • 2008
    • Erasmus MC
      Rotterdam, South Holland, Netherlands
  • 1992
    • Academic Medical Center (AMC)
      Amsterdamo, North Holland, Netherlands
  • 1991
    • Medisch Centrum Leeuwarden
      Leewarden, Friesland, Netherlands