Case report of a woman with anti amphiphysin positive stiff person syndrome.

Stiff person syndrome is a rare neuroimmunological disease, characterized by severe, involuntary stiffness with superimposed painful muscle spasms, which are worsened by external stimuli. The classical form is associated with high levels of antibodies against glutamic acid decarboxylase. One of the variant forms is associated with antibodies against amphiphysin. This entity is a paraneoplastic syndrome, caused primarily by breast cancer, secondarily by lung cancer. Symptomatic therapy of anti amphiphysin positive stiff person syndrome includes treatment with benzodiazepines and baclofen (including intrathecal baclofen therapy). The effect of immunological therapies is controversial. Treatment of the underlying cancer may be very effective. In this report, we describe a 68 year old female presenting with an unusally rapidly developing anti amphiphysin positive stiff person syndrome, which was associated with breast cancer. Her painful spasms abolished after intrathecal baclofen treatment was initiated. Her condition improved spontaneously and significantly after cancer treatment, which enabled to start her complex rehabilitation and the simultaneous dose reduction of the intrathecal baclofen. The bedridden patient improved to using a rollator walker and the baclofen pump could be removed 18 monthes after breast surgery. This highlights the importance of cancer screening and treatment in anti amphiphysin positive stiff person syndrome cases.

The comprehensive headache-related quality of life questionnaire shows significant improvement after withdrawal treatment in medication overuse headache: a pilot study.

BACKGROUND AND PURPOSE: Medication overuse headache (MOH) is a common form of disabling headache presenting in as much as 30% of the patients seen in headache subspecialty practice. Quality of life (QOL) is frequently used as a secondary endpoint in headache trials. In MOH, previous trials of QOL focused mostly on generic QOL. We report the results of a pilot study that examined the feasibility of using a new QOL questionnaire, the 23-item Comprehensive Headache-related Quality of life Questionnaire (CHQQ), as an indicator of treatment response in MOH. PATIENTS AND METHODS: Fifteen patients (13 women and two men; mean age: 39.7 +/- 12.5 years) suffering from MOH were enrolled in a complex treatment programme consisting of acute medication withdrawal, preventive pharmacological treatment, structured advice and lifestyle intervention. The clinical data were collected using a detailed headache diary. CHQQ was completed before and after the treatment programme. RESULTS: MOH patients had low QOL values at baseline which was comparable to the QOL of episodic migraine patients. The treatment programme resulted in significant reductions of the number of headache days and attacks, headache severity and analgesic consumption. The dimensions and total score of CHQQ showed a significant increase after the treatment period. Seventeen of CHQQ's 23 individual items also improved significantly. CONCLUSION: In this study the new headache-specific quality of life instrument CHQQ was able to demonstrate significant improvements after adequate treatment of MOH. This result indicates that the CHQQ may be an adequate tool for assessing quality of life in headache treatment trials.

[Prospective study of the clinical features of cluster headache]. / A cluster fejfájás klinikumának prospektív vizsgálata.

INTRODUCTION: Although cluster headache (CH) is one of the most severe human pain syndromes, its symptoms and therapeutic possibilities may be suboptimally recognised in current medical practice in Hungary. AIM: To present the clinical characteristics of CH based on a prospective study of patients attending the Headache Service of the Department of Neurology, Semmelweis University. METHODS: We collected information about the symptoms, diagnosis and previous treatment of CH patients by filling in a 108-item questionnaire during outpatient visits. RESULTS: In the 5-year period between 2004 and 2008 we obtained data from 78 CH patients (57 males and 21 females; mean age: 44.6 +/- 14.6 years). The male:female ratio did not change in subgroups based on disease onset (calendar years). Ninety-three percent considered CH the most severe pain state of their life. The pain was strictly unilateral, affecting the territory of the 1st trigeminal division in all patients. The attacks were accompanied by signs of ipsilateral cranial parasympathetic activation (lactimation 83%, conjunctival injection 67%, rhinorrhea 56%, nasal congestion 43%); less frequently, signs of sympathetic dysfunction (ptosis 48%, miosis 7%) were also present. Two patients had attacks showing the typical localisation, severity and time course of CH attacks, but not accompanied by autonomic phenomena. A considerable part of the patients also observed symptoms that are usually ascribed to migraine (nausea 41%, vomiting 18%, photophobia 68%, phonophobia 58%). This may have been instrumental in the fact that, regardless of the characteristic clinical symptoms, the diagnosis of CH took 10 years on average. At the time of their examination 63% of patients were not using adequate abortive medications and 59% did not have an adequate prophylactic measure. DISCUSSION: Cluster headache is characterised by attacks of devastating pain that warrant an early diagnosis and adequate treatment. Our study underlines that information about the diagnosis and therapy of CH should be emphasized on occasions of neurology specialty training and continuing medical education.

The validation of a new comprehensive headache-specific quality of life questionnaire.

BACKGROUND: Measuring quality of life (QOL) is an important means of assessing the impact of headache. The currently used QOL questionnaires are usually geared toward migraine and focus on a limited number of factors, thus they are not necessarily informative in other headache types. We report the psychometric properties of a new questionnaire, the Comprehensive Headache-related Quality of life Questionnaire (CHQQ) that may be more sensitive to the burden of headache. PATIENTS AND METHODS: A total of 202 patients suffering from migraine (n = 168) or tension-type headache (TTH) (n = 34) completed the CHQQ and SF-36, a generic QOL questionnaire. We assessed the reliability and validity of the CHQQ and its physical, mental and social dimensions. RESULTS: The questionnaire was easy to administer. Reliability was excellent with Cronbach's alpha being 0.913 for the whole instrument (0.814-0.832 for its dimensions). The dimensions and total score showed significant correlations with the patients' headache characteristics (criterion validity), and were also significantly correlated with the SF-36 domains (convergent validity). The total score and dimensions were significantly (p < 0.005) lower in the migraine group than in the TTH group (discriminative validity). CONCLUSION: In this study the new headache-specific QOL instrument showed adequate psychometric properties.

[Examining the psychometric properties of a new quality of life questionnaire in migraineurs]. / Uj életminoség-kérdoív pszichometriai tulajdonságainak vizsgálata migrénes betegek esetében.

BACKGROUND: The deleterious effect of primary headaches on the sufferers' quality of life (QOL) has been abundantly documented using both generic and headache-specific instruments. The currently used questionnaires focus on a limited number of factors and therefore may not be sensitive enough to detect the effect of headache type and headache characteristics on QOL, despite the obvious clinical differences. We have devised a comprehensive questionnaire that may be more sensitive to the burden of headache. OBJECTIVE: To assess the psychometric properties of the new questionnaire on a group of migraineurs. PATIENTS AND METHOD: We studied 117 migraineurs who completed the validated Hungarian version of the SF-36 generic QOL measure and our new, 25-item questionnaire. Reliability was assessed by internal consistency, measured by Cronbach's alpha of all items. Content validity was examined by calculating the correlation of the items with subscales of the SF-36 measure. The correlation of the patients' migraine characteristics with the questionnaire's items was used to assess criterion validity. RESULTS: The questionnaire was quick and easy to administer. The questionnaire demonstrated good reliability, with Cronbach's alpha being 0.893. Content validity was adequate; most "physical" items of the new questionnaire showed significant correlations with the bodily pain and role physical SF-36 subscales and most "psychical" and "social" items were correlated with mental health and social functioning SF-36 subscales. Criterion validity was adequate, with headache severity being correlated with most of the items. DISCUSSION: In this study the new headache-specific quality of life instrument showed adequate psychometric properties.

Trigemino-autonomic headache related to Gasperini syndrome.

We report the association of ipsilateral trigemino-autonomic headache to a case of right-sided nuclear facial and abducens palsy (Gasperini syndrome), ipsilateral hypacusis and right hemiataxia, caused by the occlusion of the right anterior inferior cerebellar artery. Short-lasting attacks of mild to moderate ipsilateral fronto-periorbital head pain, accompanied by lacrimation and mild conjunctival injection during more severe attacks, were present from the onset of symptoms, with a gradual worsening over the next few months and remitting during naproxen therapy. Magnetic resonance imaging showed an infarct in the right cerebellar peduncle, extending toward the pontine tegmentum, also involving the ipsilateral spinal trigeminal nucleus and tract and the trigeminal entry zone. Gasperini syndrome may be accompanied by ipsilateral trigemino-autonomic head pain.