[Significance of health-related quality of life and religiosity for the acceptance of chronic pain]. / Bedeutung der gesundheitsbezogenen Lebensqualität und Religiosität für die Akzeptanz von chronischen Schmerzen.

BACKGROUND: Modern medical analgesia is based on a bio-psycho-social model of disease. From this bio-psycho-social perspective it seems essential to include religiosity in the multidimensional and interdisciplinary assessment of pain patients. MATERIAL AND METHODS: A total of 450 consecutively referred in- and outpatients to a neurological department completed an epidemiologic pain questionnaire. This patient self-administered questionnaire included diagnostic screening tests for anxiety and depression, a generic health-related quality of life measure and sociodemographic questions. Pain severity grades and pain chronicity stages were measured. The acceptance of chronic pain was assessed with the chronic pain acceptance questionnaire. The significance of religiosity was measured employing the structure of religiosity test. RESULTS: Of the neurological patients 82% complained of having had pain within the past 3 months and 79% within the last 12 months. Patients who accepted the pain and pursued their daily activities despite the pain were less depressive and anxious and showed an enhanced health-related quality of life. The importance of religion to the pain patients was associated with a higher level of pain tolerance. CONCLUSIONS: This study proved that the significance of religiosity to the patient is related to psychic distress and health-related quality of life and at the same time may play an important role in the bio-psycho-social pain concept.

Treatment of symptomatic heterozygous aceruloplasminemia with oral zinc sulphate.

Aceruloplasminemia is an autosomal recessive and phenotypically primarily neurodegenerative disease caused by a homozygous mutation of the ceruloplasmin gene. The absence of ceruloplasmin and its ferroxidase activity leads to pathological iron overload in the brain and other organs. While heterozygous carriers of ceruloplasmin gene mutations have been believed to be asymptomatic, a number of cases with neurological deficits have recently been described. To date, an effective treatment has not been established for either aceruloplasminemia or symptomatic heterozygous aceruloplasminemia. The present report concerns the beneficial treatment of an 18-year-old girl with extrapyramidal and cerebellar-mediated movement disorder caused by a heterozygous mutation of the ceruloplasmin gene using oral zinc sulphate.

[Wound botulism in heroin addicts in Germany]. / Wundbotulismus bei Heroinabhängigen in Deutschland.

HISTORY AND ADMISSION FINDINGS: 5 heroin addicts (aged 31-44 years; 1 female, 4 men) presented with a history of blurred vision and diplopia followed by dysarthria. 3 of the patients also developed respiratory failure requiring long-term ventilatory support. Physical examination revealed cranial nerve deficits and abscesses at injection sites in 3 of them. DIAGNOSIS: In 4 patients wound botulism was diagnosed on the basis of symptoms, course of the illness and response to specific treatment. Clostridium botulinum was grown from wound swab in one patient. TREATMENT AND COURSE: Two of the patients, having been injected with antitoxin immediately after admission, were discharged almost symptom-free after only a few days. Adjuvant antibiotics and, in 3 patients, surgical débridement of the abscesses were needed. CONCLUSIONS: Progressive cranial nerve pareses in addicts who inject drugs intravenously or intramuscularly should raise the suspicion of wound botulism and require hospitalization. While indirect demonstration of toxin supports the diagnosis, false-negative results are common.

MRI and ultrasonographic imaging of a patient with carotidynia.

A patient presented with a painful, pulsating nodosity with marked tenderness on the right side of her neck. Cervical magnetic resonance imaging (MRI) exhibited pathological signal changes in the perivascular tissue of the carotid bifurcation area and around the external carotid artery. Similarly, Doppler sonography revealed an echo-poor wall change with outward bulging and slight narrowing of the affected vessel lumen. The clinical symptoms and our findings led to the diagnosis of carotidynia. We proposed that cervical MRI and Doppler sonography could be used for diagnosing carotidynia.

[Long-lasting, refractory headache after bilateral dissection of the internal carotid artery]. / Anhaltender, therapierefraktärer Kopfschmerz nach beidseitiger Dissektion der A. carotis interna.

Headache is usually the first and the most frequent symptom of cervicocephalic arterial dissection. Besides headache ipsilateral Horner's syndrome, cranial nerve palsies, or pulsatile tinnitus are observed. Serious complications of arterial dissection are ischemic strokes, which mostly occur later in the course of time. We report on a patient with spontaneous bilateral carotid artery dissection, who suffered from atypically prolonged, severe, and refractory headache. High-dose administration of nonsteroidal anti-inflammatory drugs as well as opioids were ineffective. Pain relief was only achieved after a 3-day course of corticosteroids (100 mg methylprednisolone/day).

[Remission of atypical and refractory cluster headache after topiramate administration]. / Behandlung eines atypischen und therapierefraktären Clusterkopfschmerzes mit Topiramat.

Cluster headache is the third most frequent type of primary cephalgia only exceeded in frequency by tension-type headache and migraine. Cluster headache is characterized by periodic attacks of unilateral excruciating pain and accompanying autonomic features. It is arguably the most disabling form of primary headache. Therefore, prompt recognition of this disorder is necessary to provide an opportunity for effective treatment. We report on a patient with cluster headache, who complained about an atypical interval of dull headache between the cluster attacks, thus excluding the differential diagnosis of hemicrania continua. The common drugs for acute cluster attacks proved to be ineffective as well as prophylactic treatment with steroids and verapamil. Only administration of topiramate led to relief of pain.

[Hypertrophic cranial pachymeningitis as a rare cause of headache]. / Hypertrophe kraniale Pachymeningitis als seltene Ursache für Kopfschmerzen.

Hypertrophic cranial pachymeningitis is an uncommon, fibrosing, inflammatory process that involves the dura mater. The condition is being reported more frequently owing to the use of cranial MRI. The main clinical feature is headache, whereas cranial nerve lesions, cerebellar symptoms, and epileptic seizures occur more rarely. A variety of autoimmune and infectious diseases can result in this condition, which is labeled as idiopathic in the absence of any definite inciting factor. The diagnosis of hypertrophic cranial pachymeningitis is based on neuroimaging of thickened and enhancing dura mater. It can be defined pathologically on biopsy. A specific treatment is indicated in some cases of secondary hypertrophic cranial pachymeningitis. Mostly, treatment relies on corticosteroids and immunosuppressive agents. This review summarizes the current knowledge on causes, clinical presentation, diagnosis, and treatment of this disorder.

Bilateral subdural haematomata and lumbar pseudomeningocele due to a chronic leakage of liquor cerebrospinalis after a lumbar discectomy with the application of ADCON-L gel.

The anti-adhesion gel ADCON-L has been available since the end of the 1990s. During disc surgery it can be applied to the spinal nerve roots and the dura mater spinalis in order to inhibit fibroblast migration and thus avoid postoperative adhesions or excessive keloids, respectively. Due to the way ADCON-L works, inadvertent, intraoperational dural lesions may stay open much longer than usual because ADCON-L inhibits the natural healing process. Possible consequences are a chronic leakage of cerebrospinal fluid in combination with intracranial hypotension syndrome. We report on a patient who underwent lumbar disc surgery with application of ADCON-L gel. Postoperatively he suffered from acute headache, nausea, and vomiting. A lumbar pseudomeningocele was demonstrated on magnetic resonance imaging (MRI). Furthermore, cranial MRI revealed bilateral, chronic subdural haematomata which indicated intracranial hypotension syndrome or continuous leakage of cerebrospinal fluid at the lumbar site. With conservative treatment the problems were gradually reduced and eventually the subdural haematomata were no longer detected. The pseudomeningocele persisted over a 4 month period of observation. Because of the complications we found, the local application of ADCON-L during lumbar disc surgery should be critically evaluated.

[Trigeminal autonomic headache, hemicrania continua and hypnic headache. A review of rare primary headache forms]. / Trigeminoautonome Kopfschmerzen, Hemicrania continua und Hypnic Headache. Ein Uberblick über die seltenen primären Kopfschmerzformen.

Tension-type headache and migraine are the most common types of primary headaches. Apart from these well known diseases, the group of primary headaches includes other relatively rare headache disorders. Some of these seldom syndromes have been described for the first time within the last twenty years and have been newly included in the revised IHS classification from 2004. Their typical symptomatic is less known, but offers an excellent opportunity to diagnose these syndromes. The importance of recognising these disorders is underlined by the fact, that rare primary headaches response often complete and rapid to a specific treatment. This review summarizes the current knowledge on the clinical presentation and treatment of cluster headache, paroxysmal hemicrania, SUNCT syndrome, hemicrania continua and hypnic headache.

[Iatrogenic Wernicke-Korsakow syndrome with unusual neurological deficits and MRI lesions]. / Iatrogenes Wernicke-Korsakoff-Syndrom mit ungewöhnlichen Symptomen und MRT-Veränderungen.

Wernicke-Korsakow Syndrome (WKS) is caused by thiamine (vitamin B1) deficiency and usually occurs in conjunction with chronic alcohol abuse. Our report concerns a 64-year-old, nonalcoholic, woman with no history of alcohol abuse, who became ill with WKS after 3 weeks of parenteral nourishment. As an unusual initial symptom she went blind in both eyes; this was followed a few days later by impaired consciousness and spastic tetraparesis. A cranial MRI examination showed symmetrical signal alteration (T2, FLAIR and diffusion weighting) in the medial thalamus, periaqueductal mesencephalon including the quadrigeminal plate, mamillary bodies and-most unusually-both paracentral gyri. Laboratory tests confirmed the diagnosis of WKS as significant thiamine deficiency was detected. Following several weeks of intravenous thiamine supplementation the MRI lesions were almost completely reversed but the neurological deficits regressed only partially.

Rupture of an internal carotid artery aneurysm during angiography with leakage of contrast medium via an external ventricular drain.

With a ruptured intracranial aneurysm producing subarachnoid haemorrhage (SAH) cerebral angiography is currently used for identification of the affected vessel. Aneurysm rerupturing is one of the more serious complications of cerebral angiography and has been frequently described. We report a 61-year-old man who presented with SAH who had rerupture of a large aneurysm of the internal carotid artery during angiography. A substantial amount of contrast medium escaped via a ventricular drain. The three main risk factors for rerupture of an aneurysm are: angiography performed within 6 h of the primary SAH, an aneurysm on the internal carotid artery and an unfavourable Glasgow coma score.

Is there a final common pathway in mitochondrial encephalomyopathies? Considerations based on an autopsy case of Kearns-Sayre syndrome.

A case of Kearns-Sayre syndrome (KSS) diagnosed 18 years prior to death due to stroke and heart failure with postnatal onset was followed over 15 years and confirmed by postmortem examination. Within the brain, an old cystic infarction of the left hemisphere was found. Other features included white matter gliosis and cerebellar atrophy. Equal or similar features were observed in other conditions thought to be due to failure of mitochondrial metabolism, therefore, a common evolution of neuropathological changes must be discussed.

[Decompressive craniectomy for severe intracranial hypertension due to cerebral infarction or meningoencephalitis]. / Kraniektomie bei Hirndrucksteigerung durch Hirninfarkte und Meningoenzephalitis.

We describe the clinical course and outcome following decompressive craniectomy in six patients. Five patients suffered from severe intracranial hypertension due to middle cerebral artery infarction. In one patient the cause was bacterial meningoencephalitis. Acute clinical and neuroradiological signs of intracranial hypertension were seen in all cases. Following ineffective conventional brain edema therapy, decompressive craniectomy was undertaken. In five cases intracranial pressure was sufficiently lowered. One patient developed transtentorial herniation with subsequent brain death. Four patients with middle artery infarction showed moderate neurological disorders and one patient with bacterial meningoencephalitis recovered completely after treatment. Craniectomy in malignant middle artery infarction should be taken into consideration if conventional brain edema therapy does not sufficiently reduce critically raised intracranial pressure. Craniectomy provides development of brain herniation. This treatment may reduce high lethality rate and high frequency of severe neurological disorders.

Treatment of acute ischemic stroke with the low-molecular-weight heparin certoparin: results of the TOPAS trial. Therapy of Patients With Acute Stroke (TOPAS) Investigators.

BACKGROUND AND PURPOSE: To study the safety and efficacy of the low-molecular-weight heparin certoparin, we performed a randomized, double-blind, dose-finding multicenter trial in patients with acute ischemic stroke (Therapy of Patients With Acute Stroke [TOPAS]). METHODS: We randomized 404 patients to 4 treatment groups within 12 hours of stroke onset: 3000 U anti-factor Xa (aXa) certoparin once daily (treatment group 1); 3000 U aXa twice daily (group 2); 5000 U aXa twice daily (group 3); and 8000 U aXa twice daily (group 4). The primary efficacy variable was the proportion of patients reaching a favorable functional outcome (Barthel Index >/=90 points) at 3 months. CT was performed at trial entry, after 7 days, and on clinical deterioration. RESULTS: The proportion of patients with Barthel Index >/=90 was not different between treatment arms (61.5%, 60.8%, 63.3%, and 56.3% in the 4 groups, respectively; intent-to-treat population). European Stroke Scale scores improved in all treatment groups within the first 14 days to a similar extent. During the follow-up of 6 months, percentages of patients with recurrent stroke/transient ischemic attack were 11.0%, 5.9%, 9.7%, and 13.0% in the 4 groups, respectively. Overall mortality was only 7.4%. Two parenchymal cerebral hematomas and 1 extracranial bleeding episode occurred in treatment group 1 versus 1 and 0 in group 2, 2 and 0 in group 3, and 4 and 5 in group 4, respectively. During certoparin treatment, 1 deep vein thrombosis but no pulmonary embolism was observed. CONCLUSIONS: Dose increase of certoparin up to 8000 U aXa twice daily did not improve the functional outcome of patients with ischemic stroke. Severe bleeding tended to be more frequent in the highest dose group only.

MR imaging of acute intermittent porphyria mimicking reversible posterior leukoencephalopathy syndrome.

Reversible posterior leukoencephalopathy syndrome (PLS) is characterized by headache, altered mental function, visual disturbances and seizures. Neuroimaging studies suggest a white-matter oedema, predominantly in the posterior parietal-temporal-occipital regions of the brain. We present the case of a 30-year-old woman who had suffered her first attack of acute intermittent porphyria (AIP). Following 1 week of abdominal pain she developed several generalized seizures, and hallucinations, and exhibited a progressive deterioration of the consciousness. T2-weighted images, especially fluid-attenuated inversion recovery (FLAIR) sequences showed bilateral lesions in the posterior frontal, parietal and occipital cortex and subcortical white matter. Following treatment with haematin and a high carbohydrate diet the patient's condition improved. Follow-up magnetic resonance imaging (MRI) revealed complete resolution of the lesions. To our knowledge, this is the first report concerning a completely reversible PLS in AIP.

MRI of acute cerebral infarcts, increased contrast enhancement with continuous infusion of gadolinium.

We compared contrast enhancement on T1-weighted MRI of acute cerebral infarcts after conventional bolus administration and continuous infusion of gadolinium. We examined 12 patients with a history of acute stroke with contrast-enhanced MRI once a week for a 1 month. Only ischaemic lesions were investigated after cerebral haemorrhage had been excluded by CT. Each MRI study included T2- and proton density-weighted sequences for determination of the size and site of the infarct, immediate postinjection T1-weighted imaging after bolus administration of 0.1 mmol/kg gadolinium-DPTA and delayed T1-weighted imaging after additional continuous infusion of 0.1 mmol/kg over 2 h. A total of 42 MRI studies was performed. In the first week after the onset of stroke, most infarcts (8 of 10) did not enhance after bolus administration, whereas all showed distinct contrast enhancement after the infusion. In the following weeks all but two infarcts showed contrast enhancement after bolus administration; after continuous infusion contrast enhancement could be seen in all cases. While contrast enhancement after bolus administration showed the typical gyriform pattern, enhanced areas were more extensive after the infusion and usually covered the entire infarcted area shown on T2- and proton density-weighted images. We presume that the disturbed blood-brain barrier in ischaemic areas favours delivery of contrast medium to the infarcted tissue if it is offered continuously so that a steady state can develop.

Intracerebral haemorrhage from a venous angioma following thrombosis of a draining vein.

We report a case of putaminal haemorrhage from a venous cerebral angioma following thrombosis in a draining vein. The patient complained of numbness of the right side, followed by headache and aphasia. CT demonstrated an intracerebral haemorrhage due to thrombotic occlusion of a draining vein of the venous angioma. Carotid angiography showed the venous angioma. MRI studies confirmed the diagnosis and demonstrated recanalisation of the draining vein following therapy with phenprocoumon.