[Integration of Palliative and Hospice Care Structures in the Care of Neurological Patients: Opinion of Chief Consultant Neurologists in Germany]. / Integration von Palliativ- und Hospizstrukturen in die Versorgung neurologischer Patienten ­ Meinungsbild neurologischer Chefärzte in Deutschland.

An online survey of chief neurological consultants was conducted to find out how they judged the demand for and access to palliative and hospice care (PHC) structures for their neurological patients (NP) as well as their collaboration with PHC structures. 110 of 881 chief consultants who were contacted participated in the survey. About 10 % of their NP were considered suitable for PHC. They estimated that 9 % of the deceased had died from their underlying neurological disease. The integration of PHC structures into the management of various neurological diseases was considered worthwhile but 51.9 - 78.5 % indicated that there was no or little collaboration with PHC structures. 12 % of the participants were trained in palliative care (PC). To guarantee an adequate PHC also for NP, PHC knowledge should be integrated into the curriculum and training of neurologists and the PC community should further extend its services to NP, adapting their knowledge and structures to these patients' special needs.

Intrathecal Baclofen therapy in Germany: Proceedings of the IAB-Interdisciplinary Working Group for Movement Disorders Consensus Meeting.

Continuous intrathecal Baclofen application (ITB) through an intracorporeal pump system is widely used in adults and children with spasticity of spinal and supraspinal origin. Currently, about 1200 new ITB pump systems are implanted in Germany each year. ITB is based on an interdisciplinary approach with neurologists, rehabilitation specialists, paediatricians and neurosurgeons. We are presenting the proceedings of a consensus meeting organised by IAB-Interdisciplinary Working Group for Movement Disorders. The ITB pump system consists of the implantable pump with its drug reservoir, the refill port, an additional side port and a flexible catheter. Non-programmable pumps drive the Baclofen flow by the reservoir pressure. Programmable pumps additionally contain a radiofrequency control unit, an electrical pump and a battery. They have major advantages during the dose-finding phase. ITB doses vary widely between 10 and 2000 µg/day. For spinal spasticity, they are typically in the order of 100-300 µg/day. Hereditary spastic paraplegia seems to require particularly low doses, while dystonia and brain injury require particularly high ones. Best effects are documented for tonic paraspasticity of spinal origin and the least effects for phasic muscle hyperactivity disorders of supraspinal origin. Oral antispastics are mainly effective in mild spasticity. Botulinum toxin is most effective in focal spasticity. Myotomies and denervation operations are restricted to selected cases of focal spasticity. Due to its wide-spread distribution within the cerebrospinal fluid, ITB can tackle wide-spread and severe spasticity.

Asymptomatic carriage of Klebsiella pneumoniae producing extended-spectrum beta-lactamase by patients in a neurological early rehabilitation unit: management of an outbreak.

During 11 months 58 extended-spectrum beta-lactamase-producing Klebsiella pneumoniae (ESBL-Kp) isolates were grown from 10 patients on a neurological early rehabilitation unit. The patients had no signs of infection but were colonized in the nose and trachea, and unusually only one had colonization in the gut. A single clone of ESBL-Kp was identified by pulse field gel electrophoresis. Strong hygienic precautions similar to those for Methicillin-resistant Staphylococcus aureus patients prevented spread of the bacteria to other wards. However, rehabilitation for patients with severe neurological failures made it very difficult to follow hygienic requirements. Disinfection of mucous membranes was difficult. Eventually the application of a nasal spray containing povidone-iodine proved to be successful.

Prilocaine elimination by isolated perfused rat lung and liver.

Prilocaine is assumed to undergo significant elimination by extrahepatic organs and to differ in this respect from other commonly used local anaesthetics. In order to clarify whether the lung may play an important role as a site of elimination of prilocaine, the kinetic parameters were studied in isolated perfused rat lungs and were compared to those of isolated livers. Furthermore, the structurally related compounds bupivacaine and mepivacaine were also investigated in this system. Prilocaine was dispersed into a relatively large apparent distribution volume in perfused rat lung (139 ml versus 97 ml in controls). In single-pass perfused lungs the observed maximum of concentration was decreased by about 60% compared to controls. The mean residence time was prolonged by about 40%. These observations suggest that prilocaine is substantially retained by rat lung and that this effect occurs particularly during first-pass. However, the ability of rat lung to degrade prilocaine was relatively low. The clearance values were about 0.3 ml/min equal to about 20% of the hepatic capacity calculated per g of tissue. Thus it must be assumed that prilocaine is only transiently retained by the lung and will gain systemic availability later on. In rat lungs the kinetics of prilocaine elimination were not substantially different from those of bupivacaine and mepivacaine (16 and 12%). These observations do not support the assumption that especially prilocaine undergoes extrahepatic elimination.

[Neglect dyslexia owing to traumatic fronto-temporal right hemisphere bleeding]. / Neglektdyslexie nach traumatischer frontotemporaler Blutung rechts.

Apart from unilateral disturbance of the egocentric reference system, as it is traditionally known of neglect, there are also cases with unilateral disturbances of a stimulus-centered or object-centered reference system. In these cases the spatial position of the object, from the observer's perspective, plays only a limited role in the explanation of the problems with object perception. We describe a patient who, following head-brain trauma and a fronto-temporal bleeding, showed, beside traditional neglect, also a stimulus-centered left-sided disorder in word and object perception. Our investigations showed that this disorder (a) is independent of the position of the object in the environmental space, (b) comprises words and objects, and (c) is especially sensitive for changes in the first letters of a word. Conclusively, it yields from this case that at least three different reference systems of object representation should be distinguished in neglect, which can be disturbed independently from each other and may negatively influence the behavioral potential of the patients.

[Nickel sensitization in women and its relevance to occupation]. / Zur Nickelsensibilisierung bei Frauen und ihre Relevanz zur beruflichen Tätigkeit

213 women and 26 men out of 7835 persons examined in the course of 9 years (1967--1975) had a positive reaction to nickle sulfate. The frequency of the sensitization to nickel increased during this time, from 0.9 to 5.01%. 121 of the sensitized women were less than 30 years old. The first manifestations of that contact eczema due to metal ions appeared on the earlobes, the fingers in the ring-region, the hands, the wrists and the neck. In most of the men but only in 20 women (1 photograph, 19 metallists), a professional cause could be revealed, and an occupational disease was recognized. In the other women, a non-professional cause was found. No hair-dresser had been sensitized to nickel, by an occupational contact. The increase of the allergy ot nickel from 1967 to 1975 was mainly due to wearing costume-jewelry since the localisation of the contact eczemas ofter corresponded to the trend of fashion. Before demanding the recognition as an occupational disease in case of positive tests, the relevance of the latter to the occupation of the subject should be elucidated.

Visuospatial hemi-inattention following cerebellar/brain stem bleeding.

Neglect is a unilateral lack of responsiveness to stimuli caused by visuospatial hemi-inattention, a unilateral representation deficit and/or a unilateral hypokinesia. It results most frequently from right-hemisphere brain damage, particularly of the parietal lobe but also of the frontal cortex, the basal ganglia, the thalamus, and recently it has also been described after a cerebellar lesion. We report a patient with right-sided bleeding of the posterior inferior cerebellar artery, who developed a left-sided visual hemi-inattention. She had no visual field defects, yet she had problems detecting left-sided targets in visual extinction. Furthermore, she was impaired in detecting complex motion on the left side and targets in a fixation offset paradigm. Reactions to left-sided targets in covert shifts of attention were slowed in the invalid condition. Her text reading was impaired as she could not always find the initial word of the next line. However, she was aware of her deficit. Her visuoconstructive ability was normal and she gave no indication of tactile or acoustic extinction. As the cerebellar lesion was located in the right hemisphere and the inattention involved the left side of space, we suggest that the damage to the right brain stem led to a transient imbalance of the noradrenergic ascending activation system which may explain her hemi-inattention.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL): a morphological study of a German family.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized clinically by recurrent cerebral infarcts, subcortical dementia and pseudobulbar palsy, and morphologically by a granular degeneration of cerebral and, to a lesser degree, extracerebral blood vessels. We present morphological findings in a further German family affected by CADASIL. The index case showed the typical periodic acid-Schiff-positive granular degeneration of vascular smooth muscle cells (VSMC) in cerebral vessels, which did not react with antibodies against various immunoglobulins or complement factors. Ultrastructurally, granular osmiophilic material (GOM) covered the VSMC in different cerebral regions as well as in extracerebral organs (muscle, nerve, skin, small and large intestine, liver, kidney and heart). Skin biopsy samples from other family members of the last two generations also revealed GOM irrespective of the clinical symptomatology (CADASIL, migraine only or asymptomatic). Patients in the third generation had higher amounts of GOM in skin vessels than did asymptomatic or migraine patients in the fourth generation. We conclude that skin biopsy is a useful and less invasive screening method for the differential diagnosis of CADASIL.

CADASIL: skin biopsy allows diagnosis in early stages.

OBJECTIVES: Our aim was to investigate the diagnostic impact of skin biopsies in CADASIL patients. MATERIALS AND METHODS: Eight consenting CADASIL patients belonging to a German-Caucasian kindred were assessed clinically, genetically, by MRI and skin biopsy. Skin biopsy results were compared to 5 patients suffering from sporadic leucoencephalopathies (control group). RESULTS: Six CADASIL patients presented with symptoms ranging from migraine to severe tetraparesis with dementia. Two clinically unaffected patients had abnormal MRIs. On MRI 7 patients showed various degrees of leucoencephalopathy. One 22-year-old woman with migraine had a normal MRI. Granular, electron dense, osmiophilic material (GEM) was found in skin biopsies of all 8 patients including the 22-year-old woman with migraine and a normal MRI. As shown by genetic linkage analysis she was carrying the disease haplotype. GEM was not found in the control group. CONCLUSION: Our findings substantiate the impact of skin biopsies in defining the carrier status in CADASIL families.

Phenotypic variability of the DYT1 mutation in German dystonia patients.

Primary dystonia is a clinically and genetically heterogeneous movement disorder characterized by sustained involuntary muscle contractions causing repetitive movements and/or abnormal postures. Recently, the gene locus (DYT1) and mutation responsible for a substantial number of cases suffering from early-onset primary dystonia was described. Here we report 2 German families and 1 sporadic patient with early-onset dystonia due to the DYT1 mutation in order to illustrate the variability of clinical manifestation within this molecularly defined entity. We demonstrate that writer's cramp or focal cervical dystonia is a clinical presentation of DYT1 as well as generalized dystonia.

The phenotypic spectrum of CADASIL: clinical findings in 102 cases.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an increasingly recognized autosomal dominant disorder that leads to cerebrovascular manifestations in early adulthood. This study delineates the phenotypic spectrum and the natural history of the disease in 102 affected individuals from 29 families with biopsy-proven CADASIL. Recurrent ischemic episodes (transient ischemic attack [TIA] or stroke) were the most frequent presentation found in 71% of the cases (mean age at onset, 46.1 years; range, 30-66 years; SD, 9.0 years). Forty-eight percent of the cases had developed cognitive deficits. Dementia (28%) was frequently accompanied by gait disturbance (90%), urinary incontinence (86%), and pseudobulbar palsy (52%). Thirty-nine patients (38%) had a history of migraine (mean age at onset, 26.0 years; SD, 8.2 years), which was classified as migraine with aura in 87% of the cases. Psychiatric disturbances were present in 30% of the cases, with adjustment disorder (24%) being the most frequent diagnosis. Ten patients (10%) had a history of epileptic seizures. To delineate the functional consequences of ischemic deficits, we studied the extent of disability in different age groups. The full spectrum of disability was seen in all groups older than age 45. Fifty-five percent of the patients older than age 60 were unable to walk without assistance. However, 14% in this age group exhibited no disability at all. Kaplan-Meier analysis disclosed median survival times of 64 years (males) and 69 years (females). An investigation of the 18 multiplex families revealed marked intrafamilial variations.