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Complete secondary therapy failure due to antibodies against botulinum toxin A (BoNT/A-ABs) may raise extensive treatment difficulties. We tested whether neutralizing BoNT/A-ABs can be detected in dystonic patients with good clinical responses to botulinum toxin A (BoNT/A) treatment.

Dopaminergic treatment is very effective in restless legs syndrome (RLS) and periodic leg movements in sleep (PLMS). However, neuroreceptor imaging studies that addressed altered striatal dopaminergic function have given controversial results. In this present study, 14 patients with idiopathic RLS (iRLS) and PLMS with a good response to dopaminergic and non-dopaminergic treatment and ten healthy sex- and age-matched controls were investigated off-medication by using 123I-IBZM and SPECT. RLS symptoms and sleep disturbances were evaluated using three nights of polysomnography, the Pittsburgh Sleep Quality Index, and the International RLS Study Group (IRLSSG) rating scale. The patients presented with sleep disturbances, a high PLMS index (56.2±33.1 per h), and severe RLS symptoms during SPECT (IRLSSG rating scale 23.1±8.0), and showed no significant differences in striatal to frontal IBZM binding to D2receptors compared to controls (ratio striatumfrontal cortex, right side 1.60±0.10 vs 1.63±0.08, P0.35, NS; left side 1.61±0.11 vs 1.63±0.08, P0.51, NS). These findings show normal function of striatal D2receptors in successfully treated patients with iRLS and PLMS. Dopaminergic and non-dopaminergic pretreatment does not appear to change striatal D2receptor binding as compared to healthy controls. Structures other than striatal D2receptors are discussed as possible causes of the treatment effects in RLS.

Botulinum toxin type A (BTXA) is used to treat neurologic disorders associated with increased muscle tone. Its use is often associated with pain relief.

Zusammenfassung: Der Barthel-Index (BI) ist die am weitesten verbreitete Beurteilungsskala für die Aktivitäten des täglichen Lebens. Für die Einschätzung von Fähigkeiten und Betreuungsaufwand im Alltag der Neurorehabilitation sind aber auch Störungen der psychischen und mentalen Grundfunktionen, die durch den BI nicht erfasst werden, von wesentlicher Bedeutung. Wir haben eine Skala erstellt, die Fähigkeitsstörungen in den Bereichen Bewusstsein, Kontaktfähigkeit, Orientierung, Verhalten, Emotion, Kommunikation, Gedächtnis, Problemlösung, Wahrnehmung und Nachtruhe einschließt und die in einer 0–100% Skalierung bzw. der Vergabe von 0–20 Punkten für die 10 Items die exakt gleiche Struktur wie der BI aufweist. Es wurde in der Erstellung der Items darauf Wert gelegt, dass der Summenscore die Fähigkeitsstörungen und den Betreuungsaufwand von komatösen bzw. Komaremissionspatienten (am unteren Skalenende) und Patienten mit geringen Vehaltens- oder neuropsychologischen Störungen (am oberen Skalenende) möglichst wirklichkeitsnahe abbildet. Der Neuromentalindex wurde an 179 Neurorehabilitationspatienten bezüglich seiner Testgütekriterien untersucht und erwies sich als in hohem Maß valide, reliabel und praktikabel.

Summary:  Although physical and emotional dysfunction appears to be quite frequent even among independent survivors of aneurysmal subarachnoid haemorrhage (SAH), these symptoms may easily be missed on routine follow-up examinations. To assess the long-term functional effects of SAH and to outline possible treatment approaches, a cross-sectional study using multidimensional measures of relevant areas of function was performed on 40 independent survivors. After an average follow-up period of 22 months, patients were selected and enrolled following a pre-designed protocol. The comprehensive test battery consisted of subjective and objective measures of physical, psychological and social function and relationships between the different levels of assessment were calculated. We found a considerable proportion of cognitive, emotional and physical dysfunction in this sample, but on the subjective level, the majority of the patients stated satisfaction with life in general. Mild cognitive dysfunction was frequently missed and causes distress in the family. Mild to moderate depression was underdiagnosed, although such an emotional dysfunction influences working capacity and quality of life. Referral to rehabilitation centers appears to be restricted to patients with severe impairments. Our results help to alert the neurosurgeon to these possible symptoms and show the urgent need for a prospective, interdisciplinary and multidimensional follow-up of SAH survivors.

Zusammenfassung: Die Botulinumtoxinbehandlung (BTX) gilt als Therapie der Wahl bei Torticollis spasmodicus (TS), Blepharospasmus (BL) und hemifazialem Spasmus (HFS). Da bestehende Beschwerden mit BTX meist nur vorbergehend und manchmal nicht im ganzen Ausma beseitigt werden knnen, werden von Patienten immer wieder andere Behandlungsformen versucht. Mit einem einfachen Fragebogen wurde von Patienten mit TS, BL und HFS eine subjektive Beurteilung aller bisher versuchten Therapien eingeholt. Ausgewertet wurden 200 Fragebgen (112 TS, 54 BL, 34 HFS). BTX wurde in allen drei Diagnosegruppen als subjektiv wirksamste Therapie bewertet (Median: 2=gute Wirkung). Abgesehen von Citalopram und Physiotherapie (Median: 3=durchschnittliche Wirkung) beim TS wurden alle anderen Therapien mit einem Median von ≥4 (=minimale Wirkung) bewertet. Patienten mit TS haben im Durchschnitt 7,7, Patienten mit BL 2,4 und mit HFS 2,6 Behandlungsformen ausprobiert. Zusammenfassend ist BTX die subjektiv wirksamste Behandlung bei Patienten mit TS, BL und HFS. Weitere Untersuchungen ber ergnzende Begleittherapien zur BTX-Behandlung sind erforderlich.

In experimental denervation of rabbit skeletal muscle, an increase of gangliosides has been reported. Ganglioside, DNA, total lipid and protein levels were studied in experimental denervation of rabbit gastrocnemius muscle and in human skeletal muscle with denervation atrophy. In severe human denervation atrophy total ganglioside, DNA and total lipid levels were increased, and protein levels decreased as compared with controls. The ganglioside pattern was changed in severe human denervation atrophy and showed a significant increase of a GT1b-like and moderate decrease of a GM1-like compound. Findings in experimentally denervated rabbit muscle resembled results obtained in human denervation atrophy, showing a significant increase of total ganglioside levels as well as a moderate increase of GT1b-like and decrease of GM1-like compounds.

Bei Patienten mit koronarer bzw. peripherer Gefäßerkrankung ist das Risiko einer prophylaktischen Karotisendarterektomie wesentlich grōßer als bei klinisch Gesunden. Dieser Eingriff ist vor peripherer Gefäßrekonstruktion mit einer perioperativen Schlaganfalls-rate von etwa 5% und einer Mortalitätsrate von etwa 2% verbunden. Bei Patienten vor Koronararterien-Bypass-operation kommt es im Zusammenhang mit der prophylaktischen Karotisendarterektomie bei etwa 4% der Fälle perioperativ zu zerebralen Ischämien, zusätzlich besteht eine Mortalität von etwa 3%.

Summary Seventy patients suffering from ischemic cerebrovascular disease had had their first attack before the age of 40 years. Of these patients 57 were re-examined 84 months (mean) after the initial event. Within 48 months, 22 of 51 patients (43.1%) without surgical treatment had recurrent ischemic events (78.6% patients with transient ischemic attacks, 33.3% patients with protracted reversible deficits, 28.6% patients with completed stroke). Of all patients 31.5% had their first recurrent attack during the first year, most of them during the first few months after the initial event.

Within a period of 13 months a 24-year-old male student experienced four attacks of subarachnoid haemorrhage. An intradural-extramedullary haemangioma at the level of the second thoracic segment was found to be the cause of the repeated bleeding.

We studied the initiation of saccades to visual-guided random time and remembered targets in a group of nine Parkinsonian patients with severe motor fluctuations and in 9 age matched control subjects. In contrast to a marked skeletomotor improvement during the “on” condition, saccadic latencies for both visual-guided random saccades and remembered saccades were increased in the patients during the “on” condition compared to the “off” condition. This result of dissociation between skeletomotor and oculomotor function indicates that common concepts of saccadic initiation in parkinsonian patients do not hold true in patients with severe fluctuations since dopaminergic stimulation seems to increase saccadic latencies in these patients.

Aim of this study was the characterization of the circadian melatonin profile in de novo Parkinson patients (N=9, age 60.0±3.2 years, mean ± SEM) and the comparison of these profiles with those of controls and Parkinson patients treated with I-dopa/decarboxylase inhibitor (l-dopa/DCI). We collected 14 venous blood samples during a period of 24 hours and measured the serum melatonin levels by a radioimmuno assay. De novo Parkinson patients displayed the nocturnal melatonin peak (acrophase) at the same time as controls and significantly later than l-dopa/DCI treated patients (1:54±15.6 min [average clock time ± SEM in minutes] vs. 1:45±15.6 min vs. 0:13±40.8 min). The amount of secreted melatonin did not differ among the three groups. Stage and duration of Parkinson's disease did not correlate with the amount of secreted melatonin. Patients of the tremor subgroup, however, secreted more melatonin than patients presenting only with rigidity and akinesia. The phase advance in Parkinson patients treated with l-dopa/DCI is possibly due to a central nervous dopaminergic effect elicited by l-dopa administration and not inherent to Parkinson's disease per se.

Physical activity during lifetime was investigated among 32 Parkinson patients (age 65.6±8.1 yrs, mean±SD) retrospectively by means of a structured interview. Data were compared with 31 healthy controls (age 61.7±5.8 yrs). An objective score was obtained by presenting a list of all kinds of sports, subjective estimation of physical activity was done by visual analogous scales. Until the occurrence of the first symptoms (mean=58.5 yrs) the patients did not differ from controls. During the course of disease a striking reduction in physical activity but no complete abandonment of sports was found. Swimming, hiking and gymnastics were the favoured sports in both groups. Learning of new sports seemed to be impossible for the patients.

Although several studies on the efficacy and the toxicity of exogenous melatonin in Parkinson patients have been carried out, there are no data available on melatonin secretion in these patients. We therefore performed a controlled trial in 9 Parkinson patients (aged 62.1±8.7 years, ×±SD) and in 14 control persons (58.0±10.4 years). Parkinson patients were treated with 1-dopa (300–1000 mg per day) in combination with a peripheral decarboxylase inhibitor (benserazide, carbidopa). 14 venous blood samples were taken from each person during a period of 24 hours in order to investigate the circadian secretion pattern of melatonin. Serum melatonin levels were estimated by radioimmuno assay. We found that the circadian secretion patterns of 1-dopa-treated Parkinson patients and age-matched controls were very similar except for a phase advance of the nocturnal melatonin elevation in the parkinsonian group.

Summary In diseases associated with thrombotic or thromboembolic complications, a reduction in the fibrinolytic potential may contribute to the risk to develop thrombosis.

The outcome of 142 patients suffering from ischemic cerebral circulation disorders was followed up over a period of 33 months on average. The spontaneous course of 25 patients with unilateral, hemodynamically ineffective stenoses of the vertebral artery was compared with that of 107 patients without vertebral artery stenoses. Within the first 12 months, novel clinically manifest cerebrovascular events were observed in 16.0% of patients without vertebral artery stenosis (deaths 5.0%), but in only 4.3% of the patients with vertebral artery stenosis (no deaths). Within 30 months, only 2 of the 13 patients with vertebral artery stenosis had suffered a new cerebrovascular attack. At the end of the observation period, 39.3% of the patients without vertebral artery stenosis and 48.0% of the patients with vertebral artery stenosis were significantly disabled in their social life or had died. An additional unilateral hemodynamically irrelevant vertebral artery stenosis did not influence the rate of reinfarction or the remission of neurological deficits, independently of age, the degree of the circulatory disorder, the vascular territory involved, the presence of an organic psychosyndrome, or of additional stenoses in the carotid arteries. Consequently, a vertebral artery stenosis narrowing the vessel diameter to less than 1/3 is without prognostic relevance.

Despite current antibiotic treatment, a 51‐year‐old man suffering from Whipple's disease (WD) presented with cerebral symptoms. Clinical, cerebrospinal fluid (CSF) and magnetic resonance imaging (MRI) findings revealed encephalitis resulting from WD, which was subsequently treated. The response to antibiotics was protracted, but 6 months after the onset of cerebral illness the inflammation had ceased. Thirty months later, the patient is still suffering from severe anterograde amnesia as well as a lack of initiative. Behavioural and neuropsychological assessment data were consistent with the Korsakoff syndrome. Retrospectively, a concomitant thiamine deficiency in the acute phase of cerebral manifestation was thought likely, and could have contributed to the unfavourable outcome. We therefore propose that thiamine deficiency should be considered and treated immediately in patients with established WD who present with cerebral symptoms.

Article abstract—Hypertrophic chronic pachymeningitis (HCP) is a rare disorder that causes intracranial or spinal thickening of the dura mater. This report describes a patient with progressive HCP in the craniocervical region associated with signs of rheumatic disease. A ventricular-atrial shunt had to be inserted because of increased intracranial pressure. The patient improved after suboccipital craniotomy, C1 to C6 laminectomy, and removal of the thickened dura. Additional therapy with methotrexate stopped progression, which was documented by MRI and PET.

Mutations in the valosin-containing protein (VCP) on chromosome 9p13-p12 were recently found to be associated with hereditary inclusion body myopathy, Paget disease of the bone, and frontotemporal dementia (IBMPFD). We identified a novel missense mutation in the VCP gene (R159H; 688G>A) segregating with this disease in an Austrian family of four affected siblings, who exhibited progressive proximal myopathy and Paget disease of the bone but without clinical signs of dementia.