IVIg dose increase in multifocal motor neuropathy: A prospective six month follow-up

In this prospective, non-randomized 6-month observational study we evaluated the efficacy of intravenous immunoglobulin (IVIg) dose increase in patients with multifocal motor neuropathy (MMN). Diagnosis according to AAEM criteria, repetitive IVIg treatment for at least one year, persistent paresis and conduction block, stable symptoms and findings for at least six months were inclusion criteria. Nine patients (7 men) were identified and approved to standardized increase of IVIg dose. Patients were monitored using clinical scores and electrophysiological studies. Dose was increased from a baseline of 0.5 g/kg per month [mean, range: 0.1-1.1], given at variable intervals [4-12 weeks] to 1.2 g/kg per month given over 3 consecutive days planned for 6 cycles. If the patients' motor function did not improve after two cycles they entered step two: Dose was increased to 2 g/kg per month given over 5 consecutive days. The increased dose was maintained for 6 months. Assessments were performed by the same investigator, not involved in the patient's management, at baseline, after 2 and after 6 months. Following dose increase, motor function significantly improved in 6 patients (p = 0.014), 2 patients entered step two, 1 patient withdrew due to absent efficacy. Higher doses of IVIg caused more side effects, however, transient and rarely severe (p = 0.014). IVIg dose increase may improve motor functions in patients with stable MMN on long-term IVIg therapy independent of baseline dose. Improvement of motor function was associated with shorter disease duration (p = 0.008), but not with degree of muscle atrophy (p = 0.483). The treatment strategy to try to find the lowest effective dose and the longest tolerated interval might lead to underdosing in the long-term in many patient

Pulsatile tinnitus —a review of 84 patients

Pulsatile tinnitus can be annoying for a patient and can also be the only clue to a potentially devastating and life-threatening disease. In order to understand its clinical spectrum and management better we analysed the files of 84 patients seen at our institution over a 10-year period. Noninvasive techniques (ultrasound, computed tomography, magnetic resonance imaging) and angiography were employed as investigations tailored to the individual patient. A vascular disorder [i.e. arteriovenous fistula, dissection of the internal carotid artery (ICA), fibromuscular dysplasia, aneurysm of the ICA and sinus thrombosis] was found in 36 patients (42%), most commonly a durai arteriovenous fistula or a carotid-cavernous sinus fistula. In 26 patients with a vascular abnormality, pulsatile tinnitus was the presenting symptom. In 12 patients (14%), nonvascular disorders such as glomus tumour or intracranial hypertension with a variety of causes explained the tinnitus. We conclude that patients with pulsatile tinnitus should be investigated with noninvasive techniques. If these are negative or to clarify abnormal findings of noninvasive techniques selective angiography is needed for diagnosis and to guide treatmen

IVIg dose increase in multifocal motor neuropathy: a prospective six month follow-up

In this prospective, non-randomized 6-month observational study we evaluated the efficacy of intravenous immunoglobulin (IVIg) dose increase in patients with multifocal motor neuropathy (MMN). Diagnosis according to AAEM criteria, repetitive IVIg treatment for at least one year, persistent paresis and conduction block, stable symptoms and findings for at least six months were inclusion criteria. Nine patients (7 men) were identified and approved to standardized increase of IVIg dose. Patients were monitored using clinical scores and electrophysiological studies. Dose was increased from a baseline of 0.5 g/kg per month [mean, range: 0.1-1.1], given at variable intervals [4-12 weeks] to 1.2 g/kg per month given over 3 consecutive days planned for 6 cycles. If the patients' motor function did not improve after two cycles they entered step two: Dose was increased to 2 g/kg per month given over 5 consecutive days. The increased dose was maintained for 6 months. Assessments were performed by the same investigator, not involved in the patient's management, at baseline, after 2 and after 6 months. Following dose increase, motor function significantly improved in 6 patients (p = 0.014), 2 patients entered step two, 1 patient withdrew due to absent efficacy. Higher doses of IVIg caused more side effects, however, transient and rarely severe (p = 0.014). IVIg dose increase may improve motor functions in patients with stable MMN on long-term IVIg therapy independent of baseline dose. Improvement of motor function was associated with shorter disease duration (p = 0.008), but not with degree of muscle atrophy (p = 0.483). The treatment strategy to try to find the lowest effective dose and the longest tolerated interval might lead to underdosing in the long-term in many patients

Lesions to Primary Sensory and Posterior Parietal Cortices Impair Recovery from Hand Paresis after Stroke

Neuroanatomical determinants of motor skill recovery after stroke are still poorly understood. Although lesion load onto the corticospinal tract is known to affect recovery, less is known about the effect of lesions to cortical sensorimotor areas. Here, we test the hypothesis that lesions of somatosensory cortices interfere with the capacity to recover motor skills after stroke

Psychosoziale Aspekte der Lebensbedingungen von Frauen und Männern im mittleren Lebensalter

"Hauptanliegen dieses Beitrages ist die Deskription der Lebensbedingungen und der - damit verbundenen - psychosozialen Aspekte in einer für urbane Schweizer Verhältnisse repräsentativen Stichprobe im mittleren Lebensalter. Befragt wurden 1015 Personen zweier Altersgruppen (40 bis 45 resp. 50-55 Jahre). Diese Gruppen wurden verglichen hinsichtlich der Art und Häufigkeit von erlebten Transitionen, sowie im Hinblick auf ihre sozialen Ressourcen und ihr physisches und psychisches Wohlbefinden. Besonders berücksichtigt wurden geschlechtstypische Unterschiede sowie Unterschiede der beruflichen und familiären Situation. Den Resultaten zufolge ist der Hauptanteil der befragten Personen verheiratet und hat im Schnitt ein bis zwei Kinder. Die Mehrheit der 40-45jährigen lebt noch mit mindestens einem Kind im gleichen Haushalt, im Alter von 50-55 Jahren sind jedoch bei der Hälfte der Frauen alle Kinder bereits ausgezogen. Mit steigendem Alter werden die Leute in zunehmendem Maße mit dem Sterben ihrer eigenen Eltern konfrontiert, was an der alterskorrelierten Abnahme des Anteils von Frauen und Männern, welche noch beide Eltern oder zumindest noch einen Elternteil haben, ersichtlich ist. Was die Verfügbarkeit und Nutzung sozialer Netzwerke anbelangt, zeigt sich, dass Frauen im quantitativen und qualitativen Sinne bessere soziale Netzwerke haben als Männer. Die Mehrheit der Befragten empfindet ihr Leben als eher stabil und kontinuierlich. Jedoch zeigen sich klare Unterschiede zwischen den beiden Altersgruppen: Die Leute im Übergang ins mittlere Lebensalter zeichnen sich durch eine größere Instabilität aus als die 50-55jährigen. Die erlebten Veränderungen betreffen vor allem die Partnerschaft, den Beruf und das Selbst. Die höchste Stabilität wird insbesondere für die Beziehung zu den eigenen Kindern und zu den Eltern empfunden, was für beide Altersgruppen gleichermaßen zutrifft. Die Tatsache, dass die Transition ins mittlere Lebensalter als eine eher 'bewegte' Zeit empfunden wird, spiegelt sich in der Befindlichkeit der Betroffenen wider: Die 40-45jährigen weisen bedeutsam tiefere Werte beim psychischen Wohlbefinden auf als die 50-55jährigen, obwohl es sich bei der gesundheitlichen Selbsteinschätzung gerade umgekehrt verhält: Die jüngere Altersgruppe schätzt ihre Gesundheit bedeutsam positiver ein als die ältere. Die ältere scheint sich indes psychisch, trotz zunehmender altersbedingter gesundheitlicher Probleme, an die neue Lebensphase adaptiert und neuäquilibriert zu haben. Die subjektive Gesundheitseinschätzung sowie das psychische Wohlbefinden kovariiert unseren Resultaten zufolge mit dem Alter, jedoch nicht mit dem Geschlecht." (Autorenreferat)"The main purpose of this contribution is the description of living conditions and the associated psycho-social aspects in a sample of mid-aged persons. 1015 persons of two age groups (40-45 resp. 50-55 years) were interviewed. These two groups were com¬ pared with regard to the amount and nature of perceived transitions, and regarding their social networks, and their physical and psychological well-being. Special care was given to the better understanding of gender differences. Our sample is representative for the urban population in Switzerland. Our results show that the majority of our sample is married and has on average 1-2 children. Most of the 40-45 years olds still live with one ore more children in the same household, whereas in the group of the 50- 55 years olds this is only the case for half of them. The fact that with advancing age people are more and more confronted with the death of their parents, is reflected in an age-correlated decrease of the persons having one or both parents still alive. Concerning the availability of social networks it can be said, that women have better social network than men do (this under a quantitative and qualitative point of view). Even though the majority of our sample experience their life-course as rather stable and continuous there are clear differences between both age-groups: People at the beginning of mid-life experience a larger instability in their life and report significantly more changes and turning-points than persons aged 50-55 years. These changes concern mainly partnership, job and the self. The largest stability is perceived in the relationship to the own children and parents, which is the case for both age-groups. The fact that mid-life transition is associated with more idiosyncratic changes and turning points than later on is significantly related to physical and psychological wellbeing: Even though people of the younger age-group report significant better physical well-being than the older ones, their psychological well-being is significantly lower than that of the older group. People of the older age-group seem to have adapted despite increasing health problems to the new life-phase and have found a new equilibration. Our results supply empirical evidence that the transition into middle age is marked by a variety of changes leading toward increasing equanimity and vigour with advancing age. Our findings contribute to a better understanding of living conditions in mid-aged persons and how well-being across this life phase is achieved and maintained. They underline the importance of research on middle adulthood within life-span developmental research." (author's abstract

Neuralgia of the glossopharyngeal nerve in a patient with posttonsillectomy scarring: recovery after local infiltration of procaine-case report and pathophysiologic discussion

We describe a patient with a three-year history of severe progressive left-sided glossopharyngeal neuralgia (GPN) that failed to adequately respond to various drug therapies. The application of lidocaine spray to the posterior pharyngeal wall provided no more than short-term relief. Apart from a large hypertrophic tonsillectomy scar on the left side all clinical and radiologic findings were normal. In terms of therapeutic local anaesthesia, the hypertrophic tonsillectomy scar tissue was completely infiltrated with the local anaesthetic (LA) procaine 1%. The patient has been almost completely pain-free ever since, and the lidocaine spray is no longer needed. Six weeks after the first treatment a repeat infiltration of the tonsillectomy scar led to the complete resolution of all symptoms. The patient has become totally symptom-free without the need to take any medication now for two and a half years. This is the first report of a successful therapeutic infiltration of a tonsillectomy scar using an LA in a patient with GPN that has been refractory to medical treatment for several years. A possible explanation may be that the positive feedback loop maintaining neurogenic inflammation is disrupted and "sympathetically maintained pain" resolved by LA infiltration

Tolerance of intravenous methylprednisolone for relapse treatment in demyelinating CNS disease

In Switzerland, the first course of intravenous steroids for treatment of episodes of demyelinating CNS disease is usually administered in an inpatient setting. We prospectively evaluated short term tolerance of treatment with special emphasis on sleep quality