Observations are presented on nine selected patients with chronic upper limb demyelinating neuropathy to illustrate the range of manifestations that may be observed. In three, the involvement was purely motor, in five, mixed motor and sensory and, in one, virtually purely sensory; in seven the symptoms were unilateral and in two bilateral. The presence of reduced nerve conduction velocity and conduction block and the response to treatment in seven of the cases indicate that they represented examples of chronic inflammatory demyelinating polyneuropathy (CIDP) with focal involvement. This was confirmed by nerve biopsy in two cases. The presentation in one patient was accompanied by forearm swelling initially suspected of being a tumour but shown to be due to muscle hypertrophy. This was probably the consequence of recurrent muscle cramps and fasciculation and possibly neuromyotonia. The patient with predominant sensory involvement restricted to the upper limbs demonstrates that sensory CIDP can present focally. In one patient with monomelic motor and sensory involvement, nerve biopsy showed multifocal areas of hypertrophic demyelinating neuropathy distally in the ulnar nerve without inflammatory infiltration. This patient failed to respond to therapy. Response in the others was satisfactory, although one patient with a monomelic motor neuropathy showed a severe deterioration after being given corticosteroids; he subsequently improved with intravenous human immunoglobulin therapy.
An unstable DNA sequence of a gene encoding a protein kinase has been identified as the molecular basis of myotonic dystrophy. The correlation between different symptoms of myotonic dystrophy and the size of this unstable base triplet (CTG)n repeat was investigated in 14 patients. DNA was prepared from whole blood by standard procedures. Detailed clinical, psychological, electrophysiological (quantified measurement of myotonia, electrocardiography) and other laboratory examinations (muscle biopsy in 4 patients, slit lamp examination) were performed. Triplet size correlated significantly with muscular disability and inversely with age at onset of the disease. A greater frequency of mental and gonadal dysfunction could be observed in patients with a larger repeat size. Other symptoms, however, such as cataract, myotonia, gastrointestinal dysfunction and cardiac abnormalities were not correlated with repeat size. Somatic mosaicism with different amplification rates in various tissues might be one possible explanation for the variable phenotypes. Furthermore, other factors such as different expression of the myotonic dystrophy gene might contribute to the clinical variability of the disease at a given triplet size.
Multifocal motor neuropathy (MMN) can be differentiated from motor neuron disease by electrophysiological evidence of conduction block. To increase the probability of recording conduction block, we studied the whole nerve length including proximal segments in 84 patients with pure motor syndromes, using a special stimulation technique. In 8 patients, the diagnosis of MMN was confirmed by electrophysiological evidence of conduction block or temporal dispersion. The typical clinical picture of MMN with chronic progressive, asymmetrical, marked distal weakness was observed in our patients. Electrophysiological routine tests of distal nerves were usually normal except in nerve segments with conduction block. In 4 patients, conduction block could be recorded only in proximal nerve segments or spinal roots. All patients showed rapid improvement of clinical features and parallel reduction of conduction block during or after high-dose intravenous immunoglobulin (ivIG) therapy, supporting the diagnosis of an immune-mediated neuropathy. Three of them are now in remission without any therapy, whereas 5 still receive a regular ivIG course every 2-12 weeks as long-term treatment. In all patients with pure or predominantly motor syndromes and normal findings in electrophysiological routine tests of distal nerve segments, there should be proximal conduction block studies to avoid overlooking a treatable disorder such as MMN.
The hypothesis was tested that an initial lithium-tricyclic antidepressant (TCA) combination has a better antidepressant effect than standard TCA treatment in non-refractory depression at the beginning of an episode. Twenty bipolar melancholic type depressed inpatients under lithium-TCA treatment were compared with 20 patients with the same diagnosis and TCA-placebo treatment for 5 weeks under double-blind conditions. All patients were male. Initial lithium-TCA treatment reduced depressive symptoms significantly more than antidepressant treatment with TCA and placebo after 5 weeks, but not in weeks 1 or 2. It can be concluded that lithium augmentation of TCA treatment should be started even at the beginning of antidepressant TCA treatment to provide a better treatment response in those patients who will profit from long-term lithium prophylaxis, e.g. bipolar patients with melancholic type depression.
An acute spinal epidural abscess is a rare cause of paraplegia, seen in seven patients over a period of ten years. All patients had fever and severe localized back-pain. Unless treated, within hours or a few days, there will be root defects and rapidly progressive paraplegia. Staphylococcus is the most frequent causative organism and clinically manifest septicaemia is common. Rapid diagnosis and treatment are essential in deciding the patient's fate. Myelography is an important additional examination as it demonstrates the abscess in 96% of cases. Non-contrast radiology is of little value. High-dosage antibiotics and surgical spinal decompression are the cardinal treatment procedures. Antibiotics alone are justified only so long as there are no neurological deficits and neurosurgical intervention, if needed, is immediately available.
Clinical data about the course of long-term WIG treatment in chronic inflammatory demyelinating polyneuropathy (CIDP) and in multifocal motor neuropathy (MMN) are sparse. Twenty patients with CIDP or MMN were investigated during up to 65 months of high-dose intravenous immunoglobulin (IVIG) therapy in an open and prospective study. In all therapyresponders (18/20), amelioration of symptoms was evident within the first 3 weeks of treatment. Dosage was reduced slowly according to the clinical symptoms. All 18 patients now show very few or no symptoms. The mean value of the Rankin disability scale changed from 2.5 to 1.1 during therapy. Ten patients are still regularly given IVIG. In eight patients no further therapy has been necessary for 7-63 months. Treatment could even be stopped in one patient after 46 months of therapy. No marked clinical side-effects were observed during 529 months of IVIG therapy. IVIG therapy is safe, and remains effective in the long-term treatment of CIDP and hlMN.
ZusammenfassungAls immunvermittelte und damit prinzipiell gut behandelbare Polyneuropathie sollte die CIDP möglichst frühzeitig und sicher diagnostiziert werden. Die typische klinische Symptomatik besteht in progredienten oder schubförmigen, oft proximal-betonten, symmetrischen, vorwiegend motorischen peripheren Defiziten. Grundsätzlich basiert die Diagnosesicherung aber auf elektrophysiologischen Parametern. Während entsprechend der diagnostischen Kriterien der ¹American Academy of Neurologyª eine signifikante Demyelinisierung in zahlreichen Nerven mittels verschiedener Parameter nachgewiesen werden muss, sollte ± aufgrund atypischer Sonderformen der CIDP mit mehr umschriebenen, asymmetrischen oder distal-betonten klinischen Defiziten ± mittlerweile besonderer Wert auf den Nachweis umschriebener Entmarkungen wie Leitungsblock oder fokale temporale Dispersion gelegt werden. Sind gerade zu Beginn der Erkrankung die Läsionen nur sehr umschrieben, kann die proximale Nervenstimulation zum Nachweis fokaler Entmarkungen im Bereich von Plexus oder Wurzel von besonderer Bedeutung sein. Von der idiopathischen CIDP abgegrenzt werden sollten symptomatische Formen wie die CIDP bei monoklonalen Gammopathien, da sich hier häufig therapeutische Besonderheiten ergeben. Therapeutisch kommen bei der CIDP neben den drei Basistherapieverfahren (Kortison, hoch dosierte Immunglobuline, Plasmapherese) auch verschiedene klassische immunsuppressive Medikamente in Betracht. Zur Therapiekontrolle sollte neben einer validierten klinischen Befunderhebung auch der elektrophysiologische Verlauf mit besonderer Berücksichtigung der Leitungsblockdiagnostik herangezogen werden. AbstractChronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune and treatable disease. Therefore, CIDP should be diagnosed as early as possible. Typical clinical symptoms of CIDP are progressive or stepwise, often proximal, symmetrical deficits with predilection of motor nerves. Diagnosis of CIDP is predominantly based on electrophysiological criteria. According to the diagnostic criteria of the American Academy of Neurology, significant demyelination must be demonstrated in multiple nerves by different parameters. However, most acquired demyelinating polyneuropathies with more focal, asymmetrical or distally accentuated clinical deficits are also considered to be variants of CIDP. Therefore, diagnostic evidence of focal demyelination with conduction block or focal temporal dispersion is more important. In early CIDP, focal demyelination might be limited to proximal nerve segments as plexus or dorsal roots and proximal nerve stimulation can be necessary to demonstrate focal demyelinating lesions. Symptomatic forms of CIDP as in monoclonal gammopathy should be differentiated from idiopathic CIDP because of differences in treatment response. Therapy of CIDP comprises three alternative basic regimens (steroids, immunoglobulins, plasmapheresis). In non-responders, different immunosuppressive agents can be applied. Treatment response should be evaluated by stan...
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