Multifocal motor neuropathy: Response to human immune globulin

Multifocal motor neuroparrhy (MMN) is a progressive disorder producing asymmetrical weakness and muscle wasting. Case reports suggest that patients with MMN improve after cyclophosphamide therapy, but not after prednisone or plasmapheresis, Because MMN is likely to be immune mediated, we investigated the therapeutic response to human immue globulin (HIG) in an open, uncontrolled trial. Nine patients, ages 28 to 58 years, had chronic, progressive, asymmetrical, predominantly distral, limb weakness for 5 to 18 years, Sensation was normal, and reflexes were reduced asymmetrically. All had physiological evidence of multifocal motor demyelination with partial motor conduction block, and 7 had elevated serun titers of anti-GMI IgM antibody. All patients were treated with HIG, 106 to 2.4 gm/ kg., given intravenously over 3 to 5 days. Strength inproved in all patients 3 10 days after treatment, with improvement peaking at 2 weeks and lasting for an average of 2 months. The range of functional improvement veried from dramatic to mild. The degree of partial motor conduction block was reduced, at least partially, in 7 to 8 patients. The serum anti-GM1 antibody titers did not change. Repeated courses of HIG resulted in similar improvements. We conculde that HIg may be an effective therapy for patients with MMN.     

Neurological and adrenal dysfunction in the adrenal insufficiency/alacrima/achalasia (3A) syndrome

Review of 20 patients with glucocorticoid deficiency (three cases also with salt loss) associated with absent tear secretion (19 cases) and achalasia of the cardia (15 cases) revealed neurological abnormalities in 17 including hyper-reflexia, muscle weakness, dysarthria, and ataxia together with impaired intelligence and abnormal autonomic function, particularly postural hypotension. These findings indicate that significant neurological problems are common in this multisystem disorder.     

Diverticular abscesses: percutaneous drainage

Percutaneous catheter drainage was performed in 16 patients with diverticulitis complicated by abscesses. Each patient had resolution of fever within 72 hours. Eleven patients subsequently underwent simultaneous sigmoid resection and operative anastomosis 10-40 days after percutaneous drainage. One patient required a three-stage procedure after percutaneous drainage, and one patient was too unstable for operation at any time during her course and eventually died of respiratory failure. Three patients did not undergo resection after catheter drainage and have remained asymptomatic for 1-2 1/2 years. Ten of 16 patients had fistulas, eight of which closed spontaneously. Experience with percutaneous drainage of diverticular abscesses suggests that it obviates surgical abscess drainage and permits a single operation (sigmoid resection and closure) to be performed safely. Percutaneous abscess drainage has cost-saving implications, since one or two operations may be avoided in most patients, and in some high-risk elderly patients all operations may be obviated.     

Corpus callosum and limbic system: neuroanatomic MR evaluation of developmental anomalies

Agenesis of the corpus callosum is a complex malformation of the brain that has been associated with varying degrees of limbic system maldevelopment. We retrospectively reviewed the records of 11 patients with callosal agenesis (seven total, four partial) who underwent magnetic resonance (MR) imaging, with particular attention to the associated malformations of the limbic system. Comparison was made with selected images from MR examinations of healthy volunteers and with necropsy specimens from other patients with callosal agenesis. Ten of 11 patients demonstrated limbic anomalies (severe motion artifact precluded evaluation of these structures in one patient). MR depicted not only the abnormalities intrinsic to callosal agenesis but also the frequently associated malformations of the limbic system.     

Are the associations between Alzheimer's disease and polymorphisms in the apolipoprotein E and the apolipoprotein CII genes due to linkage disequilibrium?

Allele frequencies for polymorphisms in the aplipoprotein E and the apolipoprotein CII genes were determined in subjects of Ashkenazi Jewish origin with lateonset Alzheimer's disease and in unaffected control subjects from the same ethnic group. A significant association was observed between late-onset Alzheimer's disease and the ε4 (112_Cys→Arg) allele of apolipoprotein E; however, no association was detected with apolipoprotein CII. These results suggest that the association with ε4 is probably not due to linkage disequilibrium.     

伊贝碱甙B的分离和结构测定

本文报道自吉林栽培的伊贝母(Fritillaria pallidiflora Schrenk)中分得一种新的C-去甲-D-异甾体生物碱甙,命名为伊贝碱甙B(yibeinoside B)。根据红外、质谱、氢谱和碳谱确定了结构。     

Cholinergic transmission regulates extrajunctional acetylcholine receptors

To determine the role of ACh transmission in the regulation of extrajunctional ACh receptors, we compared the effect of postsynaptic cholinergic blockade with that of surgical denervation. Blockade of ACh transmission was produced in the soleus muscles of rats by continuous local infusion of α-bungarotoxin, delivered by implantable osmotic pumps. Extrajunctional ACh receptors were measured by an ¹²⁵I-α-BuTx binding method. Our results showed an increase of extrajunctional ACh receptors quantitatively equivalent to that resulting from surgical denervation. This denervation-like effect is attributed to elimination of (i) impulse-dependent ACh transmission (which normally triggers muscle usage), and (ii) spontaneous quantal and nonquantal ACh transmission. The influence of the nerve in regulating extrajunctional ACh receptors appears to be due to the sum of these forms of ACh transmission.