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Biomedical subjects

D A Krendel

Publications and source records attributed to D A Krendel.

31 records · Page 2Linked to original sources

Myasthenia gravis presenting as weakness after magnesium administration.

We studied a patient with no prior history of neuromuscular disease who became virtually quadriplegic after parenteral magnesium administration for preeclampsia. The serum magnesium concentration was 3.0 mEq/L, which is usually well tolerated. The magnesium was stopped and she recovered over a few days. While she was weak, 2-Hz repetitive stimulation revealed a decrement without significant facilitation at rapid rates or after exercise, suggesting postsynaptic neuromuscular blockade. After her strength returned, repetitive stimulation was normal, but single fiber EMG revealed increased jitter and blocking. Her acetylcholine receptor antibody level was markedly elevated. Although paralysis after magnesium administration has been described in patients with known myasthenia gravis, it has not previously been reported to be the initial or only manifestation of the disease. Patients who are unusually sensitive to the neuromuscular effects of magnesium should be suspected of having an underlying disorder of neuromuscular transmission.

Adult↗

Biopsy-proven cerebral vasculitis associated with cocaine abuse.

We report cerebral vasculitis in 2 cocaine users who developed symptoms (transient blindness and persistent headache) while smoking "crack," followed by progressive widespread cerebral dysfunction with focal signs over the next few weeks. One patient had smoked crack exclusively, and the other also used cocaine intravenously. Sedimentation rates were elevated and HIV titers negative. Arteriography was normal in 1 patient and in the other showed multiple large-vessel occlusions without beading. Brain biopsy showed vasculitis involving small vessels in both patients. Multinucleated cells were present in the neuropil, but there were no granulomas or evidence of infection. One patient improved significantly with corticosteroid treatment, and made a good recovery. The other died despite corticosteroid and cyclophosphamide treatment.

Adult↗

Sural nerve biopsy in chronic inflammatory demyelinating polyradiculoneuropathy.

We compared histologic features of sural nerve biopsies in 14 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with those in other forms of neuropathy. In CIDP endoneurial pericapillary cellular infiltrates were found in 4 patients (29%), onion bulbs in 5 patients (36%), and predominant demyelination in 7 patients (50%). None of these abnormalities was specific, but cellular infiltrates and onion bulbs appear to be diagnostically useful when combined with clinical information. To detect macrophage infiltration of myelin, cell nuclei were counter-stained in 20 teased fiber preparations. Nine patients with CIDP had a significantly higher mean number of cells per centimeter of teased fiber than 11 patients with other neuropathies. Despite overlap, significant infiltration of myelin detected by this method suggests CIDP in an appropriate clinical setting.

Biopsy↗

Distal vacuolar myopathy with complete heart block.

We describe a 26-year-old man with a predominantly distal myopathy and complete heart block. A muscle biopsy specimen demonstrated numerous vacuoles, which, by electron microscopy, contained membranous whorls. Filamentous inclusions characteristic of inclusion body myositis were not seen. It is important to be aware that life-threatening cardiac disease may occur in this setting.

Adult↗

Single fiber electromyography in chronic progressive external ophthalmoplegia.

We have reviewed the electromyographic (EMG) studies of 17 patients with chronic progressive external ophthalmoplegia (CPEO). In 13 of 17 patients, conventional concentric needle EMG demonstrated a "myopathic" pattern, usually predominating in the shoulder muscles. Single-fiber EMG showed increased jitter and/or blocking in at least one muscle in 13 of 16 patients. Jitter was increased in the frontalis muscle in 10 of 13 patients and in an arm muscle in 5 of 12. When both muscles were tested, jitter was greater in the frontalis muscle in 5 patients and in the arm muscle in 2. These observations demonstrate that it may be difficult to distinguish myasthenia gravis from CPEO by EMG. The frequency with which abnormal jitter is found in CPEO suggests that, in addition to a mild generalized myopathy, a primary defect in neuromuscular transmission may be present.

Chronic Disease↗

Infiltrative polyneuropathy due to acute monoblastic leukemia in hematologic remission.

A 66-year-old man with acute monoblastic leukemia developed acute polyneuropathy with quadriplegia, autonomic instability, and respiratory failure while he was in hematologic remission following both systemic and intrathecal chemotherapy. Autopsy revealed dense infiltration of somatic and autonomic peripheral nerves, sparing the meninges. There was a small peripheral infiltrate in one of four dorsal root ganglia, but, otherwise, sensory and autonomic ganglia were normal. The blood-nerve barrier may allow some malignant cells to escape cytotoxic agents. The epineurium and ganglia lack a blood-tissue barrier, and malignant cells could have been eradicated at those sites.

Action Potentials↗

Hereditary motor and sensory neuropathy, X-linked: a half century follow-up.

The existence of an X-linked sensorimotor peripheral neuropathy has been debated. We reevaluated the original family, and present data on 13 affected males and 25 obligate or probable heterozygous females, documenting the devastating nature of the disease in the men and the extremely variable degree of clinical involvement in the carriers. Use of DNA probes indicates that the gene lies in the DXYS1-p58-1 region of the X-chromosome.

Adolescent↗

Influence of type of carbohydrate on atherosclerosis in baboons fed semipurified diets plus 0.1% cholesterol.

Five groups of six (three male, three female) baboons (Papio ursinus) were maintained for 17 months on a semipurified diet containing 40% carbohydrate, 25% casein, 13.9% coconut oil, 0.1% cholesterol, 15% cellulose, 5% salt mix (USP XIV) and 1% vitamin mix. The carbohydrates fed were: fructose, sucrose, starch, glucose, and lactose. A fifth group was used as control and was fed bread, fruit, and vegetables. Serum, liver, and tissue lipids were analyzed at the end of the feeding period as were cholesterol absorption (as 3H-cholesterol) and synthesis (from 14C-mevalonic acid). Serum cholesterol and beta-lipoprotein levels were elevated in all the test groups compared to final control levels or to starting levels for all the baboons. Average serum cholesterol levels of the test groups were not significantly different. Liver lipids were elevated in all test groups except that fed glucose. Baboons on the test diets absorbed more exogenous cholesterol (3H) but biosynthesis of this sterol was not inhibited. The ratio of biliary primary/secondary bile acids was below normal levels only in the animals fed fructose and sucrose. Cholesteryl ester fatty acid spectra of serum and liver reflected the dietary fat. Fecal weight was 69% higher in lactose fed animals and 31% lower in sucrose fed animals than in the controls. The ratio of endogenous or exogenous neutral/acid steroids was considerably lower in the fructose-fed baboons than in the other animals. On this diet average aortic sudanophilia (percentage of surface) was: fructose, 11.3; sucrose, 10.4; starch, 21.3; glucose, 17.2 lactose, 65.8; and control, 1.4. Gross atheromatous lesions were seen in five of six baboons fed lactose; three of six baboons fed fructose; two of six baboons fed sucrose, and one of six baboons fed starch. In a second experiment three groups of baboons were fed the control diet, the semipurified diet in which the carbohydrate was lactose, and the semipurified diet containing lactose plus 0.1% cholesterol for 8.5 months. Serum lipids were elevated in the two test groups but liver lipids were not significantly different from control levels. Average aortic sudanophilia (percentage of area) was: lactose, 2.2; lactose-cholesterol, 20.8; and control, 0.3%. One of the six baboons in the lactose-cholesterol group had visible atherosclerotic lesions. These experiments represent the first successful attempt to produce severe atherosclerosis in baboons by dietary means alone.

Animals↗

Adverse effect of verapamil in a patient with the Lambert-Eaton syndrome.

A patient with the Lambert-Eaton syndrome (LES) and small cell lung cancer developed respiratory failure several hours after verapamil was given. Improvement in respiratory function did not occur when guanidine was given, but was delayed until verapamil was discontinued 3 days later. Although other factors may have contributed to the clinical deterioration of our patient, the temporal relationship to verapamil and the theoretical danger of calcium channel blockade lead us to believe that the drug should be used cautiously in LES.

Action Potentials↗