Fulminant leukemic polyradiculoneuropathy in a case of B-cell prolymphocytic leukemia. A clinicopathologic report.

A 52 year old man with a 10-month history of B-cell prolymphocytic leukemia (PLL) died of an apparent acute fulminant polyradiculoneuropathy, a condition generally attributed to paraneoplastic complication. The pathologic examination disclosed diffuse leukemic infiltrations of the peripheral nervous system. It is suggested that this particularly aggressive form of B-cell chronic prolymphocytic leukemia presented a constellation of features that promoted the invasion of the peripheral nervous system by way of the bloodstream and may explain the unusual clinical presentation.

Magnetic resonance imaging of periventricular hyperintensity in a Veterans Administration hospital population.

Using a beta-permanent magnet (Fonar 3000), we evaluated magnetic resonance imaging of periventricular hyperintensity (PVH) patterns in 60 patients (59 white, 58 men) from the Veterans Administration Hospital, North Chicago, Ill. Eighty percent of the patients had evidence of PVH, which increased significantly in patients older than age 50 years. In addition to age, history of atherosclerotic cardiovascular disease and extracranial malignancy showed a significant association with PVH. A linear correlation was observed between the severity of PVH and an abnormal neurologic examination. In this retrospective, uncontrolled study, we concluded that PVH incidence among white men is high and increases with age or the presence of cardiovascular disease or extracranial malignancy. The degree of PVH positively correlates with abnormal neurologic evaluation and additional abnormalities seen on magnetic resonance imaging. However, the disease-related specificity is as yet undetermined.

Carbon monoxide toxicity: MR imaging in the brain.

Two cases of magnetic resonance (MR) imaging of intracranial abnormalities resulting from carbon monoxide toxicity are presented. Both cases demonstrate lesions in the globus pallidi of high MR signal intensity that correspond to previously reported abnormalities; the second case illustrates bilateral cortical high signal intensity lesions. The superiority of MR imaging in comparison with computed tomography is demonstrated in one case. The known effects of carbon monoxide toxicity in the brain are correlated with the findings of MR imaging.

Early diagnosis of spinal-epidural metastasis by magnetic resonance imaging.

Sixteen patients with various types of cancer who developed pain along the axial spine were prospectively studied by magnetic resonance imaging (MRI). The studies were performed with a Fonar Beta-3000 (Fonar Co., Melville, NY) permanent magnet operating at 0.3 Tesla (T). Detailed neurologic examinations were followed by bone x-rays, bone scans, and MRI. In 12 patients there were focal neurologic findings. Bone x-rays and bone scans were diagnostic for metastatic disease in 10 cases; MRIs were consistent with metastatic spinal-epidural disease in all 16 patients. Magnetic resonance imaging revealed bone involvement in three patients whose x-rays and bone scans were both negative indicating a higher degree of sensitivity. In six patients thecal compression was obvious on MRI; in three of these previous myelograms had been interpreted as negative. These early results suggest that MRI can serve as a useful tool for diagnosing early spinal-epidural metastases.

Recurrent multiple cranial nerve palsies (Tolosa-Hunt plus?).

Twelve patients with recurrent cranial nerve palsies in whom no focal cause was found were seen during a period of 4 years. The literature is reviewed, and the relationship and similarity to Tolosa-Hunt syndrome is discussed. Early recognition is important since the response to steroids, although not specific, is rapid in most patients, and the prognosis for complete recovery is relatively good.

Erythrocyte membrane lysophospholipase activity in muscular dystrophy.

The membrane lysophospholipase activity of erythrocytes obtained from Duchenne muscular dystrophy patients was lower than that of erythrocytes from age-matched normal boys. On the other hand, the membrane enzyme activity of erythrocytes from myotonic dystrophy patients was not different from that of their age- and sex-matched controls. Dipyridamole (0.1 mM) and glycerol 3-phosphorylcholine (2 mM) had no significant effect on these enzyme activities. These results suggest that membrane lysophospholipid metabolism may be altered in Duchenne muscular dystrophy but not in myotonic dystrophy.

Lysophosphatidylcholine-induced lysis of erythrocytes in Duchenne and myotonic dystrophies and in Huntington's disease.

Erythrocytes from Duchenne dystrophy patients lysed more readily than red cells from age-matched normal boys when lysophosphatidylcholine (LPC) concentrations that caused 50% lysis were compared. Erythrocytes from myotonic dystrophy patients appeared to be more resistant than cells from age-matched normal adults at certain medium LPC concentrations. Erythrocytes from patients with Huntington's disease showed no significant differences from erythrocytes of normal adults. Thus, the manner in which erythrocytes respond to LPC may reflect the putative membrane alterations in these diseases. Inhibition of LPC-induced lysis by 0.1 mM dipyridamole was observed in all groups. Since this agent did not inhibit LPC lysis at 0 degrees C, its action at 37 degrees C could be related to activation of a membrane enzyme. On the other hand, dipyridamole decreased osmotic fragility at 0 degrees C and 37 degrees C indicating that a physical change in membrane structure may be the primary alteration produced by this agent.

Erythrocyte phosphate content in Huntington's disease.

Total phosphate was higher in erythrocytes of patients with Huntington's disease (HD) than in erythrocytes of age- and sex-matched normal adults. A more rapid rate of organic phosphate degradation was also noted in HD erythrocytes during metabolic depletion. These variations may be associated with alterations of membrane permeabilities of HD erythrocytes to inorganic phosphate and to cations.

Erythrocytes in muscular dystrophy. Investigation with 31P nuclear magnetic resonance spectroscopy.

Phosphorus 31 nuclear magnetic resonance (31P NMR) signals were recorded from intact human erythrocytes for 16 hours. Total phosphate concentration, which was estimated as the sum of the individual 31P signals, was 25% lower in erythrocytes from men with myotonic dystrophy than in control erythrocytes. The inorganic-phosphate fraction contained the highest average phosphate concentration over the 16-hour period, and made the major contribution to the difference in total phosphate between the two groups. This result was not observed in erythrocytes from either women with myotonic dystrophy or patients with Duchenne's dystrophy and may be due to a change in cell membrane permeability to inorganic phosphate, which leads to lower steady-state concentrations of the intracellular phosphates.

Nemaline rod myopathy and Charcot-Marie-Tooth disease. Report of a case in a 10-year-old girl.

A 10-year-old girl studied with genetic, clinical, electrodiagnostic, and histopathologic methods showed evidence for both nemaline rod myopathy and Charcot-Marie-Tooth disease. Although Charcot-Marie-Tooth disease was documented in the family, no other members were found to have clinical and electrodiagnostic evidence for a primary myopathy.

Erythrocyte metabolism in muscular dystrophy.

Erythrocytes from patients with Duchenne's and myotonic muscular dystrophies contained more adenosine triphosphate (ATP) and produced more lactate than did normal erythrocytes in incubation studies conducted in vitro at an initial pH of 7.4. Since the same results were obtained in two different genetic dystrophies, these metabolic variations appear to be secondary to the primary changes occurring in these diseases. Following ouabain treatment, ATP content increased and lactate production decreased in erythrocytes from both dystrophies. This result differs from one reported earlier in experiments conducted at alkaline pH.