H. Richard Tyler

Neurologic disorders in renal failure

Abstract In her excursion through the looking glass, Alice once remarked I dont quite know yet. might. This paper can best be regarded as a look all around a very complicated and partially obscured landscape, in the hope that some features of importance may catch the attention of those who might be prepared to make a more systematic exploration of the field. With the many neurologic problems that are posed by patients with metabolic disorders, this is an urgent need that can no longer be ignored.

Abnormalities of Perception with Defective Eye Movements (Bálint's Syndrome)

Summary A patient is reported with a defect in visual perception similar to that originally described by Balint. Associated disorders of ocular motility and visual motor coordination were also present. There were significant defects in both spatial and temporal reception of visual stimuli resulting in a very restricted effective field. A significant disorder in visual attending to peripheral stimuli resulted in a very defective pattern of visual searching of the environment. It is suggested that this abnormality of visual exploration in conjunction with the temporal and spatial defects play a significant role in the distorted visual perceptions. The defects noted seemed adequate to explain the patients behavior without the necessity of postulating an “agnosic” defect.

The renal lesion in Wilson's disease

Abstract A case of hepatolenticular degeneration is reported in which the renal defect was studied physiologically and pathologically. Lesions noted chiefly in the proximal tubules in both light and electron microscopy correlated well with the functional deficits. No direct relationship between copper deposits and the pathologic lesion could be made out.

The association of amyotrophic lateral sclerosis (motor neuron disease) and carbohydrate intolerance, a clinical study☆

Abstract Eleven of 13 consecutive patients with amyotrophic lateral sclerosis (ALS) exhibited an abnormality in glucose utilization, detectable by oral and intravenous glucose tolerance tests and blood glucose response to tolbutamide. This abnormality persisted despite forced high carbohydrate intake and was present in patients with either severe or mild form of ALS. The explanation of this abnormality is not apparent.

PARANEOPLASTIC SYNDROMES OF NERVE, MUSCLE, AND NEUROMUSCULAR JUNCTION

Since the turn of the century it has been realized that patients with neoplastic disease often had neurological signs or symptoms not directly attributable to malignant invasion of the peripheral or the central nervous system.1-6 In the last two decades, after the reports of Bro~wer ,~ -* Greenfield,”ll Denny-Brown.12 and Brain and c o l l e a g u e s ~ ~ ~ 5 it has become generally recognized that peripheral neuropathies, radiculopathies, posterior root degenerations. myelopathies, cerebeller degenerations, and dementias occur in association with neoplastic disease. Polymyositis and myasthenic syndromes are also found with some frequency in these patients, There have been many recent reviews 14-20 of this subject. Recognition of these syndromes is important, because they can cause disability out of proportion .to the neoplastic disease present. Their treatment may rarely result in striking clinical improvement. The patient may present with one of these syndromes due to a yet undiagnosed malignancy. Awareness of the relationship of the syndrome to neoplasias may prove most rewarding in the diagnosis of early and surgically treatable malignancies. I have elected to separate the various systems (i.e.. muscle, nerve, and so on) to facilitate discussion of the different entities. It should be stressed at the onset, however, that this breakdown is artificial, and in any given case these syndromes may, and frequently do, overlap. Patients who have pure involvement of a single system are less common than those in whom there is a contamination of one syndrome with another. This is often helpful diagnostically. Combined syndromes may suggest the possibility that one is working with a patient who has an underlying malignancy. In male patients with cancer of the lung and in female patients with carcinoma of the ovary, the incidence of these syndromes may be as high as 16%. With most tumors, it is under 5%.20-21 Oat cell tumor of the lung is unusually prominent as a type of tumor associated with the clinical syndromes to be described.S2

Enzyme distribution in human brain. Lactic and malic dehydrogenases.

SummaryDistributions of enzymatic activities of lactic dehydrogenase and malic dehydrogenase have been studied in human brain. Their activities are increased in gray matter as compared to white matter. There are areas where concentrations of these enzymes seem consistently elevated, with higher concentration of lactic dehydrogenase in caudate nuclei, as compared to other gray matter, and optic nerve as compared to other white matter. In some areas lactic dehydrogenase activity shows no correlation with malic dehydrogenase activity. The significance of these variations is not as yet apparent.Summary Distributions of enzymatic activities of lactic dehydrogenase and malic dehydrogenase have been studied in human brain. Their activities are increased in gray matter as compared to white matter. There are areas where concentrations of these enzymes seem consistently elevated, with higher concentration of lactic dehydrogenase in caudate nuclei, as compared to other gray matter, and optic nerve as compared to other white matter. In some areas lactic dehydrogenase activity shows no correlation with malic dehydrogenase activity. The significance of these variations is not as yet apparent.