Archives of Neurology

COBB, STANLEY

doi: 10.1001/archneur.1965.00460300003001pmid: 14295955

Abstract IN his book, The Growth of the Brain, published in 1895, Donaldson1 collected an important mass of data on the size of vertebrate brains. On page 123 he succinctly states: While the large absolute weight of the brain in man as compared with other animals still demands interpretation, it is evident from the foregoing facts that conclusions based upon the proportional development of the brain may be very easily misleading, because the variation in that proportion depends upon differences in body weight. In reality there is here an extremely complex problem, and although in the estimation of the probable intelligence of any animal both the absolute and relative size of the brain are factors, yet they are but two factors among a large number, and therefore, their importance can only be determined after further analysis and comparison. Such further analysis is the goal of the present contribution. To References 1. Donaldson, H.H.: Growth of the Brain: Study of Nervous System in Relation to Education , New York: Scribner's Sons, 1895. 2. Edinger. T.: Anthropocentric Misconceptions in Paleoneurology , Proc Virchow Med Soc NY 19:56, 1962. 3. Colbert, E.H.: Career of Charles R. Knight , Curator 4:352, 1961.Crossref 4. Colbert, E.H.: Weights of Dinosaurs , Amer Museum Novitates 2076:1, 1962. 5. Lull. R.S.: Stegosaurus ungulatus , Amer J Sci 4th Series , 30:361, 1910. 6. Mayr, E.: " Behavior and Systematics " in Simpson, G.G., and Roe, A., (eds.), Behavior and Evolution , New Haven: The Yale University Press, 1958. 7. Edinger, T.: Neues aus der Paläoneurologie , Paläont Ztschr 37:49, 1963.Crossref 8. Jerison, H.J.: Interpreting the Evolution of the Brain , Hum Biol 35:263, 1963. 9. Stephan, H., and Andy, O.J.: Quantitative Comparisons of Brain Structures from Insectivores to Primates , Amer Zoo 4:59, 1964. 10. Geschwind, N.: The Development of the Brain and the Evolution of Language . Monograph Series on Language and Linguistics 17:155, Washington, DC: The Georgetown University Press, 1964. 11. Rensch, B.: Evolution Above the Species Level , New York: Columbia University Press, 1960. 12. Mayr, E.: Animal Species and Evolution , Cambridge, Mass: Harvard University Press, 1963. 13. Retzius, G.: Wachst noch die Grosse der Menschlicher Gehirns infolge der Einwirkung des "Kultur"? Ztschr Morph Anthropol 18:49, 1914. 14. Mettler, F.A.: Culture and the Structural Evolution of Neural System . James Arthur Lecture, Amer Museum of Nat Hist, New York, 1956. 15. Lilly, J.G.: Man and Dolphin , Garden City, NY: Doubleday & Co., 1961. 16. Clark, W.E. Le G: The Crucial Evidence for Human Evolution , Amer Phil Soc Proc 103:159, 1959. 17. Brummelkamp, R.: Brain Weight and Body Size , Verhand Konig Nederland Akad Weten 39:58, 1940. 18. DuBois, E.: Die gesetzmassige Beziehung von Gehirnmasse zu Körpergrösse bei den Wirbeltieren , Z Morph Anthrop 18:323, 1914. 19. Sacher, G.A.: " Lifespan of Animals ," in Ciba Foundation of Colloquia on Ageing , Boston: Little, Brown & Co., 1959, vol 5. 20. Shafer, E.A., and Symington, J.: Quain's Elements of Anatomy , London: Longmans, Green, & Co., 1909. 21. Wilder, B.G.: Exhibition of, and Preliminary Note Upon, a Brain of About One Half the Average Size From a White Man of Ordinary Weight and Intelligence , J Nerv Ment Dis 30:95, 1911. 22. Hechst, B.: Ueber einen Fall von Mikrocephalie ohne geistigen Defekt Arch Psychiat 97:64, 1932. 23. Lilly, J.: Verbal Communication, lecture at Boston Soc . Psychiat and Neurol , (January 20) , 1961. 24. Quiring, D.P.: Functional Anatomy of the Vertebrates , New York: McGraw-Hill Book Co., Inc., 1950. 25. Anthony, R.: Lecons sur le cerveau . Paris: Masson, 1928. 26. Stephan, H.: Hirn-Körpergewichtsbeziehungen , Morph Jb 99:853, 1959. 27. Colbert, E.H.: Dinosaurs, Their Discovery and Their World , New York: E.P. Dutton & Co., 1961. 28. Gratiolet, P.: Sur la volume et la forme du cerveau , Bull Soc Anthropol Paris , 2:238-275, 1861. 29. Burnett, R. W.: Life Through the Ages , California: Stanford Univ. Press, 1961. 30. Owen, R.: On Dinornis: part 3 , Trans Zool Soc London , 3:345, 1848. 31. Cobb, S., and Edinger, T.: The Brain of the Emu (Dromaeus novaehollandiae, Lath.) , Breviora mus Comp Zool #170, 1962. 32. Nisbet, I.C.T.; Drury, W.H., Jr.; and Baird, J.: Weight Loss During Migration , Bird Banding 34:107, 1963. 33. Vaufrey, R.: A propos du cerveau d'un grand homme , Anthropologie 35:193-5, 1925. 34. Tower, D.B.: Correlation of Neuron Density with Brain Size , J Comp Neurol 101:19, 1954.Crossref 35. Sholl, D.A.: The Organization of the Cerebral Cortex , London: Methuen and Co., 1956. 36. Tower, D.B., and Elliot, K.A.C.: Activity of Acetylcholine System in Cerebral Cortex of Various Unanesthetized Mammals , Amer J Physiol 168:747, 1952. 37. Suwa, N.: Personal communication, 1964. 38. Buschau, G.: Gehirn und Kultur , Wiesbaden, Germany: Bergmann, 1906, chap 44. 39. von Economo, C.: Der Zellaufbau der Grosshirnrinde und die progressive Cerebration , Ergebn Physiol 29:83, 1929.Crossref 40. Lenneberg, E. H.: New Directions in the Study of Language , Boston: Mass. Inst Tech Press, 1964.

FISHMAN, ROBERT A.

doi: 10.1001/archneur.1965.00460300010002pmid: 14295956

Abstract THE NERVOUS system is influenced greatly by alterations in the concentration of the major cations in the extracellular fluid. The neurological manifestations of disorders of calcium, sodium, and potassium metabolism have been rather well defined. There are also considerable data which demonstrate that derangement of the metabolism of magnesium, the body's fourth most abundant cation, also profoundly affects the nervous system. Several recent publications serve as useful sources for comprehensive review of the biological role of magnesium.1-4 During the last four years, we have measured serum magnesium concentrations in a large number of patients in an effort to correlate changes in concentration with neurological status in a variety of clinical settings. The purpose of this communication is to review the relationship of magnesium metabolism to neural function, to document with illustrative cases the magnesium deficiency syndrome, to outline the indications for magnesium replacement therapy, to discuss magnesium intoxication References 1. Wacker, W. E. C., and Vallee, B. L.: Magnesium Metabolism , New Eng J Med 259:431-438, 1958.Crossref 2. Wacker, W. E. C., and Vallee, B. L.: " Magnesium ," in Comar, C. L., and Bronner, F. (eds.): Mineral Metabolism: Advanced Treatise , New York: Academic Press, 1964, vol 2. Elements, pt A, pp 483-511. 3. Aikawa, J. K.: Role of Magnesium in Biologic Processes , Springfield, Ill: Charles C Thomas, Publisher, 1963, p 117. 4. MacIntyre, I.: Outline of Magnesium Metabolism in Health and Disease: Review , J Chronic Dis 16:201-215, 1963.Crossref 5. Walser, M.: Ion Association: VI. Interactions Between Calcium, Magnesium, Inorganic Phosphate, Citrate and Protein in Normal Human Plasma , J Clin Invest 40:723-730, 1961.Crossref 6. Harris, W. H., and Sonnenblick, E. H.: Study of Calcium and Magnesium in Cerebrospinal Fluid , Yale J Biol Med 27:297-303, 1955. 7. Heaton, F. W.; Hodgkinson, A.; and Rose, B. A.: Observations on Relation Between Calcium and Magnesium Metabolism in Man , Clin Sci 27:31-40, 1964. 8. Horton, R., and Biglieri, E. G.: Effects of Aldosterone on Metabolism of Magnesium , J Clin Endocr 22:1187-1192, 1962.Crossref 9. Pritchard, J. A.: Use of Magnesium Ion in Management of Eclamptogenic Toxemias , Surg Gynec Obstet 100:131-140, 1955. 10. Engbaek, L.: Pharmacological Actions of Magnesium Ions With Particular Reference to Neuromuscular and Cardiovascular System , Pharmacol Rev 4:396-414, 1952. 11. Shanes, A. M.:: Electrochemical Aspects of Physiological and Pharmacological Action in Excitable Cells , Pharmacol Rev 10:59-164, 1958. 12. del Castillo, J., and Engbaek, L.: Nature of Neuromuscular Block Produced by Magnesium , J Physiol 124:370-384, 1954. 13. Lyman, C. P., and Chatfield, P. O.: Physiology of Hibernation in Animals , Physiol Rev 35:403-425, 1955. 14. Heagy, F. C.: Use of Polyvinyl Alcohol in the Colorometric Determination of Magnesium in Plasma or Serum by Means of Titan Yellow , Canad J Res 26:295-298, 1948.Crossref 15. Hirschfelder, A. D.: Clinical Manifestations of High and Low Plasma Magnesium: Dangers of Epsom Salt Purgation in Nephritis , JAMA 102:1138-1141, 1934.Crossref 16. Flink, E. B., et al: Evidences for Clinical Magnesium Deficiency , Ann Intern Med 47:956-968, 1957.Crossref 17. Hammarsten, J. F., and Smith, W. O.: Symptomatic Magnesium Deficiency in Man , New Eng J Med 256:897-899, 1957.Crossref 18. Randall, R. E., Jr.; Rossmeisl, E. C.; and Bleifer, K. H.: Magnesium Depletion in Man , Ann Intern Med 50:257-287, 1959.Crossref 19. Barnes, B. A.; Cope, O.; and Gordon, E. B.: Magnesium Requirements and Deficits: Evaluation in Two Surgical Patients , Ann Surg 152:518-533, 1960. 20. Fletcher, R. F., et al: Case of Magnesium Deficiency Following Massive Intestinal Resection , Lancet 1:522-525 ( (March 5) ) 1960.Crossref 21. Hanna, S., et al: Syndrome of Magnesium Deficiency in Man , Lancet 2:172-176, 1960.Crossref 22. Vallee, B. L.; Wacker, W. E. C.; and Ulmer, D. D.: Magnesium Deficiency Tetany Syndrome in Man , New Eng J Med 262:155-161, 1960.Crossref 23. Smith, W. O.; Hammarsten, J. F.; and Eliel, L. P.: Clinical Expression of Magnesium Deficiency , JAMA 174:77-78, 1960.Crossref 24. Balint, J. A., and Hirschowitz, B. I.: Hypomagnesemia With Tetany in Nontropical Sprue , New Eng J Med 265:631-633, 1961.Crossref 25. Wacker, W. E. C., et al: Normocalcemic Magnesium Deficiency Tetany , JAMA 180:161-163, 1962.Crossref 26. Gerst, P. H.; Porter, M. R.; and Fishman, R. A.: Symptomatic Magnesium Deficiency in Surgical Patients , Ann Surg 159:402-406, 1964.Crossref 27. Cline, W. B., Jr., and Coleman, J. V.: Treatment of Delirium Tremens , JAMA 107:404-409, 1936.Crossref 28. Flink, E. B., et al: Magnesium Deficiency After Prolonged Parenteral Fluid Administration and After Chronic Alcoholism Complicated by Delirium Tremens , J Lab Clin Med 43:169-183, 1954. 29. Suter, C., and Klingman, W. O.: Neurologic Manifestations of Magnesium Depletion States , Neurology 5:691-699, 1955.Crossref 30. Kalbfleisch, I. M., et al: Effects of Ethanol Administration on Urinary Excretion of Magnesium and Other Electrolytes in Alcoholic and Normal Subjects , J Clin Invest 42:1471-1475, 1963.Crossref 31. Fankushen, D., et al: Significance of Hypomagnesemia in Alcoholic Patients , Amer J Med 37:802-812, 1964.Crossref 32. Glickman, L. S., et al: Cerebrospinal Fluid Cation Levels in Delirium Tremens With Special Reference to Magnesium , J Nerv Ment Dis 134:410-414, 1962.Crossref 33. Randall, R. E., Jr., et al: Hypermagnesemia in Renal Failure: Etiology and Toxic Manifestations , Ann Intern Med 61:73-88, 1964.Crossref 34. Anast, C. S.: Serum Magnesium Levels in Newborn , Pediatrics 33:969-974, 1964. 35. Barbeau, A.; Jasmin, G.; and Duchastel, Y.: Biochemistry of Parkinson's Disease , Neurology 13:56-58, 1963.Crossref

REWCASTLE, NEILL B.;HUMPHREY, JOHN G.

doi: 10.1001/archneur.1965.00460300018003pmid: 14295957

Abstract O CCASIONAL vacuolated muscle fibers may occur in any myopathy. Widespread vacuolation of muscle fibers occurs in familial periodic paralysis but only rarely has it been reported in other myopathies. Unverricht12 described such a myopathy in 1891 and noted the absence of muscle-fiber regeneration. More recently, the absence of other structural changes in muscle fibers and the lack of focal inflammatory cell infiltrations have been noted in vacuolar myopathies by Pearson8 and Denny-Brown.3 Such myopathies have been reported in association with systemic lupus erythematosus,8 rheumatoid arthritis, and carcinoma and following adrenosteroid therapy,1 or prolonged chloroquine phosphate therapy.15 This report is the summary of the clinical features of a case of vacuolar myopathy associated with systemic lupus erythematosus and prolonged chloroquine therapy. Light- and electron-microscopic appearances of the myopathy have been studied from biopsy specimens before and after discontinuation of the chloroquine and institution of References 1. Adams, R.D.; Denny-Brown, D.; and Pearson, C.M.: Diseases of Muscle , ed 2: New York: Harper & Row Publishers Inc., 1962, pp 427-446. 2. Andersson-Cedergren, E.: Ultrastructure of Motor End Plate and Sarcoplasmic Components of Mouse Skeletal Muscle Fibre as Revealed by Three Dimensional Reconstructions From Serial Sections , J Ultrast Res , (suppl 1) , pp 1-191, 1959. 3. Denny-Brown, D.: Nature of Polymyositis and Related Muscular Diseases , Trans Coll Physicians Phila 28:14-29, 1960. 4. Edwards, G.A., et al: Comparative Cytophysiology of Striated Muscle With Special Reference to Role of Endoplasmic Reticulum , J Biophys Biochem Cytol 2 ( (suppl) ):43-156, 1956.Crossref 5. Fitzhugh, O.G.; Nelson, A.A.; and Holland, O.L.: Chronic Oral Toxicity of Chloroquine , J Pharmacol Exp Ther 93:147-152, 1948. 6. Humphrey, J.G., and Shy, G.M.: Diagnostic Electromyography: Clinical and Pathological Correlation in Neuromuscular Disorders , Arch Neurol 6:339-352, 1962.Crossref 7. Levy, J.A.: Vacuolar Myopathy in Lupus Erythematosus , Arq Neuropsiquiat 20:142-145, 1962.Crossref 8. Pearson, C.M., and Yamazaki, J.N.: Vacuolar Myopathy in Systemic Lupus Erythematosus , Amer J Clin Path 29:455-463, 1958. 9. Porter, K.R., and Palade, G.E.: Studies on Endoplasmic Reticulum: III. Its Form and Distribution in Striated Skeletal Muscle , J Biophys Biochem Cytol 3:269-300, 1957.Crossref 10. Porter, K.R.: Sarcoplasmic Reticulum: Its Recent History and Present Status , J Biophys Biochem Cytol 10:211-226, 1961.Crossref 11. Shy, G.M., et al: Studies on Familial Periodic Paralysis , Ex p Neurol 3:53-121, 1961.Crossref 12. Unverricht, H.: Dermatomyositis acuta , Deutsch Med Wschr 17:41-44, 1891.Crossref 13. van Breemen, V.L.: Ultrastructure of Human Muscle: II. Observations on Dystrophic Striated Muscle Fibers , Amer J Path 37:333-341, 1960. 14. Wechsler, W., and Hager, H.: Elektronenmikroskopische Untersuchungen bei myotonischer Muskeldystrophie , Arch Psychiat Nervenkr 201:668-690, 1961.Crossref 15. Whisnant, J.P., et al: Chloroquine Neuromyopathy , Proc Mayo Clin 38:501-513, 1963. 16. Zeman, W., and Donahue, S.: Fine Structure of Lipid Bodies in Juvenile Amaurotic Idiocy , Acta Neuropath 3:144-149, 1963.Crossref

KOLODNY, EDWIN H.;HASS, WILLIAM K.;LANE, BERNARD;DRUCKER, WILLIAM D.

doi: 10.1001/archneur.1965.00460300031004pmid: 14295958

Abstract REFSUM'S syndrome, a hereditary disorder of the nervous system, was first described under the title of "Heredopathia Atactica Polyneuritiformis" by Sigvald Refsum, a Norwegian neurologist, in 1946.1 It is characterized by (1) an atypical retinitis pigmentosa with hemeralopia and concentric constriction of the visual fields, (2) a chronic polyneuritis with progressive paresis in the distal parts of the extremities, (3) an elevated cerebrospinal-fluid (CSF) protein, and (4) ataxia and other cerebellar signs. Less constant manifestations are anosmia, pupillary abnormalities, deafness, ichthyosis, alterations in the electrocardiogram, and skeletal anomalies. Refsum suggested that the disease was transmitted by an autosomal recessive gene and postulated a disturbance in lipid metabolism in affected patients. The patient to be reported has offered us an opportunity to study this syndrome in detail and to report for the first time unusual morphological changes in hepatic parenchymal cells. Report of a Case A 31-year-old, single, white References 1. Refsum, S.: Heredopathia Atactica Polyneuritiformis: Familial Syndrome Not Hitherto Described; Contribution to Clinical Study of Hereditary Diseases of Nervous System , Acta Psychiat Neurol , (supp 38) , 1946. 2. Thiebaut, F.; Lemoyne, J.; and Guillaumat, L.: Deux syndromes oto-neuro-oculistiques d'origine congenitale, leur rapports avec les phacomatoses de Van der Hoeve et autres dysplasies neuro-ectodermiques , Rev Neurol 72:71, 1939. 3. Thiebaut, F.; Lemoyne, J.; and Guillaumat, L.: Maladie de Refsum . Rev Neurol 104:152, 1961. 4. Refsum, S.; Salomonsen, L.; and Skadvedt, M.: Heredopathia Atactica Polyneuritiformis in Children: Preliminary Communication , J Pediat 35:335, 1949.Crossref 5. Reese, H., and Bareta, J.: Heredopathia Atactica Polyneuritiformis , J Neuropath Exp Neurol 9:385, 1950.Crossref 6. Clark, D.B., and Critchley, M.: Heredopathia Atactica Polyneuritiformis (Refsum's Syndrome) , Proc Roy Soc Med 44:689, 1951. 7. Kjellson, L.: Refsums sjuksom , Nord Med 49:460, 1953. 8. Fleming, R.: Refsum's Syndrome: Unusual Hereditary Neuropathy , Neurology 7:476, 1957.Crossref 9. Billings, J.J.; O'Callaghan, J.; and O'Day, K.: Refsum's Syndrome: Heredopathia Atactica Polyneuritiformis , Trans Ophthal Soc Aust 17:131, 1957. 10. Ashenhurst, E.M.; Millar, J.H.D.; and Milliken, T.G.: Refsum's Syndrome Affecting a Brother and Two Sisters , Brit Med J 2:415, 1958.Crossref 11. Heycock, J.B., and Wilson, J.: Diabetes Mellitus in a Child Showing Features of Refsum's Syndrome , Arch Dis Child 33:320, 1958.Crossref 12. Edström, R.; Grontöft, O.; and Sandring, H.: Refsum's Disease—Three Siblings, One Autopsy , Acta Psychiat Neurol Scandinav , 34:40, 1959.Crossref 13. Gordon, N., and Hudson, R.E.: Refsum's Syndrome: Heredopathia Atactica Polyneuritiformis: Report of Three Cases, Including Study of Cardiac Pathology , Brain 82:41, 1959.Crossref 14. Toussaint, D.; Coers, C.; and Toppet, N.: Heredopathia Atactica Polyneuritiformis (syndrome de Refsum): Constatations clinique et biopsiques , Bull Soc Belg Ophtal 122:383, 1959. 15. Veltema, A.N., and Verjaal, A.: Sur un cas d'heredopathic ataxique polyneuritique: Maladie de Refsum , Rev Neurol 104:15, 1961. 16. Richterich, R., et al: Refsum's Syndrom (Heredopathia atactica polyneuritiformis): Ein angeborener Defekt im Lipid-Stoffwechsel mit Speicherung von 3,7,11,15-Tetramethyl-hexadecansäure , Klin Wschr 41:800, 1963.Crossref 17. Dereux, J.: La maladie de Refsum , Rev Neurol 109:599, 1963. 18. Pecker, J.; Feuvrier, Y.M.; and LeHuerov, Y.: Maladie de Refsum: Diagnostic avec certaines polyradiculonevrites a rechutes , Rev Neurol 109:39, 1963. 19. Harders, H., and Dieckmann, H.: Heredopathia atactica polyneuritiformis: Klinik und Diagnostik des Refsum-Syndroms , Deutsch Med Wschr 89:248, 1964.Crossref 20. Olesen, T.B.: Case of Heredopathia Atactica Polyneuritiformis (morbus Refsum) , Acta Psychiat Neurol Scandinav 32:83, 1957.Crossref 21. Jager, B.V., et al: Occurrence of Retinal Pigmentation, Opthalmoplegia, Ataxia, Deafness and Heart Block , Amer J Med 29:888, 1960.Crossref 22. Bastron, J.A.: " Myotonia and Other Abnormalities of Muscular Contraction Arising From Disorders of Motor Unit ," in Raymond D. Adam (ed.): Neuromuscular Disorders , Association for Research in Nervous and Mental Diseases V38: Baltimore: Williams and Wilkins, 1960. 23. Refsum, S.: Heredopathia Atactica Polyneuritiformis Reconsideracion , World Neurol 1:334, 1960. 24. Cammermeyer, J.: Symposium on Peripheral Neuropathies; Neuropathological Changes in Hereditary Neuropathies: Manifestation of Syndrome Heredopathia Atactica Polyneuritiformis in Presence of Interstitial Hypertrophic Polyneuropathy J Neuropath Exp Neurol 15:340, 1956.Crossref 25. Drucker, W.D., et al: On Function of Endocrine Glands in Myotonic Muscular Dystrophy , Amer J Med 31:94, 1961.Crossref 26. Drucker, W.D., et al: Testes in Myotonic Muscular Dystrophy: Clinical and Pathologic Study With Comparison to Klinefelter Syndrome , J Clin Endocr 23:59, 1963.Crossref 27. Refsum, S.: Personal communication to the author, 1964. 28. Kahlke, W.: Über das Vorkommen von 3,7,11, 15-Tetramethyl-hexadecansäure im Blutserum bei Refsum-Syndrome , Klin Wschr 41:783, 1963.Crossref 29. Klenk, E., and Kahlke, W.: Über das Vorkommen der 3,7,11,15-Tetramethyl-hexadecansäure (Phytan säure) in den Cholesterinestern und anderen Lipoidfraktionen der Organe bei einem Krankheitsfall unbekannter Genese (Verdacht auf Heredopathia atactica polyneuritiformis [Refsum-Syndrom] ) , Z Physiol Chem 333:133, 1963.Crossref 30. Sonnewald, Z.W., et al: 3,7,11,15-Tetramethyl-hexadecanoic Acid: Constituent of Fat , J Lipid Res 3:351, 1962. 31. Laguens, R., and Bianchi, N.: Fine Structure of Liver in Human Idiopathic Diabetes Mellitus: I. Parenchymal Cell Mitochondria , Exp Molec Path 2:203, 1963.Crossref 32. Svoboda, D.J., and Manning, R.T.: Chronic Alcoholism With Fatty Metamorphosis of Liver , Amer J Path 44:645, 1964. 33. Gonatas, N.K., et al: Case of Juvenile Lipoidoses: Significance of Electron Microscopic And Biochemical Observations of Cerebral Biopsy , J Neuropath Exp Neurol 22:557, 1963.Crossref

TSUKAGOSHI, H.;NAKANISHI, T.;KONDO, K.;TSUBAKI, T.

doi: 10.1001/archneur.1965.00460300045005pmid: 14295959

Abstract IN 1955 Wohlfart et al1 separated hereditary proximal muscular atrophy from muscular dystrophy as a new clinical entity. Kugelberg and Welander2 in 1956 gave a detailed clinical description of this syndrome, based on 12 patients, which might be summarized as follows: (1) onset of the disease from childhood to adolescence, (2) proximal muscular atrophy resembling muscular dystrophy, (3) fasciculations, (4) absence of the syndrome of bulbar palsy, (5) apparently non-sex-linked recessive form of inheritance, (6) very slow progression, and (7) neurogenic nature of the disorder according to electromyography and muscle biopsy. Similar cases have been reported by Magee and Dejong,3 and Hausmanowa-Petrusewicz et al.4 We have recently seen five patients with muscular atrophy resembling this syndrome, except for the following two points: (1) onset of the disorder in adults; and (2) presence of the syndrome of bulbar palsy in three of the five cases. These References 1. Wohlfart, G.; Fex, J.; and Eliasson, S.: Hereditary Proximal Spinal Muscular Atrophy: Clinical Entity Simulating Progressive Muscular Dystrophy , Acta Psychiat Neurol Scand 30:395, 1955. 2. Kugelberg, E., and Welander, L.: Heredofamilial Juvenile Muscular Atrophy Simulating Muscular Dystrophy , Arch Neurol Psychiat 75:500, 1956.Crossref 3. Magee, K.R., and Dejong, R.N.: Neurogenic Muscular Atrophy Simulating Muscular Dystrophy , Arch Neurol 2:677, 1960.Crossref 4. Hausmanowa-Petrusewicz, I., et al: A propos d'atrophies musculaires juveniles hérédo-familiales , Schweiz Arch Neurol Neurochir Psychiat 90:255, 1962. 5. Haase, G.R., and Shy, G.M.: Pathological Change in Muscle Biopsies From Patients With Peroneal Muscular Atrophy , Brain 83:631, 1960.Crossref 6. Lucas, G.J., and Forster, F.M.: Charcot-Marie-Tooth Disease With Associated Myopathy: Report of a Family , Neurology 12:629, 1962.Crossref 7. Ebashi, S., et al: High Creatine Phosphokinase Activity of Sera of Progressive Muscular Atrophy Patients , J Biochem (Tokyo) 46:103, 1959. 8. Dreyfus, J.C.; Schapira, G.; and Demos, J.: Etude de la créatine-kinase sérique chez de myopathies et leurs familles , Rev Franc Etud Clin Biol 5:384, 1960. 9. Okinaka, S., et al: Serum Creatine Phosphokinase , Arch Neurol 4:520, 1961.Crossref 10. Okinaka, S., et al: Cystein Stimulated Serum Creatine Kinase in Health and Disease , J Lab Clin Med 64:299, 1964. 11. Kurland, L.T., and Mulder, D.W.: Epidemiologic Investigations of Amyotrophic Lateral Sclerosis , Neurology 5:182, 249, 1955.Crossref 12. Espinosa, R.E., et al: Hereditary Amyotrophic Lateral Sclerosis: Clinical and Pathologic Report With Comments on Classifications , Neurology 12:1, 1962.Crossref 13. Roussy, G., and L,évy, G.: Sept cas d'une maladie familiale particuliére , Rev Neurol 1:427, 1926. 14. Lapresle, J.: Contribution á l'étude de la dystasie aréflexique héréditaire; état actuel de 4 des 7 cas de Roussy et Mlle Levy trente aus aprés la premiere publication de ces auteurs , Sem Hôp Paris 32:2473, 1956. 15. Strümpel, A.: Zur Lehre von der progressiver Muskelatrophie , Deutsch Z Nervenheilk 3:470, 1893. 16. Moleen, G.A.; Johnson, W.C.; and Dixon, H.H.: Familial Progressive Muscular Atrophy , Arch Neurol Psychiat 27:645, 1932.Crossref 17. Myrianthopoulos, N.C., and Brown, I.A.: Genetic Study of Progressive Spinal Muscular Atrophy , Amer J Hum Genet 6:387, 1954. 18. Magee, R.K.: Familial Progressive Bulbar-Spinal Muscular Atrophy , Neurology 10:295, 1960.Crossref 19. Davidenkow, S.: Uber die neurotische Muskelatrophie , Zbl ges Neurol Psychiat 107:259Crossref 20. 108:344, 1927. 21. Wiesendanger, M.: Uber proximale neurogene Amyotrophien mit besonderer Berücksichitigung der Electromyographie , Deutsch Z Nervenheilk 184:532, 1960. 22. Hurwitz, L.; Lapresle, J.; and Garcin, R.: Atrophie musculaire neurogéne de topographie proximale et symétrique simulant une myopathie: Etude de deux observations , Rev Neurol 104:97, 1961.

POSER, CHARLES M.;JOHNSON, MARGARET;BUNCH, LEITHA D.

doi: 10.1001/archneur.1965.00460300052006pmid: N/A

Abstract ABOUT three years ago, we investigated a family constellation in which several members were believed to have died of amyotrophic lateral sclerosis (ALS). We performed a number of clinical biochemical determinations on ten unaffected blood relatives in the hope of discovering possible abnormalities which might have some relation to the disease process. One of the procedures was column chromatographic analysis of serum amino acids. The only noteworthy observation we made was that the fasting serum arginine level appeared to be lower than normal in four of the ten individuals tested, according to the normal ranges given by Westall.1 Although we know that this might not be clinically or statistically significant, it led us to investigate the possible role of arginine in ALS. Our preliminary results are presented in this article. Methods Chromatographic Analysis.— All amino acid analyses referred to in this paper were carried out by the following References 1. Westall, R.G.: " Free Amino Acids of Body Fluids and Some Hereditary Disorders of Amino Acid Metabolism ," in Holden, J.T. (ed.): Amino Acid Pools , Amsterdam: Elsevier, 1962, p 195. 2. Moore, S.; Spackman, D.H.; and Stein, W.H.: Chromatography of Amino Acids on Sulfonated Polystyrene Resins , Anal Chem 30:1185, 1958.Crossref 3. Stegemann, H.: Determination of Amino Acid With Dithionite-Reduced Ninhydrin , Z Physiol Chem 319:102, 1960.Crossref 4. Moore, S., and Stein, W.H.: Free Amino Acids of Human Blood Plasma , J Biol Chem 211:915, 1954. 5. Armstrong, M.D., and Yates, K.N.: Amino Acid Excretion During Pregnancy , Amer J Obstet Gynec 88:381, 1964. 6. Hoffmann, R.G.: Statistics for Medical Students , Springfield, Ill: Charles C Thomas, Publisher, 1963. 7. Orten, A.U.: Intestinal Phase of Amino Acid Nutrition , Fed Proc 22:1103, 1963. 8. Cumings, J.N.: Biochemical Aspects in Discussion on Motor Neurone Disease , Proc Roy Soc Med 55:1023, 1962. 9. Logothetis, J., and Bovis, M.: Free Amino Acid Changes in the Spinal Fluid of Patients With CNS Degeneration and Chronic Epileptic Disorders , World Neurol 3:466, 1962. 10. Newberne, P.M.; Savage, J.E.; and O'Dell, B.L.: Pathology of Arginine Deficiency in the Chick , J Nutr 72:347, 1960. 11. Poser, C.M., and Bunch, L.D.: Serum Amino Acid Studies in Amyotrophic Lateral Sclerosis: II. Results of Metrecal-Arginine-Lysine Tolerance Test in US Caucasians and Guam Chamorros , Arch Neurol , to be published.

TAKO, JOZSEF;RADO, JANOS P.

doi: 10.1001/archneur.1965.00460300058007pmid: 14295960

Abstract THE EARLY enthusiastic reports concerning the treatment of herpes zoster with corticosteroids1 have been followed by a number of publications. Several have shown that herpes zoster may develop during steroid treatment2-5 and in some cases the course of the disease was severe,4 with the development of gangrene5 and generalized herpes zoster. We have reported on an outbreak of a "house epidemic" of herpes zoster among corticosteroid-treated patients.8 In the steroid-treated patient to be described below, gangrenous herpes zoster developed, followed by a recurrence, generalization of the disease, and meningoencephalitis. We believe that the case is unique and therefore merits publication. Report of Case A female nurse, 50 years of age, was admitted July 4, 1960, with generalized herpes zoster. In childhood she had suffered from scarlet fever, morbilli, and varicella. Since the age of 40 years she had been treated with insulin for References 1. Smith, R. W., Jr., and Steffensen, E. H.: ACTH and Cortisone in Treatment of Ocular Disease , New Engl J Med 245:972, 1951.Crossref 2. Dux, E., et al: Corticosteroid Therapy and Viral Diseases , Orvosképzés 38:215-226, 1963 (in Hungarian). 3. Creighton, J.S., and Dewar, W.A.: Herpes Zoster During Steroid Therapy , Brit Med J 1:648, 1959.Crossref 4. Good, R.A.; Vernier, R.L.; and Smith, R.T.: Serious Untoward Reactions to Therapy With Cortisone and Adrenocorticotropin in Pediatric Practice , Pediatrics 19:95-118, 1957. 5. Radó J.P.; Hamvas, J.; and Bikich, G.: Some Factors Involved in "Steroid Ecchymosis" Producing Effect of New Synthetic Glucocorticoids , Magy Belorv Arch 14:107-118, 1961 (in Hungarian). 6. Mosonyi, L.; Csiky, T.; and Kucsera, P.: Varicella Developing From Herpes Zoster in Course of Cortisone Therapy , Rheum Baln Allerg 4:101-105, 1963 (in Hungarian). 7. Jaffee, H.S., and Greenberg, M.S.: Herpes Zoster Resembling Acute Varicella Associated With Multiple Myeloma , JAMA 175:1008-1010, 1961.Crossref 8. Radó J.P.; Tagó, J.; Géder, L.; and Jeney, E.: Herpes Zoster House Epidemic in Steroid Treated Patients: Clinical and Viral Study , Orv Hetil 105:1266-1271, 1964 (in Hungarian). 9. Von Bókay, J.: Über den ätiologischen Zusammenhang der Narizellen mit gewissen Fallen von Herpes Zoster , Wien Klin Wschr 22:1323-1326, 1909. 10. Weller, T.H.; Witton, H.M., and Bell, J.E.: Etiologic Agents of Varicella and Herpes Zoster: Isolation, Propagation, and Cultural Characteristics in Vitro , J Exp Med 108:843-868, 1958.Crossref 11. Taylor-Robinson, D.: Chickenpox and Herpes Zoster: III. Tissue Culture Studies , Brit J Exp Path 40:521-532, 1959. 12. Taylor-Robinson, D.: Herpes Zoster Occurring in Patient With Chickenpox , Brit Med J 5187:1713, 1960.Crossref 13. Stokes, J.: " Varicella-Herpes Zoster ," in Rivers, T.M., and Horsfall, F.L., (eds.): Viral and Rickettsial Diseases of Man , ed 3, Philadelphia: J.B. Lippincott Co., 1959, pp 773-779. 14. Kilbourne, E.D., and Hosrfall, F.L., Jr.: Lethal Infection With Coxsackie Virus of Adult Mice Given Cortisone , Proc Soc Exp Biol Med 77:135-138, 1951.Crossref 15. Kilbourne, E.D.; Smart, K.M.; and Pokerny, B.A.: Inhibition by Cortisone of Synthesis and Action of Interferon , Nature 190:650-651, 1961.Crossref 16. Kain, H.K.; Feldman, C.A.; and Cohn, L.H.: Herpes Zoster Generalisatus Pneumonia , Arch Intern Med 110:98-101, 1962.Crossref 17. Rose, F.C.; Brett, E.M.; and Burston, J.: Zoster Encephalomyelitis , Arch Neurol 11:155-172, 1964.Crossref 18. Radó, J.P.; Hammer, S.; and Szilágyi, L.: Effective Treatment of Decompensated Cirrhosis of Liver by Combined Use of Glucocorticoids and Thiazide Compounds Together With Mercurial Diuretics: Mode of Potentiation With Special Reference to "Collecting Tubular Action" of Chlorothiazide Compounds , Magy Belorv Arch 15:144-157, 1962 (in Hungarian).

PEACH, BRIAN

doi: 10.1001/archneur.1965.00460300061008pmid: 14295961

Abstract IN 1891, Chiari1 described the anomalies of the hindbrain that he found in cases of congenital hydrocephalus and divided these malformations into four categories: Type 1 showed a variable displacement of the cerebellar tonsils into the upper cervical canal. This was unaccompanied by any caudal dislocation of the medulla. Type 2 showed a variable displacement of the inferior vermis of the cerebellum into the upper cervical canal, and was accompanied by a similar caudal displacement of the lower pons and medulla together with an elongated fourth ventricle. Type 3 showed a downward displacement of the medulla with herniation of the cerebellum into a high cervical meningocele. Type 4 showed hypoplasia of the cerebellum. In a further paper in 1895, Chiari2 analyzed his findings in 24 cases of congenital hydrocephalus. Seven cases fell into his type 2 category and all these were associated with a meningomyelocele. Fourteen were References 1. Chiari, H.: Über Veränderungen des Kleinhirns, des Pons und der Medulla oblongata in Folge von congenitaler Hydrocephalie des Grosshirns , Deutsch Med Wschr 27:1172, 1891.Crossref 2. Chiari, H.: Über Veränderungen des Kleinhirns, des Pons und der Medulla oblongata in Folge von congenitaler Hydrocephalie des Grosshirns , Deutsch Akad Wiss Wien , 63:71, 1895. 3. Arnold, J.: Myelocyste, Transposition von Gewebskeimen und Symbodie , Beitr Path Anat 16:1, 1894.Crossref 4. Cleland, J.: Contribution to the Study of Spina Bifida, Encephalocoele, and Anencephalus , J Anat Physiol 17:257, 1882. 5. Schwalbe, E., and Gredig, M.: Über Entwicklungsstörungen des Kleinhirns, Hirnstamms und Halsmarks bei Spina bifida , Beitr Path Anat 40:132, 1907. 6. Russell, D.S., and Donald, C.: Mechanism of Internal Hydrocephalus in Spina Bifida , Brain 58:203, 1935.Crossref 7. Lichtenstein, B.W.: Distant Neuroanatomic Complications of Spina Bifida, Hydrocephalus, Arnold-Chiari Deformity, Stenosis of Aqueduct of Sylvius etc: Pathogenesis and Pathology , Arch Neurol Psychiat 47:195, 1942.Crossref 8. Ingraham, F.D., and Scott, H.W.: Spina Bifida and Cranium Bifidum: 5. Arnold-Chiari Malformation , New Eng J Med 229:108, 1943.Crossref 9. Feigin, I.: Arnold-Chiari Malformation With Associated Analogous Malformation of Midbrain , Neurology , 6:22, 1956.Crossref 10. Cameron, A.H.: Arnold-Chiari Malformation and Other Neuroanatomical Malformations Associated With Spina Bifida , J Path Bact 73:195, 1957.Crossref 11. McConnell, A.A., and Parker, H.L.: Deformity of Hindbrain Associated With Hydrocephalus: Its Relation to Arnold-Chiari Malformation , Brain , 61:415, 1938.Crossref 12. Ogryzlo, M.A.: Arnold-Chiari Malformation , Arch Neurol Psychiat 48:30, 1942.Crossref 13. Bucy, P.C., and Lichtenstein, B.W.: Arnold-Chiari Malformation in an Adult Without Obvious Cause , Arch Neurol Psychiat 55:162, 1946.Crossref 14. Gardner, W.J., and Goodall, R.J.: Surgical Treatment of Arnold-Chiari Malformation in Adults , J Neurosurg 7:199, 1950.Crossref 15. Hurteau, E.F.: Arnold-Chiari Malformation , J Neurosurg 7:282, 1950.Crossref 16. Swanson, H.S., and Fincher, E.F.: Arnold-Chiari Malformation Without Bony Anomalies , J Neurosurg 6:314, 1949.Crossref 17. Russell, D.S.: "Hydrocephalus," in Neurology and Psychiatry in Childhood , Res Publ Assoc Nerv Ment Dis 34:160, 1954. 18. Chamberlain, W.E.: Basilar Impression (Platybasia) , Yale J Biol Med 11:487, 1939. 19. Gustafson, W.A., and Oldberg, E.: Neurologic Significance of Platybasia , Arch Neurol Psychiat 44:1184, 1940.Crossref 20. List, C.F.: Neurologic Syndromes Accompanying Developmental Anomalies of Occipital Bone, Atlas and Axis , Arch Neurol Psychiat 45:577, 1941.Crossref 21. Ray, B.S.: Platybasis With Involvement of Central Nervous System , Ann Surg 116:231, 1942.Crossref 22. Russell, D.S.: Observations on Pathology of Hydrocephalus , Med Res Counc Spec Rep (London) (No. 265) , 1949. 23. Marburg, O.: Hydrocephalus , New York, Oskar Piest, 1940. 24. Laurence, K.M., and Martin, D.: Technique for Obtaining Undistorted Specimens of Central Nervous System , J Clin Path 12:188, 1959.Crossref 25. Peach, B.: Arnold-Chiari Malformation With Normal Spine , Arch Neurol 10:497, 1964.Crossref 26. Crome, L.: Microgyria , J Path Bact 64:479, 1952.Crossref 27. Peach, B.: Cystic Prolongation of the Fourth Ventricle: Anomaly Associated with the Arnold-Chiari Malformation , Arch Neurol 11:609, 1964.Crossref

TENG, PAUL;PAPATHEODOROU, CHRISTOS

doi: 10.1001/archneur.1965.00460300070009pmid: 14295962

Abstract ARNOLD-CHIARI malformation19 denotes a congenital anomaly consisting of (1) caudal displacement of the inferior cerebellar vermis or tonsils, and (2) "hunch-back" or nodular reduplication of the medulla oblongata accompanied by descent of the lower pons, medulla, and fourth ventricle into the spinal canal. A malformation comprised of only cerebellar displacement without medullary deformity is known as Chiari type 1 or Arnold malformation, and the reverse is called Chiari type 2 or Chiari deformity.2,5,6 Schwalbe and Gredig19 claimed that the Arnold-Chiari malformation was invariably associated with a severe degree of spina bifida, and often other anomalies of the spinal column and cranium. During the last three decades, there have been several recorded cases with normal spine, but recently in a critical review, Peach17 considered none of these as Arnold-Chiari malformation but designated all of them type 1 deformity,1,3,9,11,14,16,21 and he reported a well-documented case, a References 1. Aring, C.D.: Cerebellar Syndrome in Adult With Malformation of Cerebellum and Brain Stem (Arnold-Chiari Deformity) , J Neurol Psychiat 1:100, 1938.Crossref 2. Arnold, J.: Myelocyste, Transposition von Gewebskeimen und Sympodie , Beitr Path Anat 16:1, 1894.Crossref 3. Bucy, P.C., and Lichtenstein, B.: Arnold-Chiari Malformation in Adult Without Obvious Cause , Arch Neurol Psychiat 55:162, 1946.Crossref 4. Cameron, A.H.: Arnold-Chiari Malformation and Other Neuroanatomical Malformations Associated With Spina Bifida , J Path Bact 73:195, 1957.Crossref 5. Chiari, H.: Über Veränderungen des Kleinhirns, des Pons und der Medulla Oblongata in Folge von congenitaler Hydrocephalie des Grosshirns , Dtsch Med Wschr 27:1172, 1891.Crossref 6. Chiari, H.: Über Veränderungen des Kleinhirns, des Pons und der Medulla Oblongata in Folge von congenitaler Hydrocephalie des Grosshirns , Dtsch Akad Wiss Wien 63:71, 1895. 7. Chorobski, J., and Stepien, L.: On Syndrome of Arnold-Chiari, Report of Case , J Neurosurg 5:495, 1948.Crossref 8. Feigin, I.: Arnold-Chiari Malformation With Associated Analogous Malformation of the Midbrain , Neurology 6:22, 1956.Crossref 9. Gardner, W.J., and Goodall, R.J.: Surgical Treatment of Arnold-Chiari Malformation in Adults , J Neurosurg 7:199, 1950.Crossref 10. van Houveninge Graftdijk, C.J.: Over Hydrocephalus, dissertation, Leiden, 1932. 11. Hurteau, E.F.: Arnold-Chiari Malformation , J Neurosurg 7:282, 1950.Crossref 12. Ingraham, F.D., and Scott, H.W., Jr.: Spina Bifida and Cranium: 5. Arnold-Chiari Malformation , New Eng J Med 229:108, 1943.Crossref 13. Lichtenstein, B.W.: Distant Neuroanatomic Complications of Spina Bifida (Spinal Dysraphism) , Arch Neurol Psychiat 47:195, 1942.Crossref 14. McConnell, A.A., and Parker, H.L.: Deformity of Hindbrain Associated With Hydrocephalus: Its Relation to Arnold-Chiari Malformation , Brain 61:415, 1938.Crossref 15. Malis, L.I.; Cohen, I.; and Gross, S.W.: Arnold-Chiari Malformation , Arch Surg 63:783, 1951.Crossref 16. Ogryzlo, M.A.: Arnold-Chiari Malformation , Arch Neurol Psychiat 48:30, 1942.Crossref 17. Peach, B.: Arnold-Chiari Malformation With Normal Spine , Arch Neurol 10:497, 1964.Crossref 18. Russell, D.S.: Observations on Pathology of Hydrocephalus , Medical Research Council Report No. 265, His Majesty's Stationary Office, 1949. 19. Schwalbe, E., and Gredig, M.: Über Entwicklungsstörungen des Kleinhirns, Hirnstamms und Halsmarks bei Spina bifida , Beitr Path Anat 40:132, 1907. 20. Scoville, W.B., and Sherman, I.J.: Platybasia , Ann Surg 133:496, 1951.Crossref 21. Swanson, H.S., and Fincher, E.F.: Arnold-Chiari Deformity Without Bony Anomalies , J Neurosurg 6:314, 1949.Crossref

NIEDERMEYER, ERNST

doi: 10.1001/archneur.1965.00460300073010pmid: 14295963

Abstract THERE are only scanty reports in the literature dealing with sleep electroencephalogram (EEG) records in petit mal epilepsy. This can be easily understood since waking records are so frequently diagnostic or highly informative in this condition. It has been, however, pointed out by Gibbs and Gibbs1 that, in petit mal epileptics, seizure discharges do occur more frequently in sleep (89% of their cases) than in the waking state (84%). The Gibbses felt that the petit mal discharge is usually altered in sleep and may be replaced by a "grand mal component." Passouant et al2 investigated the reactivity to auditory stimuli during the sleep of petit mal epileptics. A more recent study from the Montpellier School by Delange et al3 dealt with nocturnal sleep in six patients with petit mal epilepsy; these authors showed suppression of paroxysmal bursts during the stage of generalized desynchronization in "paradoxical sleep." References 1. Gibbs, F., and Gibbs, E.: Atlas of Electroencephalography , ed 2, Reading, Mass: Addison-Wesley Publishing Company, Inc., 1952, vol 2. 2. Passouant, P.; Cadilhac, J.; and Philippot, M.: Rhythmicité du petit mal au cours du sommeil, décharges rhythmiques généralisées et localisées , Rev Neurol 84:659-663, 1951. 3. Delange, M., et al: Étude du sommeil de nuit au cours d'épilepsies centrencéphaliques et temporales , Rev Neurol 106:106-113, 1962. 4. Gastaut, H.: The Epilepsies: Electro-clinical Correlations , Springfield, Ill: Charles C Thomas, Publisher, 1954. 5. Niedermeyer, E.: Multiple Spikes and Multiple Spike-wave Complexes in Sleep EEGS, Proceedings of American EEG Society, Santa Fe, NM, October, 1964. 6. Schwartz, B. A.; Guilbaud, G.; and Fischgold, H.: Single and Multiple Spikes in Night Sleep of Epileptics , Electroenceph Clin Neurophysiol 16:56-67, 1964.Crossref 7. Hertoft, P.: Clinical, Electroencephalographic and Social Prognosis in Petit Mal Epilepsy , Epilepsia 4:298-314, 1963.Crossref