Archives of Neurology

Bullock, Theodore H.

doi: 10.1001/archneur.1975.00490440023001pmid: 1122179

This article is only available in the PDF format. Download the PDF to view the article, as well as its associated figures and tables. Abstract Since considerable discussion about the scientific merits and ethics of psychosurgery recently has appeared in professional and general publications, it may interest readers of the Archives to know the views of neurological scientists about the subject. The Society for Neuroscience, with headquarters in Bethesda, is a professional association of scientists active in any of the disciplines converging on the nervous system—from anatomy, behavior, and chemistry to zoology! Most of its members are not physicians. Regular members are residents of Canada, Mexico, and the United States, chiefly the latter. In November 1973, the membership was 2,954. The Neuroscience Newsletter, (vol 5, No. 2) released in August by the Society for Neuroscience, reports the results of a mail poll of the membership on four questions about psychosurgery. The society held its annual meeting Nov 7 to 10, 1973, in San Diego, Calif, attended by over 2,500 members and guests. At the business meeting

Derakhshan, Iraj

doi: 10.1001/archneur.1975.00490440025002pmid: 1122180

Abstract Since first described by Negri in 1903, the Negri body has been regarded as a pathognomonic finding in signifying the presence of rabies encephalitis. Negri bodies (light microscope) were found in the brain of a patient with conclusive evidence against the presence of rabies encephalitis. This case provided the opportunity for a pertinent review of the literature In bringing the subject into a reasoned perspective. A definitive etiologic diagnosis of rabies requires the use of electron microscopical or immunofluorescent methods or both. References 1. vanRooyan CE, Rhodes AJ: Virus Diseases of Man . New York, Thomas Nelson & Sons, 1948. 2. Kierkel ES: Rapid microscopic examination for Negri bodies and preparation of specimens for biological test , in Laboratory Techniques in Rabies , ed 2. Geneva, World Health Organization, 1966. 3. Brunberg JA, Bell WE: Reye syndrome: An association with type 1 vaccine-like poliovirus . Arch Neurol 30:304-306, 1974.Crossref 4. Collins DN: Ultrastructural study of intranuclear inclusions in the exocrine pancreas in Reye's syndrome . Lab Invest 30:333-340, 1974. 5. Matsumoto S: Electron microscope studies of rabies virus in mouse brain . J Cell Biol 19:565-576, 1963.Crossref 6. Downie AW: Poxvirus group , in Horsfall FL, Tamm I (eds): Viral and Ricketsial Diseases of Man , ed 4. Philadelphia, JB Lippincott Co, 1965, p 938. 7. Tyzzer EE: Etiology and pathology of vaccinia . J Med Res 11:181-227, 1904. 8. Councilman WT, Magrath GB, Brinckerhoff WR: Anatomy and histology of variola . J Med Res 11:12-133, 1904. 9. Calkins GN: The life history of cytoryctes variolae, Guarnieri . J Med Res 11:136-172, 1904. 10. Marchan J: Infectious ectromelia . J Pathol 33:713-728, 1930.Crossref 11. Goodpasture EW: Cellular inclusions and the etiology of virus diseases . Arch Pathol 7:114-134, 1929. 12. Goodpasture EW, Woodruff AM, Buddingh GJ: Vaccinial infection of chorio-allantoic membrane of the chick embryo . Am J Pathol 8:271-283, 1932. 13. Lillie RD: Small pox and vaccinia . Arch Pathol 28:241-291, 1930. 14. Wolman M: The nature of viral inclusion bodies and their differentiation from non-viral inclusions . Experientia 11:22-24, 1955.Crossref 15. Downie AW, Dumbell KR: Pox viruses . Ann Rev Microbiol 10:237-252, 1956.Crossref 16. Acton HW, Harvy WF: The nature and specificity of the Negri bodies . Parasitology 4:255-271, 1911.Crossref 17. Bezancon F, Philibert A: Patologie Medicale , ed 2. Paris, Masson et Cie, 1935, vol 2. 18. Boswell FW: Electron microscope studies of viral elementary bodies . Br J Exp Pathol 28:251-260, 1947. 19. Blank H, David C, Collins C: Electron microscopy in the diagnosis of cutaneous viral infections . Br J Dermatol 83:69-80, 1970.Crossref 20. Miyamoto K, Matsumoto S: The nature of the Negri body . J Cell Biol 27:677-682, 1965.Crossref 21. Matsumoto S: Rabies virus , Smith KM, Lauffer MA, Bang FB (eds): in Advances in Virus Research . New York, Academic Press, 1970, vol 16, pp 257-297. 22. Mendall JR, Markesberg WR: Neuronal intracytoplasmic hyaline inclusions . J Neuropathol Exp Neurol 30:233-239, 1971.Crossref 23. Culebras A, Feldman RG, Merk FB: Cytoplasmic inclusion bodies within neurons of the thalamus in myotonic dystrophy . J Neurol Sci 19:319-329, 1973.Crossref 24. Wolman M: Pathologic findings in human small pox . Am J Clin Pathol 21:1127-1138, 1932. 25. Kling C, Wassen E, Faahraeus J: Recherches sur l'etiologie de l'encephalite post-vaccinale . C R Soc Biol 109:1337-1344, 1932. 26. Condrea P: Contribution anatomopathologiques, a l'etude de la vaccine cerebrale . C R Soc Biol 86:895-900, 1922. 27. Frothigham L: The rapid diagnosis of rabies . J Med Res 14:471-489, 1906. 28. Johnson HN: Rabies virus , in Horsfall FL, Tamm I (eds): Viral and Ricketsial Diseases of Man , ed 4. Philadelphia, JB Lippincott Co, 1965, pp 814-835. 29. Innes JRM, Saunders LZ: Comparative Neuropathology . New York, Academic Press, 1962, pp 384-394. 30. Mackenzie I, Abrahams A: in Douthwaite AH (ed): Frenche's Index of Differential Diagnosis . Bristol, England, John Wright & Sons Ltd, 1967, pp 218, 219, 246-247. 31. Berntsen CA, Stevensen LD: Human rabies . J Neuropathol Exp Neurol 12:179-185, 1953. 32. Ford FR: Diseases of the nervous system in infancy, childhood and adolescence . Springfield, Ill, Charles C Thomas Publisher, 1960, pp 667-668. 33. Butterworth JJ: Small pox . Lancet 2:1426-1428, 1938.Crossref 34. Ricketts TF, Byles JB: The Diagnosis of the Small Pox . London, Cassel & Co, 1908, p 63. 35. Nieberg KC, Blumberg JM: Viral encephalitidies , in Minckler J (ed): Pathology of the Nervous System . New York, McGraw-Hill Book Co, 1972. 36. Goldwasser RA, Kissling RE: Fluorescent antibody staining of street and fixed rabies virus antigens . Proc Soc Exp Biol Med 98:219-223, 1958.Crossref 37. Dupont JR, Earle KM: Human rabies encephalitis . Neurology 15:1023-1034, 1965.Crossref 38. Knutti RE: Acute ascending paralysis and myelitis due to rabies virus . JAMA 93:745-758, 1929. 39. Helmbolt CF, Jungherr EL: Distemper complex in wild carnivors simulating rabies . Am J Vet Res 16:463-469, 1955. 40. Anderson JA, Daly FT, Kidd JC: Human rabies after antiserum and vaccine postexposure treatment: Case report and review . Ann Intern Med 64:1297-1302, 1966.Crossref

Tanaka, Ryuichi;Iwasaki, Yuzo;Koprowski, Hilary

doi: 10.1001/archneur.1975.00490440030003pmid: 1122181

Abstract Twenty-four plaques obtained at autopsy from the brains of six patients with multiple sclerosis were examined under an electron microscope. Specimens for ultrastructural study were processed from the unfixed brain tissues at the time of autopsies. Intranuclear filaments with a diameter of 150 to 260 Angstroms of possibly two different types were seen in the macrophages and in unidentified mononuclear cells that were infiltrating the perivascular regions of four plaque areas from three patients. Intracytoplasmic reticular filaments with a diameter of 100 to 130 A were seen in the astrocytes and in unidentified cell processes in five plaques from three cases. A possible link between these intranuclear filaments and paramyxovirus is suggested. References 1. ter Meulen V, et al: Fusion of cultured multiple sclerosis brain cells with indicator cells: Presence of nucleocapsids and virions and isolation of parainfluenza-type virus . Lancet 2:1-5, 1972.Crossref 2. Iwasaki Y, et al: Morphogenesis and structure of a virus in cells cultured from brain tissue from two cases of multiple sclerosis . Lab Invest 28:494-500, 1973. 3. Prineas J: Paramyxovirus-like particles associated with acute demyelination in chronic relapsing multiple sclerosis . Science 178:760-763, 1972.Crossref 4. Watanabe I, Okazaki H: Virus-like structure in multiple sclerosis . Lancet 2:569-570, 1973.Crossref 5. Dubois-Dalcq M, et al: Acute multiple sclerosis: Electron microscopic evidence for and against a viral agent in the plaques . Lancet 2:1408-1411, 1973.Crossref 6. Raine CS, Powers JM, Suzuki K: Acute multiple sclerosis: Confirmation of "paramyxoviruslike" intranuclear inclusions . Arch Neurol 30:39-46, 1974.Crossref 7. Oyanagi S, et al: Comparison of subacute sclerosing panencephalitis and measles virus: An electron microscope study . J Virol 7:176-187, 1971. 8. Raine CS, et al: Ultrastructural study of long-term infection in cultures of hamster dorsal-root ganglion . J Virol 8:318-329, 1971. 9. Faris AA, Martinez AJ: Primary progressive multifocal leukoencephalopathy: A central nervous system disease caused by a slow virus . Arch Neurol 27:357-360, 1972.Crossref 10. Sinkovics JG, et al: A rapidly fatal case of systemic lupus erythematosus: Structures resembling viral nucleoprotein strands in the kidney and activities of lymphocytes in culture . Texas Rep Biol Med 27:887-908, 1969. 11. Archibald RWR, et al: Measles pneumonia and the nature of the inclusion-bearing giant cells: A light and electron microscope study . J Pathol 103:27-34, 1971.Crossref 12. Kuhn NO, Harford CG: Electron microscopic examination of cytoplasmic inclusion bodies in cells infected with parainfluenza virus, type 2 . Virology 21:527-530, 1963.Crossref 13. Reczko E, Bögel K: Elektronenmikroskopische Untersuchungen über das Verkalten eines vom Kalb isolierten Parainfluenza-3 virus in Kälbernierenzellkulturen . Arch Gesamte Virusforsch 12:404-420, 1962.Crossref 14. Dales S, Franklin RM: A comparison of the changes in fine structure of L cells during single cycles of viral multiplication, following their infection with the viruses of mengo and encephalomyocarditis . J Cell Biol 14:281-302, 1962.Crossref 15. David-Ferreira JF, Manaker RA: An electron microscope study of the development of a mouse hepatitis virus in tissue culture cells . J Cell Biol 24:57-58, 1965.Crossref 16. Vaughn JE, Pease DC: Electron microscopic studies of Wallerian degeneration in rat optic nerves: II. Astrocytes, oligodendrocytes and adventitial cells . J Comp Neurol 140:207-226, 1970.Crossref

Horowitz, Steven H.;Genkins, Gabriel;Kornfeld, Peter;Papatestas, Angelos E.

doi: 10.1001/archneur.1975.00490440034004pmid: 1122182

Abstract In 7 of 14 patients with clinically restricted ocular myasthenia gravis, the regional curare test showed latent peripheral involvement. The test consisted of the intravenous administration of 0.2 mg d-tubocurarine into an ischemic arm followed by repetitive supramaximal percutaneous electrical stimulation of the median or ulnar nerves. This produced a decrease in the amplitude of the initial evoked potential and a decrement of greater than 10% in the amplitude of the succeeding three to five potentials at rates of 3, 5, or 15 stimuli/sec. Three patients underwent transcervical thymectomy with subsequent improvement in both electrical and clinical findings. Evaluation of all patients with ocular myasthenia gravis should include regional curare testing of clinically uninvolved peripheral nerves. Thymectomy should be considered for patients with abnormal results. References 1. Bennett AE, Cash PT: Curare as a diagnostic test for myasthenia gravis: Curarization as an etiologic clue in the disease . Trans Am Neurol Assoc 68:102-106, 1942. 2. Bennett AE, Cash PT: Myasthenia gravis: Curare sensitivity: A new diagnostic test and approach to causation . Arch Neurol Psychiatr 49:537-547, 1943.Crossref 3. Pelikan EW, Tether JE, Unna KR: Sensitivity of myasthenia gravis patients to tubocurarine and decamethonium . Neurology 3:284-296, 1953.Crossref 4. Rowland LP, Aranow H, Hoefer PFA: Observation on the curare test in the differential diagnosis of myasthenia gravis , in Viets HR (ed): Myasthenia Gravis . Springfield, Ill, Charles C Thomas, 1961, pp 411-434. 5. Foldes FF, Klonymus DH, Maisel W, et al: A new curare test for the diagnosis of myasthenia gravis . JAMA 203:133-137, 1968.Crossref 6. Foldes FF: Regional intravenous neuromuscular block: A new diagnostic and experimental tool , in Progress in Anaesthesiology . Amsterdam, Excerpta Medica, 1970, pp 425-430. 7. Grob D: Course and management of myasthenia gravis . JAMA 153:529-532, 1953.Crossref 8. Osserman KE: Myasthenia Gravis . New York, Grune & Stratton, 1958, pp 76-80. 9. Viets HR, Schwab RR: Thymectomy for Myasthenia Gravis . Springfield, Ill, Charles C Thomas, 1960, p 23. 10. Osserman KE, Genkins G: Studies in myasthenia gravis: Review of a twenty year experience in over 1,200 patients . Mt Sinai J Med NY 38:497-537, 1971. 11. Perlo VP, et al: The role of thymectomy in the treatment of myasthenia gravis . Ann NY Acad Sci 183:308-315, 1971.Crossref 12. Simpson JA: An evaluation of thymectomy in myasthenia gravis . Brain 81:112-144, 1958.Crossref 13. Slomic A, Rosenfalck A, Buchthal F: Electrical and mechanical response of normal and myasthenic muscle . Brain Res 10:1-78, 1968.Crossref 14. Desmedt JE: The neuromuscular disorder in myasthenia gravis: I. Electrical and mechanical response to nerve stimulation in hand muscles , in Desmedt JE (ed): New Developments in Electromyography and Clinical Neurophysiology . Basel, Switzerland, Karger, vol 1, 1973, pp 241-304. 15. Ozdemir C, Young RR: Electrical testing in myasthenia gravis . Ann NY Acad Sci 183:287-302, 1971.Crossref 16. Desmedt JE: The neuromuscular disorder in myasthenia gravis: II. Presynaptic cholinergic metabolism, myasthenic-like syndromes and a hypothesis , in Desmedt JE (ed): New Developments in Electromyography and Clinical Neurophysiology . Basel, Switzerland, Karger, vol 1, 1973, pp 305-342. 17. Grob D, Johns RJ, Harvey AM: Studies in neuromuscular function: VI. Effects of anticholinesterase compounds, d-tubocurarine, and decamethonium in patients with myasthenia gravis . Bull Johns Hopkins Hosp 99:219-238, 1956.

Takahashi, Keiichi;Oimomi, Munetada;Shinko, Takeshi;Shutta, Keiichi;Matsuo, Bonpei;Takai, Tsuneo;Imura, Hiroo

doi: 10.1001/archneur.1975.00490440039005pmid: 1122183

Abstract Serum creatine phosphokinase activity increased significantly four or six hours after the administration of prednisolone in patients with muscular dystrophy of various types, whereas it did not increase in other muscular disorders. The extent of the response correlated inversely with the grade of disability. The prednisolone test may help to differentiate muscular dystrophy from polymyositis. References 1. Pearson CM: Polymyositis and related disorders , in Walton JN (ed): Disorders of Voluntary Muscle . London, J & A Churchill Ltd, 1969, pp 501-539. 2. Oimomi M, Takahashi K, Shinko T: Response of creatine phosphokinase after the administration of prednisolone in patients with progressive muscular dystrophy . Igakuno Ayumi 71:311-312, 1969. 3. Swinyard CA, Deaver GG: Gradients of functional ability of importance in rehabilitation of patients with progressive muscular and neuromuscular diseases . Arch Phys Med 38:574-579, 1957. 4. Okinaka S, Kumagai H, Ebashi S, et al: Serum creatine phosphokinase . Arch Neurol 4:520-525, 1961.Crossref 5. Nuttall FQ, Wedin DS: A simple rapid colorimetric method for determination of creatine kinase activity . J Lab Clin Med 68:324-332, 1966. 6. Craig F, Ross G: Serum CPK in thyroid disease . Metabolism 12:57-59, 1963. 7. Kakuta E: A study of creatine phosphokinase (CPK) activity in serum and cerebrospinal fluid of the patients with cerebrovascular attack . Clin Neurol 13:102-110, 1973. 8. Takahashi K, Tsuji S, Kato F, et al: Studies of muscular dystrophy (seventh report): Serum creatine phosphokinase and change of creatine metabolism, abstracted . Clin Neurol 8:460, 1968.

Kurihara, Teruyuki;Brooks, John E.

doi: 10.1001/archneur.1975.00490440042006pmid: 164844

Abstract In the rat hemidiaphragm, in vitro dantrolene sodium irreversibly reduced twitch tension up to 20% of the original value in 20 minutes of exposure. The phenomenon was dose related. Dantrolene sodium did not affect (1) resting membrane potential (RMP); (2) miniature endplate potential frequency or amplitude; or (3) endplate potential amplitude decrement of repetitively stimulated partially curarized muscle. A combination of hyperosmolar solutions of glycerol and dantrolene sodium abolished contraction of mammalian muscle with no demonstrable effect on neuromuscular transmission or muscle fiber RMP. With this technique, prolonged recording from repetitively activated muscle fibers is possible. References 1. Eisenberg RS, Gage PW: Frog skeletal muscle fibers: Changes in electrical properties after disruption of transverse tubular system . Science 158:1700-1701, 1967.Crossref 2. Gage PW, Eisenberg RS: Action potentials without contraction in frog skeletal muscle fibers with disrupted transverse tubules . Science 158:1702-1703, 1967.Crossref 3. Eisenberg B, Eisenberg RS: Transverse tubular system in glycerol-treated skeletal muscle . Science 160:1243-1244, 1968.Crossref 4. Krolenko SA: Changes in the T-system of muscle fibers under the influence of influx and efflux of glycerol . Nature 221:966-968, 1969.Crossref 5. Howell JN: A lesion of the transverse tubules of skeletal muscle . J Physiol 201:515-533, 1969. 6. Eisenberg RS, Howell JN, Vaughan PC: The maintenance of resting potentials in glycerol-treated muscle fibers . J Physiol 215:95-102, 1971. 7. Byrant SH: Cable properties of myotonic muscle fibers after tubular disruption . Fed Proc 29:A456, 1970. 8. Chyatle SB, Birdsong JH: The use of dantrolene sodium in disorders of the central nervous system . South Med J 64:830-834, 1971.Crossref 9. Chipman MK: Efficacy of dantrolene sodium in treatment of spasticity . Neurology 22:401, 1972. 10. Heald DE, Matsumoto Y: Inhibition of contraction of frog skeletal muscle by dantrolene sodium . Fed Proc 30:A378, 1971. 11. Forychta E, Esplin DW, Capek R, et al: The actions of dantrolene on extrafusal and intrafusal striated muscle . Fed Proc 30:A669, 1971. 12. Ellis KO, Carpenter JF: The effects of dantrolene sodium (F-440) on skeletal muscle . Fed Proc 30:A670, 1971. 13. Honkomp LJ, Halliday RP, Wessels FL: Dantrolene, 1-{(5-(P-nitrophenyl) furfurylidine) amino } hydantoin: A unique skeletal muscle relaxant . Pharmacology 12:301, 1971. 14. Heald DE, Matsumoto Y: The effect of dantrolene sodium on striated muscle . Pharmacology 13:264, 1971. 15. Krnjevic K, Miledi R: Adrenaline and failure of neuromuscular transmission . Nature 180:814-815, 1957.Crossref 16. Otsuka M, Endo M: Presynaptic nature of neuromuscular depression . Jap J Physiol 12:573-584, 1962.Crossref 17. Kuba K, Tomita T: Effect of prostigmine on the time course of the endplate potential in the rat diaphragm . J Physiol 213:533-544, 1971. 18. Hubbard JI, Jones SF, Landau EM: An examination of the effects of osmotic pressure changes upon transmitter release from mammalian motor nerve terminals . J Physiol 197:639-657, 1968.

Robinson, Norman;Duncan, Phillip;Gehrt, Mary;Sances, Anthony;Evans, Silas

doi: 10.1001/archneur.1975.00490440048007pmid: 164845

Abstract Electrodes were implanted into the rat hippocampus in both hemispheres for increasing periods of up to 60 days, and the effects of trauma and electrical stimulation on enzymes controlling cell metabolism in the region of implantation were examined and assessed in relation to studies on humans. In the unstimulated hippocampus as a control, enzyme changes were mainly confined to a narrow area of tissue damage surrounding the electrode. The enzyme changes in response to trauma varied widely; some enzymes controlling tissue respiration showed early and rapid changes, increasing in hyperactive, swollen glial cells and vascular endothelium and decreasing in nerve cells and neuropile. Acid phosphatase activity also increased rapidly in glial cells; other phosphate-releasing enzymes increased more gradually with time. A turning point in these changes was apparent between 25 and 40 days, followed by a reversion to more normal levels at 60 days. Electrical stimulation of the hippocampus in the contralateral hemisphere produced no detectable enzyme changes from those of the unstimulated hippocampus. References 1. Konig JFR, Klippel RA: The Rat Brain: A Stereotaxic Atlas . New York, Krieger, 1970, pp 30-51. 2. Robinson N: Histochemistry of human cervical posterior root ganglion cells and a comparison with anterior horn cells . J Anat 104:55-64, 1969. 3. Friede RL: Topographic Brain Chemistry . New York, Academic Press Inc, 1966, p 55. 4. Brodal A: Neurological Anatomy in Relation to Clinical Medicine, ed 2 . New York, Oxford University Press Inc, 1969, p 531. 5. Douglas RJ: The hippocampus and behaviour . Psychol Bull 67:416-442, 1967.Crossref 6. Barbizet J: Defect of memorising of hippocampal-mammillary body origin: A review . J Neurol Neurosurg Psychiatry 26:127-135, 1963.Crossref 7. Isaacson RL: Hippocampal destruction in man and other animals . Neurophysiologia 10:47-64, 1972. 8. De Jong RN: The hippocampus and its role in memory: Clinical manifestation and theoretical considerations . J Neurol Sci 19:73-83, 1973.Crossref 9. Walsh EG: Physiology of the Nervous System . London, Longmans Ltd, 1964, p 251. 10. Fleischhauer K: Zur chemoarchitektonik der Ammonsformation . Nervenarzt 30:305-309, 1959. 11. Rubinstein LJ, Klatzo I, Miquel J: Histochemical observations on oxidative enzyme activity of glial cells in a local brain injury . J Neuropathol Exp Neurol 21:116-136, 1962.Crossref 12. Friede RL: The enzymatic response to astrocytes to various ions in vitro . J Cell Biol 20:5-15, 1964.Crossref 13. Friede RL: Enzyme histochemical studies of multiple sclerosis . Arch Neurol 5:433-443, 1961.Crossref 14. Samorajski T, Zeman W, Ordy JM: Histochemistry of particle microbeam lesions in the brain of the mouse . J Neuropathol Exp Neurol 23:264-280, 1964.Crossref 15. Baron KD, Tuncbay TO: Phosphatase in cuneate nuclei after brachial plexotomy . Arch Neurol 7:203-210, 1962.Crossref 16. Ibrahim MZM, Adams CWM: The relationship between enzyme activity and neuroglia in plaques in multiple sclerosis . J Neurol Neurosurg Psychiatry 26:101-110, 1963.Crossref 17. Schwarzacher HG: Der cholinesterasegehalt motorischer Nervenzellen wahrend der axonalen Reaktion . Acta Anat 32:51-65, 1958.Crossref

Kornfeld, Mario;Snyder, Russell D.;MacGee, Joseph;Appenzeller, Otto

doi: 10.1001/archneur.1975.00490440053008pmid: 1122172

Abstract Clinical, light microscopical, ultrastructural, and biochemical studies were done on nerve and muscle biopsy specimens from five patients with the oculo-cerebral-renal syndrome of Lowe. Four patients were American Indians, a racial group in whom this disease has not previously been recognized. The hypotonia, areflexia, and diffuse atrophy of muscles are associated with slowed motor nerve conduction velocities, and the morphologic changes in sensory nerves are attributed to a "dying-back" phenomenon probably resulting from an unknown metabolic derangement. References 1. Lowe CU, Terry M, MacLachlan EA: Organic-aciduria, decreased renal ammonia production, hydrophthalmos, and mental retardation: A clinical entity . Am J Dis Child 83:164-184, 1952. 2. Abbassi V, Lowe CU, Calcagno PL: Oculocerebro-renal syndrome . Am J Dis Child 115:145-168, 1968.Crossref 3. MacGee J: Characterization of mammalian tissues and microorganisms by gas-liquid chromatography . J Gas Chromat 6:48-52, 1968.Crossref 4. Terslev E: Two cases of aminoaciduria, ocular changes and retarded mental and somatic development (Lowe's syndrome) . Acta Paediatr Scand 49:635-644, 1960.Crossref 5. Holmes GE, Tucker V: Oculo-cerebro-renal syndrome: A four generation family study and case reports of two living children . Clin Pediatr 11:119-124, 1972.Crossref 6. Garzuly F, Jellinger K, Szabo L, et al: Morbid changes in Lowe's oculo-cerebro-renal syndrome . Neuropadiatrie 4:304-313, 1973.Crossref 7. Engel WK: Muscle biopsy . Clin Orthop 39:80-105, 1965. 8. Hooft C, Valcke R, Herpol J, et al: Neurologie et neuropathologie du syndrome de Lowe . J Neurol Sci 3:353-373, 1966.Crossref 9. Chaptal J, Jean R, Grastes De Paulet A, et al: Étude clinique et biologique d'un enfant atteint d'un syndrome de Lowe . Arch Franc Pediatrie 16:849-850, 1959. 10. Brenton A, Gaudier B, Ponté C, et al: Syndrome de Lowe: Étude d'un observation . Pediatrie 14:908-915, 1959. 11. Richards W, Donnell GN, Wilson WA, et al: The oculo-cerebro-renal syndrome of Lowe . Am J Dis Child 100:707-709, 1960. 12. Richards W, Donnell GN, Wilson WA, et al: The oculo-cerebro-renal syndrome of Lowe . Am J Dis Child 109:185-203, 1965. 13. Chutorian A, Rowland LP: Lowe's syndrome . Neurology 16:115-122, 1966.Crossref 14. Rutsaert J, Segers-Cadranel A, Potvliege P: Lowe's syndrome: Pathological studies of four cases . Pathol Eur 7:249-262, 1972. 15. Appenzeller O, Snyder RD, Kornfeld M: Sural nerve biopsies in pediatric neurological disorders . Dev Med Child Neurol 12:42-48, 1970.Crossref 16. Prineas J: The pathogenesis of dying-back polyneuropathies: II. An ultrastructural study of experimental acrylamide intoxication in the cat . J Neuropathol Exp Neurol 28:598-621, 1969.Crossref 17. AppenzellerO, MacGee J: Gas-liquid chromatographic analysis of sural nerves in peripheral neuropathies . J Neurol Sci 7:593-603, 1968.Crossref 18. Appenzeller O, Kornfeld M, MacGee J: Neuropathy in chronic renal disease: A microscopic, ultrastructural, and biochemical study of sural nerve biopsies . Arch Neurol 24:449-461, 1971.Crossref

Perry, Thomas L.;Bratty, Paul J. A.;Hansen, Shirley;Kennedy, Janet;Urquhart, Nadine;Dolman, Clarisse L.

doi: 10.1001/archneur.1975.00490440058009pmid: 1122173

Abstract An unusual neuropsychiatric disorder inherited in autosomal dominant fashion occurred in three successive generations of a family. Symptoms commenced late in the fifth decade in six affected patients and led to death in four to six years. The earliest and most prominent symptom was mental depression not responsive to antidepressant drugs or electroconvulsive therapy. This was accompanied by exhaustion, sleep disturbances, and marked weight loss. Later in the disease, symptoms of parkinsonism appeared, and respiratory failure occurred terminally. The most recently affected family member was investigated biochemically late in his illness. Concentrations of taurine were greatly diminished in plasma and cerebrospinal fluid, and at autopsy, all regions of brain examined had a markedly reduced taurine content. Since taurine is a putative inhibitory synaptic transmitter, deficiency of brain taurine may possibly have caused the psychiatric and neurological manifestations of this disorder. References 1. Bell J, Clark AJ: A pedigree of paralysis agitans . Ann Eugenics 1:455-462, 1926.Crossref 2. Kehrer F: Der Ursachenkreis des Parkinsonismus (Erblichkeit, Trauma, Syphilis) . Arch Psychiatr Nervenkr 91:187-268, 1930.Crossref 3. Allan W: Inheritance of the shaking palsy . Arch Intern Med 60:424-436, 1937.Crossref 4. Hove H: Paralysis agitans hereditaria . Ugeskr Laeger 116:614-616, 1954. 5. Branger F: Une forme familiale de paralysie agitante dans une souche des Grisons . J Genet Hum 5:261-270, 1956. 6. Pescetto G: Nuovi aspetti dell'ereditarietâ nel morbo di Parkinson . Riv Neurobiol 3:611-632, 1957. 7. Spellman GG: Report of familial cases of parkinsonism: Evidence of a dominant trait in a patient's family . JAMA 179:372-374, 1962.Crossref 8. Dorzab J, Baker M, Cadoret RJ, et al: Depressive disease: Familial psychiatric illness . Am J Psychiatr 127:1128-1133, 1971. 9. Warburton JW: Depressive symptoms in Parkinson patients referred for thalamotomy . J Neurol Neurosurg Psychiatr 30:368-370, 1967.Crossref 10. Perry TL, Stedman D, Hansen S: A versatile lithium buffer elution system for single column automatic amino acid chromatography . J Chromatogr 38:460-466, 1968.Crossref 11. Perry TL, Hansen S: Technical pitfalls leading to errors in the quantitation of plasma amino acids . Clin Chim Acta 25:53-58, 1969.Crossref 12. Perry TL, Berry K, Hansen S, et al: Regional distribution of amino acids in human brain obtained at autopsy . J Neurochem 18:513-519, 1971.Crossref 13. Perry TL, Hansen S, Berry K, et al: Free amino acids and related compounds in biopsies of human brain . J Neurochem 18:521-528, 1971.Crossref 14. Perry TL, Hansen S, Kloster M: Huntington's chorea: Deficiency of γ-aminobutyric acid in brain . N Engl J Med 288:337-342, 1973.Crossref 15. Armstrong MD: Decreased taurine excretion in relation to childbirth, lactation and progestin-estrogen therapy . Clin Chim Acta 46:253-256, 1973.Crossref 16. Jacobsen JG, Smith LH Jr: Biochemistry and physiology of taurine and taurine derivatives . Physiol Rev 48:424-511, 1968. 17. Peck EJ Jr, Awapara J: Formation of taurine and isethionic acid in rat brain . Biochim Biophys Acta 141:499-506, 1967.Crossref 18. Curtis DR, Watkins JC: The pharmacology of amino acids related to gamma-aminobutyric acid . Pharmacol Rev 17:347-391, 1965. 19. Jasper H, Koyama I: Rate of release of amino acids from the cerebral cortex in the cat as affected by brain-stem and thalamic stimulation . Canad J Physiol Pharmacol 47:889-905, 1969.Crossref 20. Kaczmarek LK, Davison AN: Uptake and release of taurine from rat brain slices . J Neurochem 19:2355-2362, 1972.Crossref 21. Lähdesmäki P, Oja SS: Neurotransmitter or modulator: Does taurine qualify? Scand J Clin Lab Invest 29( (suppl 122) ):71, 1972.Crossref 22. Pasantes-Morales H, Urban PF, Klethi J, et al: Light-stimulated release of [35S]taurine from chicken retina . Brain Res 51:375-378, 1973.Crossref 23. Urquhart N, Perry TL, Hansen S, et al: Passage of taurine into adult mammalian brain . J Neurochem 22:871-872, 1974.Crossref 24. Bird ED, Mackay AVP, Rayner CN, et al: Reduced glutamic-acid-decarboxylase activity of post-mortem brain in Huntington's chorea . Lancet 1:1090-1092, 1973.Crossref

Ambani, Lalit M.;Van Woert, Melvin H.;Murphy, Sean

doi: 10.1001/archneur.1975.00490440064010pmid: 1122174

Abstract Peroxidase and catalase activities were determined in various regions of parkinsonian brains and control brains from patients with nonneurological diseases. The highest peroxidase activity was localized in the substantia nigra of the normal brain. In Parkinson disease, the peroxidase activity was decreased in the substantia nigra, caudate, and putamen. Catalase activity was also reduced in the substantia nigra and putamen of the parkinsonian brain. These enzyme changes may be causally related to the degeneration and depigmentation of the substantia nigra neurons in Parkinson disease. References 1. Greenfield JG: Paralysis agitans (Parkinson's disease) , in Blackwood H, McMenemy WH, Meyer A, et al (eds): Greenfield's Neuropathology , ed 2. London, Edward Arnold Ltd, 1963, p 582. 2. Stein L, Wise CD: Possible etiology of schizophrenia: Progressive damage to the noradrenergic reward system by 6-hydroxydopamine . Science 171:1032-1036, 1971.Crossref 3. Sandler M: The role of minor pathways of dopa metabolism , in Barbeau A, McDowell FH (eds): L-Dopa and Parkinsonism . Philadelphia, FA Davis Co, 1970, p 72. 4. Uretsky NJ, Iversen LI: Effects of 6-hydro-xydopamine on noradrenaline-containing neurons in the rat brain . Nature 221:557-599, 1969.Crossref 5. Laverty R, Taylor KM: Effects of intraventricular 2,4,5-trihydroxyphenylethylamine (6-hydroxydopamine) on rat behavior and brain catecholamine metabolism . Br J Pharmacol 40:836-840, 1970.Crossref 6. Breese GR, Traylor TD: Effect of 6-hydro-xydopamine on brain norepinephrine and dopamine: Evidence for selective degeneration of catecholamine neurons . J Pharmacol Exp Ther 174:413-420, 1970. 7. Senoh S, Creveling CR, Udenfriend S, et al: Chemical enzymatic and metabolic studies on the mechanism of oxidation of dopamine . J Am Chem Soc 81:6236-6240, 1959.Crossref 8. Van Woert MH, Ambani LM, Bowers MB Jr: Levodopa and cholinergic hypersensitivity in Parkinson's disease . Neurology 22:86-93, 1972.Crossref 9. Heikkila RE, Cohen G: Inhibition of biogenic amine uptake by hydrogen peroxide: A mechanism for toxic effects of 6-hydroxydopamine . Science 172:1257-1258, 1971.Crossref 10. Heikkila RE, Cohen G: In vivo generation of hydrogen peroxide from 6-hydroxydopamine . Experientia 28:1197, 1972.Crossref 11. Coyle JT: Tyrosine hydroxylase in rat brain: cofactor requirements, regional and subcellular distribution . Biochem Pharmacol 21:1935-1944, 1972.Crossref 12. Coyle JT, Axelrod J: Dopamine-β-hydroxylase in the rat brain: Development characteristics . J Neurochem 19:449-459, 1972.Crossref 13. McGeer PL, McGeer E: Cholinergic enzyme systems in Parkinson's disease . Arch Neurol 25:265-268, 1971.Crossref 14. Ehringer H, Hornykiewicz O: Das verhalten des dopamin: metaboliten homoranillinsaure im gehirn von normalen und Parkinson-kranken menschen . Arch Exp Pathol Pharmakol 247:305-306, 1961. 15. Shiman R, Akino M, Kaufman S: Solubilization and partial purification of tyrosine hydroxylase from bovine adrenal medulla . J Biol Chem 246:1330-1340, 1971. 16. Mason H, Onoprienko I, Buhler D: Hydroxylation: The activation of oxygen by peroxidase . Biochim Biophys Acta 24:225-226, 1957.Crossref 17. Patel R, Okun M, Edelstein D, et al: Bio-chemical studies of peroxidase-mediated oxidation of tyrosine to melanin . Biochem J 124:439-441, 1971. 18. Bayse GS, Morrison M: The role of peroxidase in catalyzing oxidation of polyphenols . Biochim Biophys Acta 244:77-84, 1971.Crossref 19. Lundquist I, Josefsson JO: Sensitive method for determination of peroxidase activity in tissues by means of coupled oxidation reaction . Anal Biochem 41:567-577, 1971.Crossref 20. Goldstein DB: A method for assay of catalase with the oxygen cathode . Anal Biochem 24:431-437, 1968.Crossref 21. Keilin D, Hartree EF: Purification of horseradish peroxidase and comparison of its properties with those of catalase and methaemoglobin . Biochem J 49:88-104, 1951. 22. Abrams R, Altschul AM, Hagness TB: Cytochrome c peroxidase II: The peroxidase-hydrogen peroxide complex . J Biol Chem 142:303-316, 1942. 23. Polis BD, Shmukler HW: Crystalline lactoperoxidase: I. Isolation by displacement chromatography . J Biol Chem 201:475-500, 1953. 24. Agner K: Verdoperoxidase: A ferment isolated from leucocytes . Acta Physiol Scand 2( (suppl 8) ):1-62, 1941.Crossref 25. Knox WE: The action of peroxidases with enzymically generated peroxide in the presence of catalase . Biochim Biophys Acta 14:117-126, 1954.Crossref 26. Hosoya T, Morrison M: The isolation and purification of thyroid peroxidase . J Biol Chem 242:2828-2836, 1967. 27. Okun MR, Donnellan B, Lever WF, et al: Peroxidase-dependent oxidation of tyrosine or dopa to melanin in neurons . Histochemie 25:289-296, 1971.Crossref 28. Okun MR, Edelstein LM, Nur O, et al: Oxidation of tyrosine and dopa to melanin by mammalian peroxidase: The possible role of peroxidase in melanin synthesis and catecholamine synthesis in vivo , in Riley V (ed): Pigmentation: Its Genesis and Biologic Control . New York, Appleton-Century-Crofts, 1972, pp 571-592. 29. Ambani LM, Van Woert MH: Catalase and peroxidase in human brain . Trans Am Neurol Assoc 98:7-10, 1973. 30. Van Woert MH, Prasad KN, Borg DC: Spectroscopic studies of substantia nigra pigment in human subjects . J Neurochem 14:707-716, 1967.Crossref 31. Foley JM, Baxter D: On the nature of pigment granules in the cells of the locus caeruleus and substantia nigra . J Neuropathol Exp Neurol 17:586-598, 1958.Crossref 32. Cote LJ, Fahn S: Some aspects of the biochemistry of the substantia nigra of the rhesus monkey , in Barbeau A, Brunette J (eds): Progress in Neuro-Genetics . Amsterdam, Excerpta Medica Foundation, 1969, pp 311-317. 33. Van Woert MH, Cotzias GC: Possible roles of melanin in some extrapyramidal functions , in Spiegel EA (ed): Parkinson's disease . New York, Grune & Stratton Inc, 1965, pp 95-97. 34. Bernheimer H, Birkmayer W, Hornykiewicz O: Verhalten der monoaminoxydase im gehirn des menschen nach therapie mit monoaminoxdase-hemmern . Wien Klin Wochenschr 74:558-559, 1962. 35. Regan MAG, Regan DH, Demopoulos HD: The vital respiratory role of tyrosinase in pigmented S-91 melanomas , in Riley V (ed): Pigmentation: Its Genesis and Biologic Control . New York, Appleton-Century-Crofts, 1972, pp 543-549. 36. Cohen G, Hochstein P: Enzymatic mechanisms of drug sensitivity in brain . Dis Nerv Syst 24:44-48, 1963. 37. Cohen G, Hochstein P: Glucose-6-phosphate dehydrogenase and detoxification of hydrogen peroxide in human erythrocytes . Science 134:1756-1757, 1961.Crossref 38. Sano I, Gamo T, Kakimoto Y, et al: Distribution of catechol compounds in human brain . Biochim Biophys Acta 32:586-587, 1959.Crossref 39. Barden H: The histochemical relationship of neuromelanin and lipofuscin . J Neuropathol Exp Neurol 28:419-441, 1969.Crossref 40. Allan W: Inheritance of shaking palsy . Arch Intern Med 60:424-436, 1937.Crossref 41. Mjones H: Paralysis agitans: A clinical and genetic study . Acta Psychiatr Scand 54:1-194, 1949. 42. Martin WE, Young WI, Anderson VE: Parkinson's disease: A genetic study . Brain 96:495-506, 1973.Crossref 43. Kondo K, Kurland LT, Schull WJ: Parkinson's disease: Genetic analysis and evidence of a multifactorial etiology . Mayo Clin Proc 48:465-475, 1973. 44. Myrianthopoulos NC, Waldrop FN, Vincent BL: A repeat study of hereditary predisposition in drug-induced parkinsonism , in Barbeau A, Brunette JR (eds): Progress in Neurogenetics . Amsterdam, Excerpta Medica Foundation, 1969, pp 486-491. 45. Kurland LT: Epidemiology: Incidence, geographic distribution and genetic considerations , in Fields WS (ed): Pathogenesis and Treatment of Parkinsonism . Springfield, Ill, CC Thomas, pp 5-49. 46. Kessler II: Epidemiologic studies of Parkinson's disease: III. A community-based survey . Am J Epidemiol 96:242-254, 1972.