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Interstitial Hypertrophic Neuropathy: Biochemical Study of the Peripheral Nervous System

Interstitial Hypertrophic Neuropathy: Biochemical Study of the Peripheral Nervous System Abstract Peripheral nerve tissue of a patient with an electron microscopically verified interstitial hypertrophic neuropathy contained excessive amounts of water and protein but less lipid than comparable control tissue. In the tissue hydrolyzate, hydroxyproline was increased fourfold, hexosamines, twofold. The reduction of lipid was associated with higher than normal levels of cholesteryl esters. The fatty acid profile of the triglycerides, cholesteryl esters, and phospholipids showed abnormally high concentrations of stearic acid and increased stearic acid-oleic acid ratios. The lipids of myelin isolated from the diseased peripheral nerve had most of the characteristics of human peripheral myelin (lower cholesterol and galactolipid than central nervous system myelin) but differed by a higher concentration of choline phosphoglycerides. References 1. Wolf A, Rubinowitz AH, Burchell SC: Interstitial hypertrophic neuritis of Dejerine and Sottas . Bull Neuro Inst NY 2:373-428, 1932. 2. Austin JH: Observations on the syndrome of hypertrophic neuritis: The hypertrophic interstitial radiculo-neuropathies . Medicine 35:187-237, 1956.Crossref 3. Marie P, Bertrand I: Contribution a l'anatomie pathologique de la névrite hypertrophique familiale . Ann Med 5:209-258, 1918. 4. Souques A, Bertrand I: Contribution a l'étude anatomopathologique de la névrite hypertrophique familiale . Ann Med 9:305-329, 1921. 5. Krücke W: Die mucoide Degeneration der peripheren Nerven . Arch Path Anat Physiol 304:442-463, 1939.Crossref 6. Fardeau M, Engel WK: Ultrastructural study of a peripheral nerve biopsy in Refsum's disease . Neuropath Exp Neurol 28:278-294, 1969.Crossref 7. Reese H, Bareta J: Heredopathia atactica polyneuritiformis . J Neuropath Exp Neurol 9:385-395, 1950.Crossref 8. Gruner JE: La biopsie nerveuse en microscopie électronique . CR Soc Biol 154:1632-1634, 1960. 9. Garcin R, Lapresle J, Fardeau M, et al: Etude au microscope électronique du nerf péripherique prélevé par biosie dans quatre cas de névrite hypertrophique de Dejerine-Sottas . Rev Neurol 115:917-932, 1966. 10. Weller RO: An electron microscopic study of hypertrophic neuropathy of Dejerine and Sottas . J Neurol Neurosurg Psychiat 30:111-125, 1967.Crossref 11. Thomas PK, Lascelles RG: Hypertrophic neuropathy . Quart J Med 36:223-238 ( (No. 142) ), 1967. 12. Webster HD, Schröder JM, Asbury AK, et al: The role of Schwann cells in the formation of "onion bulbs" found in chronic neuropathies . J Neuropath Exp Neurol 26:276-299, 1967.Crossref 13. Dyck PJ, Gomez MR: Segmental demyelination in Dejerine-Sottas disease: Light, phase-contrast, and electron microscopic studies . Mayo Clin Proc 43:280-296, 1968. 14. Dyck PJ: Experimental hypertrophic neuropathy . Arch Neurol 21:73-95, 1969.Crossref 15. Weller RO, Das Gupta TK: Experimental hypertrophic neuropathy: An electron microscopic study . J Neurol Neurosurg Psychiat 31:34-42, 1968.Crossref 16. Lampert PW, Schochet SS: Demyelination and remyelination in lead neuropathy . J Neuropath Exp Neurol 27:527-545, 1968. 17. Gilroy J, Meyer JS, Bauer RB, et al: Clinical biochemical and neurophysiological studies of chronic interstitial hypertrophic polyneuropathy . Amer J Med 40:368-383, 1966.Crossref 18. Klenk E, Kahlke W: Verdacht auf Heredopathia atactica polyneuritiformis (Refsum-Syndrom) . Z Physiol Chem 333:133-139, 1963.Crossref 19. Kahlke W: Refsum-Syndrom-Lipoidchemische Untersuchungen bei 9 Fällen . Klin Wschr 41:1011-1016, 1964.Crossref 20. Hansen RP: 3, 7, 11, 15-tetramethylhexadecanoic acid: Its occurrence in the tissues of humans afficted with Refsum's syndrome . Biochim Biophys Acta 106:304-310, 1965.Crossref 21. Kahlke W, Wagener W: Conversion of H3-phytol to phytanic acid and its incorporation into plasma lipid fractions in heredopathia atactica polyneuritiformis . Metabolism 15:687-693, 1966.Crossref 22. Rake M, Saunders M: Refsum's disease: A disorder of lipid metabolism . J Neurol Neurosurg Psychiat 29:417-422, 1966.Crossref 23. Alexander WS: Phytanic acid in Refsum's syndrome . J Neurol Neurosurg Psychiat 29:412-416, 1966.Crossref 24. Karlsson KA, Norrby A, Samuelson B: Use of thin-layer chromatography for the preliminary diagnosis of Refsum's disease (heredopathia atactica polyneuritiformis) . Biochim Biophys Acta 144:162-164, 1967.Crossref 25. MacBrinn M, O'Brien JS: Lipid composition of the nervous system in Refsum's disease . J Lipid Res 9:552-561, 1968. 26. Skrbic TR, Cumings JN: Phytanic acid in tissue lipids in Refsum's disease . Clin Chim Acta 23:17-21, 1970.Crossref 27. Dyck PJ, Ellefson RD, Lais AC, et al: Histologic and lipid studies of sural nerves in inherited hypertrophic neuropathy: Preliminary report of a lipid abnormality in nerve and liver in Dejerine-Sottas disease . Mayo Clin Proc 45:286-327, 1970. 28. Drachman DA: Ophthalmoplegia plus . Arch Neurol 18:654-674, 1968.Crossref 29. Karlsson U, Schultz RL: Fixation of the central nervous system for electron microscopy by aldehyde perfusion: I. Preservation with aldehyde perfusates versus direct perfusion with osmium tetroxide with special reference to membranes and the extracellular space . J Ultrastruc Res 12:160-186, 1965.Crossref 30. Luft JH: Improvement in epoxy resin embedding methods . J Biophys Biochem Cytol 9:409-414, 1961.Crossref 31. Karnovsky MJ: Simple methods for "staining with lead" at high pH in electron microscopy . J Biophys Biochem Cytol 11:729-732, 1961.Crossref 32. Folch J, Lees M, Sloane Stanley GH: A simple method for the isolation and purification of total lipides from animal tissues . J Biol Chem 226:497-509, 1957. 33. Stern I, Shapiro B: A rapid and simple method for the determination of esterified fatty acids and for total fatty acids in blood . J Clin Path 6:158-160, 1953.Crossref 34. Searcy RL, Bergqvist LM: A new color reaction for the quantitation of serum cholesterol . Clin Chim Acta 5:192-199, 1960.Crossref 35. Bartlett GR: Phosphorus assay in column chromatography . J Biol Chem 234:466-468, 1959. 36. Hess HH, Lewin E: Microassay of biochemical structural components in nervous tissue: II. Method for cerebrosides, proteolipid proteins and residue proteins . J Neurochem 12:205-211, 1965.Crossref 37. Wittenberg JB, Korey SR, Swenson FH: The determination of higher fatty aldehydes in tissues . J Biol Chem 219:39-47, 1956. 38. O'Brien JS, Rouser G: Analysis of hydroxy fatty acids by gas-liquid chromatography . Anat Biochem 7:288-296, 1964.Crossref 39. Prockop DJ, Udenfried S: A specific method for the analysis of hydroxyproline in tissues and urine . Anal Biochem 1:228-239, 1960.Crossref 40. Elson LA, Morgan WTJ: A colorimetric method for the determination of glucosamine and chondrosamine . Biochem J 27:1824-1828, 1933. 41. Boas NF: Method for the determination of hexosamines in tissues . J Biol Chem 204:553-563, 1953. 42. Horrocks LA: Composition of myelin from peripheral and central nervous system of the squirrel monkey . J Lipid Res 8:569-576, 1967. 43. Pease DC: Histological Techniques for Electron Microscopy . New York, Academic Press, 1960, p 36. 44. Eastoe JE: Composition of collagen and allied proteins , in Ramachandran GN (ed): Treatise on Collagen . New York, Academic Press, 1967, vol 1, p 1. 45. O'Brien JS, Sampson EL, Stern MB: Lipid composition of myelin from the peripheral nervous system . J Neurochem 14:357-365, 1967.Crossref 46. Rossiter RJ: Chemical constituents of brain and nerve , in Elliott KAC, et al (eds): Neurochemistry . Charles C Thomas Publisher, Springfield, Ill, 1962, p 34. 47. Chandrasekaran EV, Bachhawat BK: Isolation and characterization of glycosaminoglycan in peripheral nerve and spinal cord of monkey . J Neurochem 16:1529-1532, 1969.Crossref 48. Rambourg A, Leblond CP: Electron microscope observations on the carbohydrate-rich cell coat present at the surface of cells in the rat . J Cell Biol 32:27-53, 1967.Crossref 49. Amaducci L: The distribution of proteolipids in the human nervous system . J Neurochem 9:153-160, 1962.Crossref 50. Abercrombie M, Johnson ML: Collagen content of rabbit sciatic nerve during Wallerian degeneration . J Neurol Neurosurg Psychiat 9:113-118, 1946.Crossref 51. Johnson AC, McNabb AR, Rossiter RJ: Chemical studies of peripheral nerve during Wallerian degeneration . Biochem J 45:500-508, 1949. 52. McCaman RE, Robins E: Quantitative biochemical studies of Wallerian degeneration in the peripheral and central nervous systems: I. Chemical constituents . J Neurochem 5:18-31, 1959.Crossref 53. Berry JF, Cevallos WH, Wade RR Jr: Lipid class and fatty acid composition of intact peripheral nerve and during Wallerian degeneration . J Amer Oil Chem Soc 42:492-500, 1965.Crossref 54. Johnson AC, McNabb AR, Rossiter RJ: Lipids of peripheral nerve . Biochem J 43:578-580, 1948. 55. Lang AK, Mayer H: Über die tierische Fettsäuredehydrase und ihre Codehydrase . Z Physiol Chem 261:249-252, 1939.Crossref 56. Annau E, Eperjessy A, Felszeghy O: Biologische Dehydrierung der Lecithine und der Fettsäuren . Z Physiol Chem 277:58-65, 1942.Crossref 57. Marsh JB, James AT: The conversion of stearic to oleic acid by liver and yeast preparations . Biochim Biophys Acta 60:320-328, 1962.Crossref 58. Blomstrand R, Dahlbäck O: Gas-liquid chromatography of human lymph fatty acids after feeding C14-labelled fats . Acta Soc Med Upsal 114:177-184, 1959. 59. Eng LF, Noble EP: The maturation of rat brain myelin . Lipids 3:157-162, 1968.Crossref 60. Horrocks LA: Composition of mouse brain myelin during development . J Neurochem 15:483-488, 1968.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Interstitial Hypertrophic Neuropathy: Biochemical Study of the Peripheral Nervous System

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References (69)

Publisher
American Medical Association
Copyright
Copyright © 1971 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1971.00480340072008
Publisher site
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Abstract

Abstract Peripheral nerve tissue of a patient with an electron microscopically verified interstitial hypertrophic neuropathy contained excessive amounts of water and protein but less lipid than comparable control tissue. In the tissue hydrolyzate, hydroxyproline was increased fourfold, hexosamines, twofold. The reduction of lipid was associated with higher than normal levels of cholesteryl esters. The fatty acid profile of the triglycerides, cholesteryl esters, and phospholipids showed abnormally high concentrations of stearic acid and increased stearic acid-oleic acid ratios. The lipids of myelin isolated from the diseased peripheral nerve had most of the characteristics of human peripheral myelin (lower cholesterol and galactolipid than central nervous system myelin) but differed by a higher concentration of choline phosphoglycerides. References 1. Wolf A, Rubinowitz AH, Burchell SC: Interstitial hypertrophic neuritis of Dejerine and Sottas . Bull Neuro Inst NY 2:373-428, 1932. 2. Austin JH: Observations on the syndrome of hypertrophic neuritis: The hypertrophic interstitial radiculo-neuropathies . Medicine 35:187-237, 1956.Crossref 3. Marie P, Bertrand I: Contribution a l'anatomie pathologique de la névrite hypertrophique familiale . Ann Med 5:209-258, 1918. 4. Souques A, Bertrand I: Contribution a l'étude anatomopathologique de la névrite hypertrophique familiale . Ann Med 9:305-329, 1921. 5. Krücke W: Die mucoide Degeneration der peripheren Nerven . Arch Path Anat Physiol 304:442-463, 1939.Crossref 6. Fardeau M, Engel WK: Ultrastructural study of a peripheral nerve biopsy in Refsum's disease . Neuropath Exp Neurol 28:278-294, 1969.Crossref 7. Reese H, Bareta J: Heredopathia atactica polyneuritiformis . J Neuropath Exp Neurol 9:385-395, 1950.Crossref 8. Gruner JE: La biopsie nerveuse en microscopie électronique . CR Soc Biol 154:1632-1634, 1960. 9. Garcin R, Lapresle J, Fardeau M, et al: Etude au microscope électronique du nerf péripherique prélevé par biosie dans quatre cas de névrite hypertrophique de Dejerine-Sottas . Rev Neurol 115:917-932, 1966. 10. Weller RO: An electron microscopic study of hypertrophic neuropathy of Dejerine and Sottas . J Neurol Neurosurg Psychiat 30:111-125, 1967.Crossref 11. Thomas PK, Lascelles RG: Hypertrophic neuropathy . Quart J Med 36:223-238 ( (No. 142) ), 1967. 12. Webster HD, Schröder JM, Asbury AK, et al: The role of Schwann cells in the formation of "onion bulbs" found in chronic neuropathies . J Neuropath Exp Neurol 26:276-299, 1967.Crossref 13. Dyck PJ, Gomez MR: Segmental demyelination in Dejerine-Sottas disease: Light, phase-contrast, and electron microscopic studies . Mayo Clin Proc 43:280-296, 1968. 14. Dyck PJ: Experimental hypertrophic neuropathy . Arch Neurol 21:73-95, 1969.Crossref 15. Weller RO, Das Gupta TK: Experimental hypertrophic neuropathy: An electron microscopic study . J Neurol Neurosurg Psychiat 31:34-42, 1968.Crossref 16. Lampert PW, Schochet SS: Demyelination and remyelination in lead neuropathy . J Neuropath Exp Neurol 27:527-545, 1968. 17. Gilroy J, Meyer JS, Bauer RB, et al: Clinical biochemical and neurophysiological studies of chronic interstitial hypertrophic polyneuropathy . Amer J Med 40:368-383, 1966.Crossref 18. Klenk E, Kahlke W: Verdacht auf Heredopathia atactica polyneuritiformis (Refsum-Syndrom) . Z Physiol Chem 333:133-139, 1963.Crossref 19. Kahlke W: Refsum-Syndrom-Lipoidchemische Untersuchungen bei 9 Fällen . Klin Wschr 41:1011-1016, 1964.Crossref 20. Hansen RP: 3, 7, 11, 15-tetramethylhexadecanoic acid: Its occurrence in the tissues of humans afficted with Refsum's syndrome . Biochim Biophys Acta 106:304-310, 1965.Crossref 21. Kahlke W, Wagener W: Conversion of H3-phytol to phytanic acid and its incorporation into plasma lipid fractions in heredopathia atactica polyneuritiformis . Metabolism 15:687-693, 1966.Crossref 22. Rake M, Saunders M: Refsum's disease: A disorder of lipid metabolism . J Neurol Neurosurg Psychiat 29:417-422, 1966.Crossref 23. Alexander WS: Phytanic acid in Refsum's syndrome . J Neurol Neurosurg Psychiat 29:412-416, 1966.Crossref 24. Karlsson KA, Norrby A, Samuelson B: Use of thin-layer chromatography for the preliminary diagnosis of Refsum's disease (heredopathia atactica polyneuritiformis) . Biochim Biophys Acta 144:162-164, 1967.Crossref 25. MacBrinn M, O'Brien JS: Lipid composition of the nervous system in Refsum's disease . J Lipid Res 9:552-561, 1968. 26. Skrbic TR, Cumings JN: Phytanic acid in tissue lipids in Refsum's disease . Clin Chim Acta 23:17-21, 1970.Crossref 27. Dyck PJ, Ellefson RD, Lais AC, et al: Histologic and lipid studies of sural nerves in inherited hypertrophic neuropathy: Preliminary report of a lipid abnormality in nerve and liver in Dejerine-Sottas disease . Mayo Clin Proc 45:286-327, 1970. 28. Drachman DA: Ophthalmoplegia plus . Arch Neurol 18:654-674, 1968.Crossref 29. Karlsson U, Schultz RL: Fixation of the central nervous system for electron microscopy by aldehyde perfusion: I. Preservation with aldehyde perfusates versus direct perfusion with osmium tetroxide with special reference to membranes and the extracellular space . J Ultrastruc Res 12:160-186, 1965.Crossref 30. Luft JH: Improvement in epoxy resin embedding methods . J Biophys Biochem Cytol 9:409-414, 1961.Crossref 31. Karnovsky MJ: Simple methods for "staining with lead" at high pH in electron microscopy . J Biophys Biochem Cytol 11:729-732, 1961.Crossref 32. Folch J, Lees M, Sloane Stanley GH: A simple method for the isolation and purification of total lipides from animal tissues . J Biol Chem 226:497-509, 1957. 33. Stern I, Shapiro B: A rapid and simple method for the determination of esterified fatty acids and for total fatty acids in blood . J Clin Path 6:158-160, 1953.Crossref 34. Searcy RL, Bergqvist LM: A new color reaction for the quantitation of serum cholesterol . Clin Chim Acta 5:192-199, 1960.Crossref 35. Bartlett GR: Phosphorus assay in column chromatography . J Biol Chem 234:466-468, 1959. 36. Hess HH, Lewin E: Microassay of biochemical structural components in nervous tissue: II. Method for cerebrosides, proteolipid proteins and residue proteins . J Neurochem 12:205-211, 1965.Crossref 37. Wittenberg JB, Korey SR, Swenson FH: The determination of higher fatty aldehydes in tissues . J Biol Chem 219:39-47, 1956. 38. O'Brien JS, Rouser G: Analysis of hydroxy fatty acids by gas-liquid chromatography . Anat Biochem 7:288-296, 1964.Crossref 39. Prockop DJ, Udenfried S: A specific method for the analysis of hydroxyproline in tissues and urine . Anal Biochem 1:228-239, 1960.Crossref 40. Elson LA, Morgan WTJ: A colorimetric method for the determination of glucosamine and chondrosamine . Biochem J 27:1824-1828, 1933. 41. Boas NF: Method for the determination of hexosamines in tissues . J Biol Chem 204:553-563, 1953. 42. Horrocks LA: Composition of myelin from peripheral and central nervous system of the squirrel monkey . J Lipid Res 8:569-576, 1967. 43. Pease DC: Histological Techniques for Electron Microscopy . New York, Academic Press, 1960, p 36. 44. Eastoe JE: Composition of collagen and allied proteins , in Ramachandran GN (ed): Treatise on Collagen . New York, Academic Press, 1967, vol 1, p 1. 45. O'Brien JS, Sampson EL, Stern MB: Lipid composition of myelin from the peripheral nervous system . J Neurochem 14:357-365, 1967.Crossref 46. Rossiter RJ: Chemical constituents of brain and nerve , in Elliott KAC, et al (eds): Neurochemistry . Charles C Thomas Publisher, Springfield, Ill, 1962, p 34. 47. Chandrasekaran EV, Bachhawat BK: Isolation and characterization of glycosaminoglycan in peripheral nerve and spinal cord of monkey . J Neurochem 16:1529-1532, 1969.Crossref 48. Rambourg A, Leblond CP: Electron microscope observations on the carbohydrate-rich cell coat present at the surface of cells in the rat . J Cell Biol 32:27-53, 1967.Crossref 49. Amaducci L: The distribution of proteolipids in the human nervous system . J Neurochem 9:153-160, 1962.Crossref 50. Abercrombie M, Johnson ML: Collagen content of rabbit sciatic nerve during Wallerian degeneration . J Neurol Neurosurg Psychiat 9:113-118, 1946.Crossref 51. Johnson AC, McNabb AR, Rossiter RJ: Chemical studies of peripheral nerve during Wallerian degeneration . Biochem J 45:500-508, 1949. 52. McCaman RE, Robins E: Quantitative biochemical studies of Wallerian degeneration in the peripheral and central nervous systems: I. Chemical constituents . J Neurochem 5:18-31, 1959.Crossref 53. Berry JF, Cevallos WH, Wade RR Jr: Lipid class and fatty acid composition of intact peripheral nerve and during Wallerian degeneration . J Amer Oil Chem Soc 42:492-500, 1965.Crossref 54. Johnson AC, McNabb AR, Rossiter RJ: Lipids of peripheral nerve . Biochem J 43:578-580, 1948. 55. Lang AK, Mayer H: Über die tierische Fettsäuredehydrase und ihre Codehydrase . Z Physiol Chem 261:249-252, 1939.Crossref 56. Annau E, Eperjessy A, Felszeghy O: Biologische Dehydrierung der Lecithine und der Fettsäuren . Z Physiol Chem 277:58-65, 1942.Crossref 57. Marsh JB, James AT: The conversion of stearic to oleic acid by liver and yeast preparations . Biochim Biophys Acta 60:320-328, 1962.Crossref 58. Blomstrand R, Dahlbäck O: Gas-liquid chromatography of human lymph fatty acids after feeding C14-labelled fats . Acta Soc Med Upsal 114:177-184, 1959. 59. Eng LF, Noble EP: The maturation of rat brain myelin . Lipids 3:157-162, 1968.Crossref 60. Horrocks LA: Composition of mouse brain myelin during development . J Neurochem 15:483-488, 1968.Crossref

Journal

Archives of NeurologyAmerican Medical Association

Published: Apr 1, 1971

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