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Experimental Hypertrophic Neuropathy: Title and subTitle BreakPathogenesis of Onion-Bulb Formations Produced by Repeated Tourniquet Applications

Peter James Dyck, MD
[+] Author Affiliations

Submitted for publication Nov 18, 1968; accepted Feb 5, 1969.

Reprint requests to the Section of Neurology, Mayo Clinic and Mayo Foundation, Rochester, Minn 55901.


Arch Neurol. 1969;21(1):73-95. doi:10.1001/archneur.1969.00480130087010
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IN A PAPER on "Progressive Hypertrophic Interstitial Neuropathy of Infancy," Dejerine and Sottas1 described the clinical and pathologic features of a recessively inherited, severe, mixed peripheral neuropathy with enlargement of nerves. Subsequently, the enlargement of nerves seenclinically and postmortem and the histologic features of onion-bulb formations were found to be associated with disorders which seemed to be different, as judged by their natural history, pattern of inheritance, and biochemical alterations.2-8

In reports based on light-microscopic examination of peripheral nerves, there has been some disagreement on the origin and cellular composition of the onion-bulb formation (also called "cuff" or "whorl"). The majority of authors favored a Schwann-cell origin,6,9-19 though a few of them noted a connective-tissue constituent in the whorl.14,15 Dejerine and Sottas,1 and particularly Dejerine20 with AndréThomas,2 and others22-26 favored a connective-tissue origin. That myelinated fibers lay at the center of

REFERENCES

Dejerine, J., and Sottas, J.:  Sur la névrite interstitielle, hypertrophique et progressive de l'enfance , C R Soc Biol 45:63-96, 1893;.
Dawidenkow, S.:  Űber einige strittige Fragen der Nosographie des neurotischen Muskelschwundes , Z Ges Neurol Psychiat 129:244-249, 1930;.
Refsum, S.:  Heredoataxia hemeralopica polyneuritiformis et tidligere ikke beskrevet familiaert syndrom? En foreløbig meddelelse , Nord Med 28:2682-2685 ( (Dec 21) ) 1945;.
Refsum, S.:  Heredopathia Atactica Polyneuritiformis: A Familial Syndrome Not Hitherto Described , Acta Psychiat Neurol , (Suppl 38) , pp 1-303, 1946;.
Cammermeyer, J.:  Neuropathological Changes in Hereditary Neuropathies: Manifestation of the Syndrome Heredopathia Atactica Polyneuritiformis in the Presence of Interstitial Hypertrophic Polyneuropathy , J Neuropath Exp Neurol 15:340-361 ( (July) ) 1956;.
Austin, J.H.:  Observations on the Syndrome of Hypertrophic Neuritis (the Hypertrophic Interstitial Radiculo-neuropathies) , Medicine 35:187-237 ( (Sept) ) 1956;.
Dyck, P.J., and Lambert, E.H.:  Lower Motor and Primary Sensory Neuron Diseases With Peroneal Muscular Atrophy: I. Neurologic, Genetic, and Electrophysiologic Findings in Hereditary Polyneuropathies , Arch Neurol 18:603-618 ( (June) ) 1968;.
Dyck, P.J., and Lambert, E.H.:  Lower Motor and Primary Sensory Neuron Diseases With Peroneal Muscular Atrophy: II. Neurologic, Genetic, and Electrophysiologic Findings in Various Neuronal Degenerations , Arch Neurol 18:619-625 ( (June) ) 1968;.
Boveri, P.:  De la névrite hypertrophique familiale (type Pierre Marie) , Sem Med 30:145-150 ( (March 30) ) 1910;.
Yokomori, K.:  Űber Neuritis interstitialis hypertrophica et progressiva (Déjérine und Sottas) mit einem Sektionsbefund , Tokyo Imperial Univ Coll Med Mitt 15:1-48 ( (Dec 27) ) 1915;.
Marie, P., and Bertrand, I.:  Contribution a l'anatomie pathologique de la névrite hypertrophique familiale , Ann Med 5:209-238 ( (May-June) ) 1918;.
Roussy, G., and Cornil, L.:  Névrite hypertrophique progressive non familiale de l'adulte , Ann Med 6:296-305, 1919;.
Souques, A., and Bertrand, I.:  Contribution a l'étude anatomo-pathologique de la névrite hypertrophique familiale , Ann Med 9:305-329 ( (April) ) 1921;.
Bielschowsky, M.:  Familiare hypertrophische Neuritis und Neurofibromatose , J Psych Neurol 29:182-205, 1922;.
Slauck:  Űber progressive hypertrophische Neuritis (Hoffmannsche Krankheit) , Z Ges Neurol Psychiat 92:34-77, 1924;.
Harris, W., and Newcomb, W.D.:  A Case of Relapsing Interstitial Hypertrophic Polyneuritis , Brain 52:108-116 ( (April) ) 1929;.
Cornil, L., et al:  Nevrite hypertrophique progressive non familiale (étude anatomo-clinique) , Rev Neurol 1:1187-1192 ( (June) ) 1930;.
Wolf, A.; Rubinowitz, A.H.; and Burchell, S.C.:  Interstitial Hypertrophic Neuritis of Déjérine and Sottas: A Report of Three Cases , Bull Neurol Inst NY 2:373-428 ( (Nov) ) 1932;.
Souques, A., and Bertrand, I.:  Un cas anatomo-clinique atypique de névrite hypertrophique progressive de l'enfance , Rev Neurol 2:513-530 ( (Nov) ) 1934;.
Dejerine, J.:  Contribution a l'étude de la névrite interstitielle hypertrophique et progressive de l'enfance , Rev Med 16:881-925 ( (Nov) ) 1896;.
Dejerine J., and André-Thomas:  Sur la névrite interstitielle hypertrophique et progressive de l'enfance , Nouv Iconog Salpetriere 19:477-509 ( (Nov-Dec) ) 1906;.
Schaller, W.F.:  Progressive Interstitial Hypertrophic Neuritis of Childhood of Déjérine and Sottas: Report of a Case , Arch Intern Med 10:399-404 ( (Oct) ) 1912;.
Russell, W.R., and Garland, H.G.:  Progressive Hypertrophic Polyneuritis, With Case Reports , Brain 53:376-384 ( (Oct) ) 1930;.
Sears, W.G.:  Progressive Hypertrophic Polyneuritis , J Neurol Psychopath 12:137-147 ( (Oct) ) 1931;.
Krücke, W.:  ödem und seröse Entzündung im peripheren Nerven , Virchow Arch Path Anat 308:1-13 ( (Nov 26) ) 1941;.
Krücke, W.:  Zur Histopathologie der neuralen Muskelatrophie, der hypertrophischen Neuritis und Neurofibromatose , Arch Psychiat Nervenkr 115:180-236 ( (Nov 17) ) 1942;.
Gombault, A., and Mallet:  Un cas de tabés ayant débuté dans i'enfance: Autopsie , Arch Med Exp Anat Path 1:385-415, 1889;.
Gruner, J.E.:  La biopsie nerveuse en microscopie électronique , C R Soc Biol 154:1632-1634, 1960;.
Dyck, P.J.:  Histologic Measurements and Fine Structure of Biopsied Sural Nerve: Normal, and in Peroneal Muscular Atrophy, Hypertrophic Neuropathy, and Congenital Sensory Neuropathy , Mayo Clin Proc 41:742-774 ( (Nov) ) 1966;.
Garcin, R., et al:  Etude au microscope électronique du nerf périphérique prélevé par biopsie dans quatre cas de néverite hypertrophique de Dejerine-Sottas , Rev Neurol 115:917-932 ( (Nov) ) 1966;.
Webster, H. deF., et al:  The Role of Schwann Cells in the Formation of "Onion Bulbs" Found in Chronic Neuropathies , J Neuropath Exp Neurol 26:276-299 ( (April) ) 1967;.
Weller, R.O.:  An Electron Microscopic Study of Hypertrophic Neuropathy of Dejerine and Sottas , J Neurol Neurosurg Psychiat 30:111-125 ( (April) ) 1967;.
Thomas, P.K., and Lascelles, R.G.:  Hypertrophic Neuropathy , Quart J Med 36:223-238 ( (April) ) 1967;.
Dyck, P.J., and Gomez, M.R.:  Segmental Demyelinization in Dejerine-Sottas Disease: Light, Phase-Contrast, and Electron Microscopic Studies , Mayo Clin Proc 43:280-296 ( (April) ) 1968;.
Gutrecht, J.A., and Dyck, P.J.:  Segmental Demyelinization in Peroneal Muscular Atrophy: Nerve Fibers Teased From Sural Nerve Biopsy Specimens , Mayo Clin Proc 41:775-777 ( (Nov) ) 1966;.
Dyck, P.J., et al:  Histologic and Teased-Fiber Measurements of Sural Nerve in Disorders of Lower Motor and Primary Sensory Neurons , Mayo Clin Proc 43:81-123 ( (Feb) ) 1968;.
Creutzfeldt, H.G.; Curtius, F.; and Krüger, K.H.:  Zur Klinik, Histologie und Genealogie der Déjerine-Sottasschen Krankheit , Arch Psychiat Nervenkr 186:341-360 ( (June 1) ) 1951;.
Nichols, P.C.; Dyck, P.J.; and Miller, D.R.:  Experimental Hypertrophic Neuropathy: Change in Fascicular Area and Fiber Spectrum After Acute Crush Injury , Mayo Clin Proc 43:297-305 ( (April) ) 1968;.
Weller, R.O., and Das Gupta, T.K.:  Experimental Hypertrophic Neuropathy: An Electron Miroscope Study , J Neurol Neurosurg Psychiat 31:34-42 ( (Feb) ) 1968;.
Denny-Brown, D., and Brenner, C.:  Lesion in Peripheral Nerve Resulting From Compression by Spring Clip , Arch Neurol Psychiat 52:1-19 ( (July) ) 1944;.
Mayer, R.F., and Denny-Brown, D.:  Conduction Velocity in Peripheral Nerve During Experimental Demyelination in the Cat , Neurology 14:714-726 ( (Aug) ) 1964;.
Lampert, P.W., and Schochet, S.S., Jr.:  Demyelination and Remyelination in Lead Neuropathy: Electron Microscopic Studies , J Neuropath Exp Neurol 27:527-545 ( (Oct) ) 1968;.

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Dejerine, J., and Sottas, J.:  Sur la névrite interstitielle, hypertrophique et progressive de l'enfance , C R Soc Biol 45:63-96, 1893;.
Dawidenkow, S.:  Űber einige strittige Fragen der Nosographie des neurotischen Muskelschwundes , Z Ges Neurol Psychiat 129:244-249, 1930;.
Refsum, S.:  Heredoataxia hemeralopica polyneuritiformis et tidligere ikke beskrevet familiaert syndrom? En foreløbig meddelelse , Nord Med 28:2682-2685 ( (Dec 21) ) 1945;.
Refsum, S.:  Heredopathia Atactica Polyneuritiformis: A Familial Syndrome Not Hitherto Described , Acta Psychiat Neurol , (Suppl 38) , pp 1-303, 1946;.
Cammermeyer, J.:  Neuropathological Changes in Hereditary Neuropathies: Manifestation of the Syndrome Heredopathia Atactica Polyneuritiformis in the Presence of Interstitial Hypertrophic Polyneuropathy , J Neuropath Exp Neurol 15:340-361 ( (July) ) 1956;.
Austin, J.H.:  Observations on the Syndrome of Hypertrophic Neuritis (the Hypertrophic Interstitial Radiculo-neuropathies) , Medicine 35:187-237 ( (Sept) ) 1956;.
Dyck, P.J., and Lambert, E.H.:  Lower Motor and Primary Sensory Neuron Diseases With Peroneal Muscular Atrophy: I. Neurologic, Genetic, and Electrophysiologic Findings in Hereditary Polyneuropathies , Arch Neurol 18:603-618 ( (June) ) 1968;.
Dyck, P.J., and Lambert, E.H.:  Lower Motor and Primary Sensory Neuron Diseases With Peroneal Muscular Atrophy: II. Neurologic, Genetic, and Electrophysiologic Findings in Various Neuronal Degenerations , Arch Neurol 18:619-625 ( (June) ) 1968;.
Boveri, P.:  De la névrite hypertrophique familiale (type Pierre Marie) , Sem Med 30:145-150 ( (March 30) ) 1910;.
Yokomori, K.:  Űber Neuritis interstitialis hypertrophica et progressiva (Déjérine und Sottas) mit einem Sektionsbefund , Tokyo Imperial Univ Coll Med Mitt 15:1-48 ( (Dec 27) ) 1915;.
Marie, P., and Bertrand, I.:  Contribution a l'anatomie pathologique de la névrite hypertrophique familiale , Ann Med 5:209-238 ( (May-June) ) 1918;.
Roussy, G., and Cornil, L.:  Névrite hypertrophique progressive non familiale de l'adulte , Ann Med 6:296-305, 1919;.
Souques, A., and Bertrand, I.:  Contribution a l'étude anatomo-pathologique de la névrite hypertrophique familiale , Ann Med 9:305-329 ( (April) ) 1921;.
Bielschowsky, M.:  Familiare hypertrophische Neuritis und Neurofibromatose , J Psych Neurol 29:182-205, 1922;.
Slauck:  Űber progressive hypertrophische Neuritis (Hoffmannsche Krankheit) , Z Ges Neurol Psychiat 92:34-77, 1924;.
Harris, W., and Newcomb, W.D.:  A Case of Relapsing Interstitial Hypertrophic Polyneuritis , Brain 52:108-116 ( (April) ) 1929;.
Cornil, L., et al:  Nevrite hypertrophique progressive non familiale (étude anatomo-clinique) , Rev Neurol 1:1187-1192 ( (June) ) 1930;.
Wolf, A.; Rubinowitz, A.H.; and Burchell, S.C.:  Interstitial Hypertrophic Neuritis of Déjérine and Sottas: A Report of Three Cases , Bull Neurol Inst NY 2:373-428 ( (Nov) ) 1932;.
Souques, A., and Bertrand, I.:  Un cas anatomo-clinique atypique de névrite hypertrophique progressive de l'enfance , Rev Neurol 2:513-530 ( (Nov) ) 1934;.
Dejerine, J.:  Contribution a l'étude de la névrite interstitielle hypertrophique et progressive de l'enfance , Rev Med 16:881-925 ( (Nov) ) 1896;.
Dejerine J., and André-Thomas:  Sur la névrite interstitielle hypertrophique et progressive de l'enfance , Nouv Iconog Salpetriere 19:477-509 ( (Nov-Dec) ) 1906;.
Schaller, W.F.:  Progressive Interstitial Hypertrophic Neuritis of Childhood of Déjérine and Sottas: Report of a Case , Arch Intern Med 10:399-404 ( (Oct) ) 1912;.
Russell, W.R., and Garland, H.G.:  Progressive Hypertrophic Polyneuritis, With Case Reports , Brain 53:376-384 ( (Oct) ) 1930;.
Sears, W.G.:  Progressive Hypertrophic Polyneuritis , J Neurol Psychopath 12:137-147 ( (Oct) ) 1931;.
Krücke, W.:  ödem und seröse Entzündung im peripheren Nerven , Virchow Arch Path Anat 308:1-13 ( (Nov 26) ) 1941;.
Krücke, W.:  Zur Histopathologie der neuralen Muskelatrophie, der hypertrophischen Neuritis und Neurofibromatose , Arch Psychiat Nervenkr 115:180-236 ( (Nov 17) ) 1942;.
Gombault, A., and Mallet:  Un cas de tabés ayant débuté dans i'enfance: Autopsie , Arch Med Exp Anat Path 1:385-415, 1889;.
Gruner, J.E.:  La biopsie nerveuse en microscopie électronique , C R Soc Biol 154:1632-1634, 1960;.
Dyck, P.J.:  Histologic Measurements and Fine Structure of Biopsied Sural Nerve: Normal, and in Peroneal Muscular Atrophy, Hypertrophic Neuropathy, and Congenital Sensory Neuropathy , Mayo Clin Proc 41:742-774 ( (Nov) ) 1966;.
Garcin, R., et al:  Etude au microscope électronique du nerf périphérique prélevé par biopsie dans quatre cas de néverite hypertrophique de Dejerine-Sottas , Rev Neurol 115:917-932 ( (Nov) ) 1966;.
Webster, H. deF., et al:  The Role of Schwann Cells in the Formation of "Onion Bulbs" Found in Chronic Neuropathies , J Neuropath Exp Neurol 26:276-299 ( (April) ) 1967;.
Weller, R.O.:  An Electron Microscopic Study of Hypertrophic Neuropathy of Dejerine and Sottas , J Neurol Neurosurg Psychiat 30:111-125 ( (April) ) 1967;.
Thomas, P.K., and Lascelles, R.G.:  Hypertrophic Neuropathy , Quart J Med 36:223-238 ( (April) ) 1967;.
Dyck, P.J., and Gomez, M.R.:  Segmental Demyelinization in Dejerine-Sottas Disease: Light, Phase-Contrast, and Electron Microscopic Studies , Mayo Clin Proc 43:280-296 ( (April) ) 1968;.
Gutrecht, J.A., and Dyck, P.J.:  Segmental Demyelinization in Peroneal Muscular Atrophy: Nerve Fibers Teased From Sural Nerve Biopsy Specimens , Mayo Clin Proc 41:775-777 ( (Nov) ) 1966;.
Dyck, P.J., et al:  Histologic and Teased-Fiber Measurements of Sural Nerve in Disorders of Lower Motor and Primary Sensory Neurons , Mayo Clin Proc 43:81-123 ( (Feb) ) 1968;.
Creutzfeldt, H.G.; Curtius, F.; and Krüger, K.H.:  Zur Klinik, Histologie und Genealogie der Déjerine-Sottasschen Krankheit , Arch Psychiat Nervenkr 186:341-360 ( (June 1) ) 1951;.
Nichols, P.C.; Dyck, P.J.; and Miller, D.R.:  Experimental Hypertrophic Neuropathy: Change in Fascicular Area and Fiber Spectrum After Acute Crush Injury , Mayo Clin Proc 43:297-305 ( (April) ) 1968;.
Weller, R.O., and Das Gupta, T.K.:  Experimental Hypertrophic Neuropathy: An Electron Miroscope Study , J Neurol Neurosurg Psychiat 31:34-42 ( (Feb) ) 1968;.
Denny-Brown, D., and Brenner, C.:  Lesion in Peripheral Nerve Resulting From Compression by Spring Clip , Arch Neurol Psychiat 52:1-19 ( (July) ) 1944;.
Mayer, R.F., and Denny-Brown, D.:  Conduction Velocity in Peripheral Nerve During Experimental Demyelination in the Cat , Neurology 14:714-726 ( (Aug) ) 1964;.
Lampert, P.W., and Schochet, S.S., Jr.:  Demyelination and Remyelination in Lead Neuropathy: Electron Microscopic Studies , J Neuropath Exp Neurol 27:527-545 ( (Oct) ) 1968;.

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