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August 1985, Vol 42, No. 8 >
Article | August 1985

Primary Aberrant Regeneration of the Oculomotor Nerve: Title and subTitle BreakOccurrence in a Patient With Abetalipoproteinemia

David A. Cohen, MD; Thomas M. Bosley, MD; Peter J. Savino, MD; Robert C. Sergott, MD; Norman J. Schatz, MD
[+] Author Affiliations

Accepted for publication June 13, 1984.

Reprint requests to Neuro-ophthalmology Service, Wills Eye Hospital, Ninth and Walnut streets, Philadelphia, PA 19107 (Dr Bosley).


Arch Neurol. 1985;42(8):821-823. doi:10.1001/archneur.1985.04210090089025
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Primary aberrant regeneration of the oculomotor nerve is a clinical syndrome involving eyelid, pupil, and extraocular movement abnormalities following damage to the peripheral portion of the oculomotor nerve. It has been described only with meningiomas and internal carotid artery aneurysms within the cavernous sinus.1-6 We describe herein a patient with abetalipoproteinemia (Bassen-Kornzweig syndrome [BKS]) who has bilateral primary aberrant regeneration of the oculomotor nerve.

REPORT OF A CASE  A 31-year-old right-handed man was diagnosed as having BKS at age 9 years because of acanthocytosis and serum β lipoprotein levels less than 4% of normal7. Visual fields and funduscopic examination findings were normal, and visual acuity was 6/15 - 1 OD and 6/15 + 1 OS uncorrected. Extraocular movements and pupillary reaction were normal at that time, but he had moderate bilateral ptosis, greater on the right. He required assistance to walk, and his gait was broad based and ataxic. At age

REFERENCES

1 +
Levin PM:  Intracranial aneurysms . Arch Neurol 1952;;67:771-786.
2 +
Schatz NJ, Savino PJ, Corbett JJ:  Primary aberrant oculomotor regeneration: A sign of intracavernous meningioma . Arch Neurol 1977;; 34:29-32.
3 +
Boghen D, Chartrand JP, Laflamme P, et al:  Primary aberrant third nerve regeneration . Ann Neurol 1979;;6:415-418.
4 +
Trobe JD, Glaser JS, Post JD:  Meningiomas and aneurysms of the cavernous sinus . Arch Ophthalmol 1976;;94:571-575.
5 +
Cox TA, Wurster JB, Godfrey WA:  Primary aberrant oculomotor regeneration due to intracranial aneurysm . Arch Neurol 1979;;36:570-571.
6 +
Lepore FE, Glaser JS:  Misdirection revisited: A critical appraisal of acquired oculomotor nerve synkinesis . Arch Ophthalmol 1980;;98:2206-2209.
7 +
Schwartz JF, Rowland LP, Eder H, et al:  Bassen-Kornzweig syndrome: Deficiency of serum beta-lipoprotein . Arch Neurol 1963;;8:438-454.
8 +
Isselbacher KJ, Scheig R, Plotkin GR, et al:  Congenital belalipoprotein deficiency: An hereditary disorder involving a defect in the absorption and transport of lipids . Medicine 1964;;43:347-361.
9 +
Czarnecki JSC, Thompson HS:  The iris sphincter in aberrant regeneration of the third nerve . Arch Ophthalmol 1978;;96:1606-1610.
10 +
Kayden HJ:  Abetalipoproteinemia . Annu Rev Med 1972;;23:285-296.
11 +
Forster RK, Schatz NJ, Smith JL:  A subtle eyelid sign in aberrant regeneration of the third nerve . Am J Ophthalmol 1969;;67:696-698.
12 +
Yee RD, Cogan DG, Zee DS:  Ophthalmoplegia and dissociated nystagmus in abetalipoproteinemia . Arch Ophthalmol 1976;;94:571-575.
13 +
Jampel RS, Falls HF:  Atypical retinitis pigmentosa, acanthocytosis, and heredodegenerative neuromuscular disease . Arch Ophthalmol 1958;;59:818-820.
14 +
Yuill GM, Scholz C, Lascelles RG:  Abetalipoproteinemia: A case report with pathological studies . Postgrad Med J 1976;;52:713-720.
15 +
Sobrevilla LA, Goodman ML, Kane CA:  Demyelinating central nervous system disease, macular atrophy, and acanthocytosis (BassenKornzweig syndrome) . Am J Med 1964;;37:821-828.
16 +
Dische MR, Porro RS:  The cardiac lesions in Bassen-Kornzweig syndrome: Report of a case with autopsy findings . Am J Med 1970;;49:568-571.
17 +
Miller RG, Davis CJF, Illingworth R, et al:  The neuropathy of abetalipoproteinemia . Neurology 1980;;30:1286-1291.
18 +
Kott E, Delpre G, Kadish U, et al:  Abetalipoproteinemia (Bassen-Kornzweig syndrome): Muscle involvement . Acta Neuropathol 1977;; 37:255-258.
19 +
Walsh FB:  Third nerve regeneration: A clinical evaluation . Br J Ophthalmol 1957;;41:577-598.
20 +
Illingworth DR, Connor WE, Miller RG:  Abetalipoproteinemia: Report of two cases and review of therapy . Arch Neurol 1980;;37:659-662.
21 +
Muller DPR, Lloyd JK:  Effect of large oral doses of vitamin E on the neurological sequellae of patients with abetalipoproteinemia . Ann NY Acad Sci 1982;;133-144.
22 +
Walsh FB, Hoyt WF: Clinical Neuro-ophthalmology , ed 3. Baltimore, Williams & Wilkins Co. 1969;, vol 1, pp 945-954
23 +
vol 2, pp 1133, 1221, 1468-1469.
24 +
Dyck PJ, Lais AC, Ohta M, et al:  Chronic inflammatory polyradiculoneuropathy . Mayo Clin Proc 1975;;50:621-637.

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1 +
Levin PM:  Intracranial aneurysms . Arch Neurol 1952;;67:771-786.
2 +
Schatz NJ, Savino PJ, Corbett JJ:  Primary aberrant oculomotor regeneration: A sign of intracavernous meningioma . Arch Neurol 1977;; 34:29-32.
3 +
Boghen D, Chartrand JP, Laflamme P, et al:  Primary aberrant third nerve regeneration . Ann Neurol 1979;;6:415-418.
4 +
Trobe JD, Glaser JS, Post JD:  Meningiomas and aneurysms of the cavernous sinus . Arch Ophthalmol 1976;;94:571-575.
5 +
Cox TA, Wurster JB, Godfrey WA:  Primary aberrant oculomotor regeneration due to intracranial aneurysm . Arch Neurol 1979;;36:570-571.
6 +
Lepore FE, Glaser JS:  Misdirection revisited: A critical appraisal of acquired oculomotor nerve synkinesis . Arch Ophthalmol 1980;;98:2206-2209.
7 +
Schwartz JF, Rowland LP, Eder H, et al:  Bassen-Kornzweig syndrome: Deficiency of serum beta-lipoprotein . Arch Neurol 1963;;8:438-454.
8 +
Isselbacher KJ, Scheig R, Plotkin GR, et al:  Congenital belalipoprotein deficiency: An hereditary disorder involving a defect in the absorption and transport of lipids . Medicine 1964;;43:347-361.
9 +
Czarnecki JSC, Thompson HS:  The iris sphincter in aberrant regeneration of the third nerve . Arch Ophthalmol 1978;;96:1606-1610.
10 +
Kayden HJ:  Abetalipoproteinemia . Annu Rev Med 1972;;23:285-296.
11 +
Forster RK, Schatz NJ, Smith JL:  A subtle eyelid sign in aberrant regeneration of the third nerve . Am J Ophthalmol 1969;;67:696-698.
12 +
Yee RD, Cogan DG, Zee DS:  Ophthalmoplegia and dissociated nystagmus in abetalipoproteinemia . Arch Ophthalmol 1976;;94:571-575.
13 +
Jampel RS, Falls HF:  Atypical retinitis pigmentosa, acanthocytosis, and heredodegenerative neuromuscular disease . Arch Ophthalmol 1958;;59:818-820.
14 +
Yuill GM, Scholz C, Lascelles RG:  Abetalipoproteinemia: A case report with pathological studies . Postgrad Med J 1976;;52:713-720.
15 +
Sobrevilla LA, Goodman ML, Kane CA:  Demyelinating central nervous system disease, macular atrophy, and acanthocytosis (BassenKornzweig syndrome) . Am J Med 1964;;37:821-828.
16 +
Dische MR, Porro RS:  The cardiac lesions in Bassen-Kornzweig syndrome: Report of a case with autopsy findings . Am J Med 1970;;49:568-571.
17 +
Miller RG, Davis CJF, Illingworth R, et al:  The neuropathy of abetalipoproteinemia . Neurology 1980;;30:1286-1291.
18 +
Kott E, Delpre G, Kadish U, et al:  Abetalipoproteinemia (Bassen-Kornzweig syndrome): Muscle involvement . Acta Neuropathol 1977;; 37:255-258.
19 +
Walsh FB:  Third nerve regeneration: A clinical evaluation . Br J Ophthalmol 1957;;41:577-598.
20 +
Illingworth DR, Connor WE, Miller RG:  Abetalipoproteinemia: Report of two cases and review of therapy . Arch Neurol 1980;;37:659-662.
21 +
Muller DPR, Lloyd JK:  Effect of large oral doses of vitamin E on the neurological sequellae of patients with abetalipoproteinemia . Ann NY Acad Sci 1982;;133-144.
22 +
Walsh FB, Hoyt WF: Clinical Neuro-ophthalmology , ed 3. Baltimore, Williams & Wilkins Co. 1969;, vol 1, pp 945-954
23 +
vol 2, pp 1133, 1221, 1468-1469.
24 +
Dyck PJ, Lais AC, Ohta M, et al:  Chronic inflammatory polyradiculoneuropathy . Mayo Clin Proc 1975;;50:621-637.

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