Abstract
The true frequency of blepharoptosis and its particular subtypes is not known. The vast majority of upper eyelid ptosis is due to benign etiologies, including involutional and congenital. A recent review of 251 patients across all ages in a tertiary care center reported aponeurotic ptosis as the most frequent subtype (60%), followed by a relatively similar distribution of traumatic, congenital, and mechanical (9–11%). Neurogenic etiologies made up a minority of cases (6%), with 38% due to oculomotor palsy, 29% from myasthenia gravis, and 7% from Horner syndrome. However, this small subset of ptosis cannot be missed - neurologic ptosis may be the sole manifestation of significant systemic and neurologic disease.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Houghton Mifflin Harcourt Publishing Company. The American Heritage dictionary of the English language. 4th ed. Boston: Houghton Mifflin; 2000.
Murchison AP, Sires BA, Jian-Amadi A. Margin reflex distance in different ethnic groups. Arch Facial Plast Surg. 2009;11(5):303–5.
Price KM, Gupta PK, Woodward JA, Stinnett SS, Murchison AP. Eyebrow and eyelid dimensions: an anthropometric analysis of African Americans and Caucasians. Plast Reconstr Surg. 2009;124(2):615–23.
Lim JM, Hou JH, Singa RM, Aakalu VK, Setabutr P. Relative incidence of blepharoptosis subtypes in an oculoplastics practice at a tertiary care center. Orbit. 2013;32(4):231–4.
Nerad J. Evaluation and treatment of the patient with ptosis. In: Nerad JA, editor. Oculoplastic surgery the requisites in ophthalmology. St. Louis: Mosby Inc; 2001. p. 157–92.
Putman JRNW, Tanenbaum M, McCord CD Jr. Blepharoptosis. In: McCord Jr CD, Tannenbaum M, Nunery WR, editors. Oculoplastic surgery. New York: Raven Press; 1995. p. 175–220.
Lam BL, Thompson HS, Corbett JJ. The prevalence of simple anisocoria. Am J Ophthalmol. 1987;104(1):69–73.
Moeller JJ, Maxner CE. The dilated pupil: an update. Curr Neurol Neurosci Rep. 2007;7(5):417–22.
Bartley GB. The epidemiologic characteristics and clinical course of ophthalmopathy associated with autoimmune thyroid disease in Olmsted County, Minnesota. Trans Am Ophthalmol Soc. 1994;92:477–588.
Bartley GB. The differential diagnosis and classification of eyelid retraction. Ophthalmology. 1996;103(1):168–76.
Chang EL, Rubin PA. Upper and lower eyelid retraction. Int Ophthalmol Clin. 2002;42(2):45–59.
Bartley GB, Fatourechi V, Kadrmas EF, et al. Chronology of Graves’ ophthalmopathy in an incidence cohort. Am J Ophthalmol. 1996;121(4):426–34.
Rubin PA, Rumelt S. Functional indications for enophthalmos repair. Ophthal Plast Reconstr Surg. 1999;15(4):284–92.
Cline RA, Rootman J. Enophthalmos: a clinical review. Ophthalmology. 1984;91(3):229–37.
Mattacks CA, Pond CM. The effects of dietary restriction and exercise on the volume of adipocytes in two intra-orbital depots in the guinea-pig. Br J Nutr. 1985;53(2):207–13.
Athanasiov PA, Prabhakaran VC, Selva D. Non-traumatic enophthalmos: a review. Acta Ophthalmol. 2008;86(4):356–64.
Shields CL, Stopyra GA, Marr BP, Moster ML, Shields JA. Enophthalmos as initial manifestation of occult, mammogram-negative carcinoma of the breast. Ophthalmic Surg Lasers Imaging. 2004;35(1):56–7.
Goldberg RA, Rootman J. Clinical characteristics of metastatic orbital tumors. Ophthalmology. 1990;97(5):620–4.
Soparkar CN, Patrinely JR, Cuaycong MJ, et al. The silent sinus syndrome. A cause of spontaneous enophthalmos. Ophthalmology. 1994;101(4):772–8.
Ficker LA, Collin JR, Lee JP. Management of ipsilateral ptosis with hypotropia. Br J Ophthalmol. 1986;70(10):732–6.
Hallett M, Evinger C, Jankovic J, Stacy M. Update on blepharospasm: report from the BEBRF international workshop. Neurology. 2008;71(16):1275–82.
Frueh BR, Musch DC. Treatment of facial spasm with botulinum toxin. An interim report. Ophthalmology. 1986;93(7):917–23.
Etgen T, Muhlau M, Gaser C, Sander D. Bilateral grey-matter increase in the putamen in primary blepharospasm. J Neurol Neurosurg Psychiatry. 2006;77(9):1017–20.
Obermann M, Yaldizli O, De Greiff A, et al. Morphometric changes of sensorimotor structures in focal dystonia. Mov Disord. 2007;22(8):1117–23.
Colosimo C, Pantano P, Calistri V, Totaro P, Fabbrini G, Berardelli A. Diffusion tensor imaging in primary cervical dystonia. J Neurol Neurosurg Psychiatry. 2005;76(11):1591–3.
Kerrison JB, Lancaster JL, Zamarripa FE, et al. Positron emission tomography scanning in essential blepharospasm. Am J Ophthalmol. 2003;136(5):846–52.
Suzuki Y, Mizoguchi S, Kiyosawa M, et al. Glucose hypermetabolism in the thalamus of patients with essential blepharospasm. J Neurol. 2007;254(7):890–6.
Nakashima K, Kusumi M, Inoue Y, Takahashi K. Prevalence of focal dystonias in the western area of Tottori Prefecture in Japan. Mov Disord. 1995;10(4):440–3.
Nutt JG, Muenter MD, Aronson A, Kurland LT, Melton LJ 3rd. Epidemiology of focal and generalized dystonia in Rochester, Minnesota. Mov Disord. 1988;3(3):188–94.
Epidemiological Study of Dystonia in Europe (ESDE) Collaborative Group. A prevalence study of primary dystonia in eight European countries. J Neurol. 2000;247(10):787–92.
Epidemiologic Study of Dystonia in Europe (ESDE) Collaborative Group. Sex-related influences on the frequency and age of onset of primary dystonia. Neurology. 1999;53(8):1871–3.
Defazio G, Berardelli A, Abbruzzese G, et al. Possible risk factors for primary adult onset dystonia: a case-control investigation by the Italian Movement Disorders Study Group. J Neurol Neurosurg Psychiatry. 1998;64(1):25–32.
Hallett M, Daroff RB. Blepharospasm: report of a workshop. Neurology. 1996;46(5):1213–8.
Defazio G, Martino D, Aniello MS, et al. A family study on primary blepharospasm. J Neurol Neurosurg Psychiatry. 2006;77(2):252–4.
Seiff SR, Zwick OM. Botulinum toxin management of upper facial rhytidosis and blepharospasm. Otolaryngol Clin N Am. 2005;38(5):887–902.
Defazio G, Berardelli A, Abbruzzese G, et al. Risk factors for spread of primary adult onset blepharospasm: a multicentre investigation of the Italian movement disorders study group. J Neurol Neurosurg Psychiatry. 1999;67(5):613–9.
Kanazawa M, Shimohata T, Sato M, Onodera O, Tanaka K, Nishizawa M. Botulinum toxin A injections improve apraxia of eyelid opening without overt blepharospasm associated with neurodegenerative diseases. Mov Disord. 2007;22(4):597–8.
Lamberti P, De Mari M, Zenzola A, Aniello MS, Defazio G. Frequency of apraxia of eyelid opening in the general population and in patients with extrapyramidal disorders. Neurol Sci. 2002;23(Suppl 2):S81–2.
Krack P, Marion MH. “Apraxia of lid opening,” a focal eyelid dystonia: clinical study of 32 patients. Mov Disord. 1994;9(6):610–5.
Boghen D. Apraxia of lid opening: a review. Neurology. 1997;48(6):1491–4.
Defazio G, Livrea P, Lamberti P, et al. Isolated so-called apraxia of eyelid opening: report of 10 cases and a review of the literature. Eur Neurol. 1998;39(4):204–10.
Cherian V, Foroozan R. Benign unilateral apraxia of eyelid opening. Ophthalmology. 2010;117(6):1265–8.
Jordan DR, Anderson RL, Digre KB. Apraxia of lid opening in blepharospasm. Ophthalmic Surg. 1990;21(5):331–4.
Kerty E, Eidal K. Apraxia of eyelid opening: clinical features and therapy. Eur J Ophthalmol. 2006;16(2):204–8.
Holds JB, Anderson RL, Jordan DR, Patrinely JR. Bilateral hemifacial spasm. J Clin Neuroophthalmol. 1990;10(2):153–4.
Auger RG, Whisnant JP. Hemifacial spasm in Rochester and Olmsted County, Minnesota, 1960 to 1984. Arch Neurol. 1990;47(11):1233–4.
Bernardi B, Zimmerman RA, Savino PJ, Adler C. Magnetic resonance tomographic angiography in the investigation of hemifacial spasm. Neuroradiology. 1993;35(8):606–11.
Hosoya T, Watanabe N, Yamaguchi K, Saito S, Nakai O. Three-dimensional-MRI of neurovascular compression in patients with hemifacial spasm. Neuroradiology. 1995;37(5):350–2.
Nielsen VK, Jannetta PJ. Pathophysiology of hemifacial spasm: III. Effects of facial nerve decompression. Neurology. 1984;34(7):891–7.
Nielsen VK. Pathophysiology of hemifacial spasm: II. Lateral spread of the supraorbital nerve reflex. Neurology. 1984;34(4):427–31.
Nielsen VK. Pathophysiology of hemifacial spasm: I. Ephaptic transmission and ectopic excitation. Neurology. 1984;34(4):418–26.
Matsuura N, Kondo A. Trigeminal neuralgia and hemifacial spasm as false localizing signs in patients with a contralateral mass of the posterior cranial fossa. Report of three cases. J Neurosurg. 1996;84(6):1067–71.
Nagata S, Matsushima T, Fujii K, Fukui M, Kuromatsu C. Hemifacial spasm due to tumor, aneurysm, or arteriovenous malformation. Surg Neurol. 1992;38(3):204–9.
Wang A, Jankovic J. Hemifacial spasm: clinical findings and treatment. Muscle Nerve. 1998;21(12):1740–7.
Koutsis G, Kokotis P, Sarrigiannis P, Anagnostouli M, Sfagos C, Karandreas N. Spastic paretic hemifacial contracture in multiple sclerosis: a neglected clinical and EMG entity. Mult Scler. 2008;14(7):927–32.
Krauss JK, Wakhloo AK, Scheremet R, Seeger W. Facial myokymia and spastic paretic facial contracture as the result of anaplastic pontocerebellar glioma. Neurosurgery. 1993;32(6):1031–4.
Savino PJ, Maus M. Botulinum toxin therapy. Neurol Clin. 1991;9(1):205–24.
Simpson DM, Blitzer A, Brashear A, et al. Assessment: botulinum neurotoxin for the treatment of movement disorders (an evidence-based review): report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2008;70(19):1699–706.
Bilyk JR, Yen MT, Bradley EA, Wladis EJ, Mawn LA. Chemodenervation for the treatment of facial dystonia: a report by the American Academy of Ophthalmology. Ophthalmology. 2018;125(9):1459–67.
Blackburn MK, Lamb RD, Digre KB, et al. FL-41 tint improves blink frequency, light sensitivity, and functional limitations in patients with benign essential blepharospasm. Ophthalmology. 2009;116(5):997–1001.
Chapman KL, Bartley GB, Waller RR, Hodge DO. Follow-up of patients with essential blepharospasm who underwent eyelid protractor myectomy at the Mayo Clinic from 1980 through 1995. Ophthal Plast Reconstr Surg. 1999;15(2):106–10.
Grivet D, Robert PY, Thuret G, et al. Assessment of blepharospasm surgery using an improved disability scale: study of 138 patients. Ophthal Plast Reconstr Surg. 2005;21(3):230–4.
Nicoletti AG, Pereira IC, Matayoshi S. Browlifting as an alternative procedure for apraxia of eyelid opening. Ophthal Plast Reconstr Surg. 2009;25(1):46–7.
Fante RG, Frueh BR. Differential section of the seventh nerve as a tertiary procedure for the treatment of benign essential blepharospasm. Ophthal Plast Reconstr Surg. 2001;17(4):276–80.
Barker FG 2nd, Jannetta PJ, Bissonette DJ, Shields PT, Larkins MV, Jho HD. Microvascular decompression for hemifacial spasm. J Neurosurg. 1995;82(2):201–10.
Artz GJ, Hux FJ, Larouere MJ, Bojrab DI, Babu S, Pieper DR. Endoscopic vascular decompression. Otol Neurotol. 2008;29(7):995–1000.
Pfaffenbach DD, Cross HE, Kearns TP. Congenital anomalies in Duane’s retraction syndrome. Arch Ophthalmol. 1972;88(6):635–9.
DeRespinis PA, Caputo AR, Wagner RS, Guo S. Duane’s retraction syndrome. Surv Ophthalmol. 1993;38(3):257–88.
Raab EL. Clinical features of Duane’s syndrome. J Pediatr Ophthalmol Strabismus. 1986;23(2):64–8.
Huber A. Electrophysiology of the retraction syndromes. Br J Ophthalmol. 1974;58(3):293–300.
Alexandrakis G, Saunders RA. Duane retraction syndrome. Ophthalmol Clin N Am. 2001;14(3):407–17.
Fowler AM, Dutton JJ. Floppy eyelid syndrome as a subset of lax eyelid conditions: relationships and clinical relevance (an ASOPRS thesis). Ophthal Plast Reconstr Surg. 2010;26(3):195–204.
Taban M, Perry JD. Plasma leptin levels in patients with floppy eyelid syndrome. Ophthal Plast Reconstr Surg. 2006;22(5):375–7.
Schlotzer-Schrehardt U, Stojkovic M, Hofmann-Rummelt C, Cursiefen C, Kruse FE, Holbach LM. The pathogenesis of floppy eyelid syndrome: involvement of matrix metalloproteinases in elastic fiber degradation. Ophthalmology. 2005;112(4):694–704.
Goldberg R, Seiff S, McFarland J, Simons K, Shorr N. Floppy eyelid syndrome and blepharochalasis. Am J Ophthalmol. 1986;102(3):376–81.
McNab AA. Floppy eyelid syndrome and obstructive sleep apnea. Ophthal Plast Reconstr Surg. 1997;13(2):98–114.
Langford JD, Linberg JV. A new physical finding in floppy eyelid syndrome. Ophthalmology. 1998;105(1):165–9.
Culbertson WW, Tseng SC. Corneal disorders in floppy eyelid syndrome. Cornea. 1994;13(1):33–42.
Dutton JJ. Surgical management of floppy eyelid syndrome. Am J Ophthalmol. 1985;99(5):557–60.
Katusic SK, Beard CM, Wiederholt WC, Bergstralh EJ, Kurland LT. Incidence, clinical features, and prognosis in Bell’s palsy, Rochester, Minnesota, 1968-1982. Ann Neurol. 1986;20(5):622–7.
Kimura J, Rodnitzky RL, Okawara SH. Electrophysiologic analysis of aberrant regeneration after facial nerve paralysis. Neurology. 1975;25(10):989–93.
Celik M, Forta H, Vural C. The development of synkinesis after facial nerve paralysis. Eur Neurol. 2000;43(3):147–51.
Yamamoto E, Nishimura H, Hirono Y. Occurrence of sequelae in Bell’s palsy. Acta Otolaryngol Suppl. 1988;446:93–6.
Moran CJ, Neely JG. Patterns of facial nerve synkinesis. Laryngoscope. 1996;106(12 Pt 1):1491–6.
Rijntjes M, Tegenthoff M, Liepert J, et al. Cortical reorganization in patients with facial palsy. Ann Neurol. 1997;41(5):621–30.
Valls-Sole J, Montero J. Movement disorders in patients with peripheral facial palsy. Mov Disord. 2003;18(12):1424–35.
Keegan DJ, Geerling G, Lee JP, Blake G, Collin JR, Plant GT. Botulinum toxin treatment for hyperlacrimation secondary to aberrant regenerated seventh nerve palsy or salivary gland transplantation. Br J Ophthalmol. 2002;86(1):43–6.
Lee V, Currie Z, Collin JR. Ophthalmic management of facial nerve palsy. Eye (Lond). 2004;18(12):1225–34.
VanSwearingen JM, Brach JS. Changes in facial movement and synkinesis with facial neuromuscular reeducation. Plast Reconstr Surg. 2003;111(7):2370–5.
Cronin GW, Steenerson RL. The effectiveness of neuromuscular facial retraining combined with electromyography in facial paralysis rehabilitation. Otolaryngol Head Neck Surg. 2003;128(4):534–8.
Demer JL, Clark RA, Engle EC. Magnetic resonance imaging evidence for widespread orbital dysinnervation in congenital fibrosis of extraocular muscles due to mutations in KIF21A. Invest Ophthalmol Vis Sci. 2005;46(2):530–9.
Engle EC, Goumnerov BC, McKeown CA, et al. Oculomotor nerve and muscle abnormalities in congenital fibrosis of the extraocular muscles. Ann Neurol. 1997;41(3):314–25.
Engle EC. The genetic basis of complex strabismus. Pediatr Res. 2006;59(3):343–8.
Egan RA, Kerrison JB. Survey of genetic neuro-ophthalmic disorders. Ophthalmol Clin N Am. 2003;16(4):595–605, vii.
Engle EC, Kunkel LM, Specht LA, Beggs AH. Mapping a gene for congenital fibrosis of the extraocular muscles to the centromeric region of chromosome 12. Nat Genet. 1994;7(1):69–73.
Wang SM, Zwaan J, Mullaney PB, et al. Congenital fibrosis of the extraocular muscles type 2, an inherited exotropic strabismus fixus, maps to distal 11q13. Am J Hum Genet. 1998;63(2):517–25.
Engle EC, Marondel I, Houtman WA, et al. Congenital fibrosis of the extraocular muscles (autosomal dominant congenital external ophthalmoplegia): genetic homogeneity, linkage refinement, and physical mapping on chromosome 12. Am J Hum Genet. 1995;57(5):1086–94.
Aubourg P, Krahn M, Bernard R, et al. Assignment of a new congenital fibrosis of extraocular muscles type 3 (CFEOM3) locus, FEOM4, based on a balanced translocation t(2;13) (q37.3;q12.11) and identification of candidate genes. J Med Genet. 2005;42(3):253–9.
Doherty EJ, Macy ME, Wang SM, Dykeman CP, Melanson MT, Engle EC. CFEOM3: a new extraocular congenital fibrosis syndrome that maps to 16q24.2-q24.3. Invest Ophthalmol Vis Sci. 1999;40(8):1687–94.
Heidary G, Engle EC, Hunter DG. Congenital fibrosis of the extraocular muscles. Semin Ophthalmol. 2008;23(1):3–8.
Harley RD, Rodrigues MM, Crawford JS. Congenital fibrosis of the extraocular muscles. J Pediatr Ophthalmol Strabismus. 1978;15(6):346–58.
MOxley RT 3rd. Proximal myotonic myopathy: mini-review of a recently delineated clinical disorder. Neuromuscul Disord. 1996;6(2):87–93.
Day JW, Ricker K, Jacobsen JF, et al. Myotonic dystrophy type 2: molecular, diagnostic and clinical spectrum. Neurology. 2003;60(4):657–64.
Ranum LP, Day JW. Myotonic dystrophy: RNA pathogenesis comes into focus. Am J Hum Genet. 2004;74(5):793–804.
Mathieu J, Allard P, Potvin L, Prevost C, Begin P. A 10-year study of mortality in a cohort of patients with myotonic dystrophy. Neurology. 1999;52(8):1658–62.
Brais B, Xie YG, Sanson M, et al. The oculopharyngeal muscular dystrophy locus maps to the region of the cardiac alpha and beta myosin heavy chain genes on chromosome 14q11.2-q13. Hum Mol Genet. 1995;4(3):429–34.
Blumen SC, Nisipeanu P, Sadeh M, et al. Epidemiology and inheritance of oculopharyngeal muscular dystrophy in Israel. Neuromuscul Disord. 1997;7(Suppl 1):S38–40.
Brunet G, Tome FM, Eymard B, Robert JM, Fardeau M. Genealogical study of oculopharyngeal muscular dystrophy in France. Neuromuscul Disord. 1997;7(Suppl 1):S34–7.
Grewal RP, Karkera JD, Grewal RK, Detera-Wadleigh SD. Mutation analysis of oculopharyngeal muscular dystrophy in Hispanic American families. Arch Neurol. 1999;56(11):1378–81.
Becher MW, Morrison L, Davis LE, et al. Oculopharyngeal muscular dystrophy in Hispanic New Mexicans. JAMA. 2001;286(19):2437–40.
Bouchard JP, Brais B, Brunet D, Gould PV, Rouleau GA. Recent studies on oculopharyngeal muscular dystrophy in Quebec. Neuromuscul Disord. 1997;7(Suppl 1):S22–9.
Victor M, Hayes R, Adams RD. Oculopharyngeal muscular dystrophy. A familial disease of late life characterized by dysphagia and progressive ptosis of the eyelids. N Engl J Med. 1962;267:1267–72.
Murphy SF, Drachman DB. The oculopharyngeal syndrome. JAMA. 1968;203(12):1003–8.
Hill ME, Creed GA, McMullan TF, et al. Oculopharyngeal muscular dystrophy: phenotypic and genotypic studies in a UK population. Brain. 2001;124(Pt 3):522–6.
Brais B, Bouchard JP, Xie YG, et al. Short GCG expansions in the PABP2 gene cause oculopharyngeal muscular dystrophy. Nat Genet. 1998;18(2):164–7.
Blumen SC, Brais B, Korczyn AD, et al. Homozygotes for oculopharyngeal muscular dystrophy have a severe form of the disease. Ann Neurol. 1999;46(1):115–8.
Ruegg S, Lehky Hagen M, Hohl U, et al. Oculopharyngeal muscular dystrophy - an under-diagnosed disorder? Swiss Med Wkly. 2005;135(39–40):574–86.
Rodrigue D, Molgat YM. Surgical correction of blepharoptosis in oculopharyngeal muscular dystrophy. Neuromuscul Disord. 1997;7(Suppl 1):S82–4.
Codere F. Oculopharyngeal muscular dystrophy. Can J Ophthalmol. 1993;28(1):1–2.
Caron MJ, Girard F, Girard DC, et al. Cisatracurium pharmacodynamics in patients with oculopharyngeal muscular dystrophy. Anesth Analg. 2005;100(2):393–7.
Edmond JC. Mitochondrial disorders. Int Ophthalmol Clin. 2009;49(3):27–33.
Greaves LC, Yu-Wai-Man P, Blakely EL, et al. Mitochondrial DNA defects and selective extraocular muscle involvement in CPEO. Invest Ophthalmol Vis Sci. 2010;51(7):3340–6.
Finsterer J. Mitochondrial disorder mimicking ocular myasthenia. Acta Neurol Belg. 2010;110(1):110–2.
Lelli GJ Jr, Musch DC, Frueh BR, Nelson CC. Outcomes in silicone rod frontalis suspension surgery for high-risk noncongenital blepharoptosis. Ophthal Plast Reconstr Surg. 2009;25(5):361–5.
Almousa R, Charlton A, Rajesh ST, Sundar G, Amrith S. Optimizing muscle biopsy for the diagnosis of mitochondrial myopathy. Ophthal Plast Reconstr Surg. 2009;25(5):366–70.
Lopez-Gallardo E, Lopez-Perez MJ, Montoya J, Ruiz-Pesini E. CPEO and KSS differ in the percentage and location of the mtDNA deletion. Mitochondrion. 2009;9(5):314–7.
Yesil M, Bayata S, Postaci N, Arikan E. Progression of conduction system disease in a paced patient with Kearns-Sayre syndrome. Clin Cardiol. 2009;32(6):E65–7.
Gronlund MA, Honarvar AK, Andersson S, et al. Ophthalmological findings in children and young adults with genetically verified mitochondrial disease. Br J Ophthalmol. 2010;94(1):121–7.
Snyder LA, Rismondo V, Miller NR. The Fisher variant of Guillain-Barre syndrome (Fisher syndrome). J Neuroophthalmol. 2009;29(4):312–24.
Hughes RA, Cornblath DR. Guillain-Barre syndrome. Lancet. 2005;366(9497):1653–66.
Hughes RA, Swan AV, van Doorn PA. Corticosteroids for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2010;2:CD001446.
van der Meche FG, van Doorn PA. Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy: immune mechanisms and update on current therapies. Ann Neurol. 1995;37(Suppl 1):S14–31.
Yang YR, Liu SL, Qin ZY, et al. Comparative proteomics analysis of cerebrospinal fluid of patients with Guillain-Barre syndrome. Cell Mol Neurobiol. 2008;28(5):737–44.
Raphael JC, Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2002;2:CD001798.
Hughes RA, Raphael JC, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2006;1:CD002063.
Tan H. Bilateral oculomotor palsy secondary to pseudotumor cerebri. Pediatr Neurol. 2010;42(2):141–2.
Bahmani Kashkouli M, Khalatbari MR, Yahyavi ST, Borghei-Razavi H, Soltan-Sanjari M. Pituitary apoplexy presenting as acute painful isolated unilateral third cranial nerve palsy. Arch Iran Med. 2008;11(4):466–8.
Beckmann YY, Deniz B, Gelal F, Secil Y. Third cranial nerve palsy as the presenting neuro-ophthalmic feature of nasopharyngeal carcinoma. J Neuroophthalmol. 2010;30(1):102–3.
Beleza P, Machado A, Soares-Fernandes J, et al. Isolated oculomotor nerve paresis as the presenting sign of multiple sclerosis. Arq Neuropsiquiatr. 2008;66(2A):254–5.
Brazis PW. Isolated palsies of cranial nerves III, IV, and VI. Semin Neurol. 2009;29(1):14–28.
Akagi T, Miyamoto K, Kashii S, Yoshimura N. Cause and prognosis of neurologically isolated third, fourth, or sixth cranial nerve dysfunction in cases of oculomotor palsy. Jpn J Ophthalmol. 2008;52(1):32–5.
Cullom ME, Savino PJ, Sergott RC, Bosley TM. Relative pupillary sparing third nerve palsies. To arteriogram or not? J Neuroophthalmol. 1995;15(3):136–40; discussion 140–131.
Trobe JD. The evaluation of horner syndrome. J Neuroophthalmol. 2010;30(1):1–2.
Jacobson DM. Pupil involvement in patients with diabetes-associated oculomotor nerve palsy. Arch Ophthalmol. 1998;116(6):723–7.
Jacobson DM, Trobe JD. The emerging role of magnetic resonance angiography in the management of patients with third cranial nerve palsy. Am J Ophthalmol. 1999;128(1):94–6.
Trobe JD. Searching for brain aneurysm in third cranial nerve palsy. J Neuroophthalmol. 2009;29(3):171–3.
Tamhankar MA, Biousse V, Ying GS, et al. Isolated third, fourth, and sixth cranial nerve palsies from presumed microvascular versus other causes: a prospective study. Ophthalmology. 2013;120(11):2264–9.
Wilker SC, Rucker JC, Newman NJ, Biousse V, Tomsak RL. Pain in ischaemic ocular motor cranial nerve palsies. Br J Ophthalmol. 2009;93(12):1657–9.
Cogan DG, Lincoff HA. Unilateral headache and oculomotor paralysis not caused by aneurysm. AMA Arch Ophthalmol. 1957;57(2):181–9.
Landau K. Discovering a dys-covering lid. Surv Ophthalmol. 1997;42(1):87–91.
Carrasco JR, Savino PJ, Bilyk JR. Primary aberrant oculomotor nerve regeneration from a posterior communicating artery aneurysm. Arch Ophthalmol. 2002;120(5):663–5.
Varma R, Miller NR. Primary oculomotor nerve synkinesis caused by an extracavernous intradural aneurysm. Am J Ophthalmol. 1994;118(1):83–7.
Bartleson JD, Trautmann JC, Sundt TM Jr. Minimal oculomotor nerve paresis secondary to unruptured intracranial aneurysm. Arch Neurol. 1986;43(10):1015–20.
Payne JW, Adamkiewicz J Jr. Unilateral internal ophthalmoplegia with intracranial aneurysm. Am J Ophthalmol. 1969;68(2):349–52.
Chaudhary N, Davagnanam I, Ansari SA, Pandey A, Thompson BG, Gemmete JJ. Imaging of intracranial aneurysms causing isolated third cranial nerve palsy. J Neuroophthalmol. 2009;29(3):238–44.
Lee AG, Johnson MC, Policeni BA, Smoker WR. Imaging for neuro-ophthalmic and orbital disease – a review. Clin Exp Ophthalmol. 2009;37(1):30–53.
Lee AG, Hayman LA, Brazis PW. The evaluation of isolated third nerve palsy revisited: an update on the evolving role of magnetic resonance, computed tomography, and catheter angiography. Surv Ophthalmol. 2002;47(2):137–57.
van Rooij WJ, Sprengers ME, de Gast AN, Peluso JP, Sluzewski M. 3D rotational angiography: the new gold standard in the detection of additional intracranial aneurysms. AJNR Am J Neuroradiol. 2008;29(5):976–9.
Mathew MR, Teasdale E, McFadzean RM. Multidetector computed tomographic angiography in isolated third nerve palsy. Ophthalmology. 2008;115(8):1411–5.
White PM, Wardlaw JM, Lindsay KW, Sloss S, Patel DK, Teasdale EM. The non-invasive detection of intracranial aneurysms: are neuroradiologists any better than other observers? Eur Radiol. 2003;13(2):389–96.
Loevner LA, Sonners AI, Schulman BJ, et al. Reinterpretation of cross-sectional images in patients with head and neck cancer in the setting of a multidisciplinary cancer center. AJNR Am J Neuroradiol. 2002;23(10):1622–6.
Elmalem VI, Hudgins PA, Bruce BB, Newman NJ, Biousse V. Underdiagnosis of posterior communicating artery aneurysm in noninvasive brain vascular studies. J Neuroophthalmol. 2011;31(2):103–9.
Beard C. Ptosis. 3rd ed. St. Louis: Mosby; 1981.
Duke-Elder S. System of ophthalmology. London: Kimpton; 1976.
Pratt SG, Beyer CK, Johnson CC. The Marcus Gunn phenomenon. A review of 71 cases. Ophthalmology. 1984;91(1):27–30.
Demirci H, Frueh BR, Nelson CC. Marcus Gunn jaw-winking synkinesis: clinical features and management. Ophthalmology. 2010;117(7):1447–52.
Bowyer JD, Sullivan TJ. Management of Marcus Gunn jaw winking synkinesis. Ophthal Plast Reconstr Surg. 2004;20(2):92–8.
Dillman DB, Anderson RL. Levator myectomy in synkinetic ptosis. Arch Ophthalmol. 1984;102(3):422–3.
Spillane J, Beeson DJ, Kullmann DM. Myasthenia and related disorders of the neuromuscular junction. J Neurol Neurosurg Psychiatry. 2010;81(8):850–7.
Elrod RD, Weinberg DA. Ocular myasthenia gravis. Ophthalmol Clin N Am. 2004;17(3):275–309; v.
Seybold ME. Myasthenia gravis. A clinical and basic science review. JAMA. 1983;250(18):2516–21.
Berrih-Aknin S, Frenkian-Cuvelier M, Eymard B. Diagnostic and clinical classification of autoimmune myasthenia gravis. J Autoimmun. 2014;48–49:143–8.
Punga T, Le Panse R, Andersson M, Truffault F, Berrih-Aknin S, Punga AR. Circulating miRNAs in myasthenia gravis: miR-150-5p as a new potential biomarker. Ann Clin Transl Neurol. 2014;1(1):49–58.
Jayam Trouth A, Dabi A, Solieman N, Kurukumbi M, Kalyanam J. Myasthenia gravis: a review. Autoimmune Dis. 2012;2012:874680.
Grob D, Brunner NG, Namba T. The natural course of myasthenia gravis and effect of therapeutic measures. Ann N Y Acad Sci. 1981;377:652–69.
Zhang X, Yang M, Xu J, et al. Clinical and serological study of myasthenia gravis in HuBei Province, China. J Neurol Neurosurg Psychiatry. 2007;78(4):386–90.
Eymard B, Vernet-der Garabedian B, Berrih-Aknin S, Pannier C, Bach JF, Morel E. Anti-acetylcholine receptor antibodies in neonatal myasthenia gravis: heterogeneity and pathogenic significance. J Autoimmun. 1991;4(2):185–95.
Vincent A, Clover L, Buckley C, Grimley Evans J, Rothwell PM. Evidence of underdiagnosis of myasthenia gravis in older people. J Neurol Neurosurg Psychiatry. 2003;74(8):1105–8.
Sommer N, Sigg B, Melms A, et al. Ocular myasthenia gravis: response to long-term immunosuppressive treatment. J Neurol Neurosurg Psychiatry. 1997;62(2):156–62.
Monsul NT, Patwa HS, Knorr AM, Lesser RL, Goldstein JM. The effect of prednisone on the progression from ocular to generalized myasthenia gravis. J Neurol Sci. 2004;217(2):131–3.
Kupersmith MJ, Latkany R, Homel P. Development of generalized disease at 2 years in patients with ocular myasthenia gravis. Arch Neurol. 2003;60(2):243–8.
Grob D, Brunner N, Namba T, Pagala M. Lifetime course of myasthenia gravis. Muscle Nerve. 2008;37(2):141–9.
Bever CT Jr, Aquino AV, Penn AS, Lovelace RE, Rowland LP. Prognosis of ocular myasthenia. Ann Neurol. 1983;14(5):516–9.
Gorelick PB, Rosenberg M, Pagano RJ. Enhanced ptosis in myasthenia gravis. Arch Neurol. 1981;38(8):531.
Gay AJ, Salmon ML, Windsor CE. Hering’s law, the levators, and their relationship in disease states. Arch Ophthalmol. 1967;77(2):157–60.
Cogan DG. Myasthenia gravis: a review of the disease and a description of lid twitch as a characteristic sign. Arch Ophthalmol. 1965;74:217–21.
Kubis KC, Danesh-Meyer HV, Savino PJ, Sergott RC. The ice test versus the rest test in myasthenia gravis. Ophthalmology. 2000;107(11):1995–8.
Sethi KD, Rivner MH, Swift TR. Ice pack test for myasthenia gravis. Neurology. 1987;37(8):1383–5.
Golnik KC, Pena R, Lee AG, Eggenberger ER. An ice test for the diagnosis of myasthenia gravis. Ophthalmology. 1999;106(7):1282–6.
Fakiri MO, Tavy DL, Hama-Amin AD, Wirtz PW. Accuracy of the ice test in the diagnosis of myasthenia gravis in patients with ptosis. Muscle Nerve. 2013;48(6):902–4.
Chatzistefanou KI, Kouris T, Iliakis E, et al. The ice pack test in the differential diagnosis of myasthenic diplopia. Ophthalmology. 2009;116(11):2236–43.
Seybold ME. The office Tensilon test for ocular myasthenia gravis. Arch Neurol. 1986;43(8):842–3.
Barton JJ, Fouladvand M. Ocular aspects of myasthenia gravis. Semin Neurol. 2000;20(1):7–20.
Vincent A, Newsom-Davis J. Acetylcholine receptor antibody as a diagnostic test for myasthenia gravis: results in 153 validated cases and 2967 diagnostic assays. J Neurol Neurosurg Psychiatry. 1985;48(12):1246–52.
Srinivasan A, Kleinberg TT, Murchison AP, Bilyk JR. Laboratory investigations for diagnosis of autoimmune and inflammatory periocular disease: part I. Ophthal Plast Reconstr Surg. 2016;32(5):321–8.
Bindu PS, Nirmala M, Patil SA, Taly AB. Myasthenia gravis and acetylcholine receptor antibodies: a clinico immunological correlative study on South Indian patients. Ann Indian Acad Neurol. 2008;11(4):242–4.
Fulpius BW, Miskin R, Reich E. Antibodies from myasthenic patients that compete with cholinergic agents for binding to nicotinic receptors. Proc Natl Acad Sci U S A. 1980;77(7):4326–30.
Keesey JC. Clinical evaluation and management of myasthenia gravis. Muscle Nerve. 2004;29(4):484–505.
Vrolix K, Fraussen J, Molenaar PC, et al. The auto-antigen repertoire in myasthenia gravis. Autoimmunity. 2010;43(5–6):380–400.
Berrih-Aknin S, Le Panse R. Myasthenia gravis: a comprehensive review of immune dysregulation and etiological mechanisms. J Autoimmun. 2014;52:90–100.
Hoch W, McConville J, Helms S, Newsom-Davis J, Melms A, Vincent A. Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. Nat Med. 2001;7(3):365–8.
Evoli A, Tonali PA, Padua L, et al. Clinical correlates with anti-MuSK antibodies in generalized seronegative myasthenia gravis. Brain. 2003;126(Pt 10):2304–11.
Leite MI, Strobel P, Jones M, et al. Fewer thymic changes in MuSK antibody-positive than in MuSK antibody-negative MG. Ann Neurol. 2005;57(3):444–8.
Drachman DB. Myasthenia gravis. N Engl J Med. 1994;330(25):1797–810.
Benatar M, Kaminski H. Medical and surgical treatment for ocular myasthenia. Cochrane Database Syst Rev. 2006;2:CD005081.
Seybold ME, Drachman DB. Gradually increasing doses of prednisone in myasthenia gravis. Reducing the hazards of treatment. N Engl J Med. 1974;290(2):81–4.
Wong VA, Beckingsale PS, Oley CA, Sullivan TJ. Management of myogenic ptosis. Ophthalmology. 2002;109(5):1023–31.
Demartelaere SL, Blaydon SM, Shore JW. Tarsal switch levator resection for the treatment of blepharoptosis in patients with poor eye protective mechanisms. Ophthalmology. 2006;113(12):2357–63.
Walton KA, Buono LM. Horner syndrome. Curr Opin Ophthalmol. 2003;14(6):357–63.
Mughal M, Longmuir R. Current pharmacologic testing for Horner syndrome. Curr Neurol Neurosci Rep. 2009;9(5):384–9.
Jacobson DM, Berg R, Grinstead GF, Kruse JR. Duration of positive urine for cocaine metabolite after ophthalmic administration: implications for testing patients with suspected Horner syndrome using ophthalmic cocaine. Am J Ophthalmol. 2001;131(6):742–7.
Dewan MA, Harrison AR, Lee MS. False-negative apraclonidine testing in acute Horner syndrome. Can J Ophthalmol. 2009;44(1):109–10.
Kawasaki A, Borruat FX. False negative apraclonidine test in two patients with Horner syndrome. Klin Monatsbl Augenheilkd. 2008;225(5):520–2.
Bremner F. Apraclonidine is better than cocaine for detection of Horner syndrome. Front Neurol. 2019;10:55.
Almog Y, Gepstein R, Kesler A. Diagnostic value of imaging in horner syndrome in adults. J Neuroophthalmol. 2010;30(1):7–11.
Thakker MM, Huang J, Possin DE, et al. Human orbital sympathetic nerve pathways. Ophthal Plast Reconstr Surg. 2008;24(5):360–6.
Demetriades AM, Miller NR, Garibaldi DC. Bilateral internal carotid artery dissection presenting as isolated unilateral Horner syndrome. Ophthal Plast Reconstr Surg. 2009;25(6):485–6.
Kerty E. The ophthalmology of internal carotid artery dissection. Acta Ophthalmol Scand. 1999;77(4):418–21.
Rigamonti A, Iurlaro S, Reganati P, Zilioli A, Agostoni E. Cluster headache and internal carotid artery dissection: two cases and review of the literature. Headache. 2008;48(3):467–70.
Biousse V, Touboul PJ, D’Anglejan-Chatillon J, Levy C, Schaison M, Bousser MG. Ophthalmologic manifestations of internal carotid artery dissection. Am J Ophthalmol. 1998;126(4):565–77.
Reede DL, Garcon E, Smoker WR, Kardon R. Horner’s syndrome: clinical and radiographic evaluation. Neuroimaging Clin N Am. 2008;18(2):369–85, xi.
Menon R, Kerry S, Norris JW, Markus HS. Treatment of cervical artery dissection: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry. 2008;79(10):1122–7.
Smith SJ, Diehl N, Leavitt JA, Mohney BG. Incidence of pediatric Horner syndrome and the risk of neuroblastoma: a population-based study. Arch Ophthalmol. 2010;128(3):324–9.
Jeffery AR, Ellis FJ, Repka MX, Buncic JR. Pediatric Horner syndrome. J AAPOS. 1998;2(3):159–67.
Mahoney NR, Liu GT, Menacker SJ, Wilson MC, Hogarty MD, Maris JM. Pediatric horner syndrome: etiologies and roles of imaging and urine studies to detect neuroblastoma and other responsible mass lesions. Am J Ophthalmol. 2006;142(4):651–9.
Pirouzian A, Holz HA, Ip KC, Sudesh R. Acquired infantile Horner syndrome and spontaneous internal carotid artery dissection: a case report and review of literature. J AAPOS. 2010;14(2):172–4.
Sobel J. Botulism. Clin Infect Dis. 2005;41(8):1167–73.
Caya JG. Clostridium botulinum and the ophthalmologist: a review of botulism, including biological warfare ramifications of botulinum toxin. Surv Ophthalmol. 2001;46(1):25–34.
Capobianco DJ, Dodick DW. Diagnosis and treatment of cluster headache. Semin Neurol. 2006;26(2):242–59.
Silberstein SD, Lipton RB, Dodick D, Wolff HG. In: Silberstein SD, Lipton RB, Dodick DW, editors. Wolff’s headache and other head pain. 8th ed. Oxford/New York: Oxford University Press; 2008.
DaSilva AF, Goadsby PJ, Borsook D. Cluster headache: a review of neuroimaging findings. Curr Pain Headache Rep. 2007;11(2):131–6.
Pinessi L, Rainero I, Rivoiro C, Rubino E, Gallone S. Genetics of cluster headache: an update. J Headache Pain. 2005;6(4):234–6.
Schurks M, Diener HC. Cluster headache and lifestyle habits. Curr Pain Headache Rep. 2008;12(2):115–21.
May A, Leone M, Afra J, et al. EFNS guidelines on the treatment of cluster headache and other trigeminal-autonomic cephalalgias. Eur J Neurol. 2006;13(10):1066–77.
Hansen SL, Borelli-Moller L, Strange P, Nielsen BM, Olesen J. Ophthalmoplegic migraine: diagnostic criteria, incidence of hospitalization and possible etiology. Acta Neurol Scand. 1990;81(1):54–60.
Levin M, Ward TN. Ophthalmoplegic migraine. Curr Pain Headache Rep. 2004;8(4):306–9.
Headache Classification Committee of the International Headache Society. Classification and diagnostic criteria for headache disorders, cranial neuralgias and facial pain. Cephalalgia. 1988;8(Suppl 7):1–96.
Bek S, Genc G, Demirkaya S, Eroglu E, Odabasi Z. Ophthalmoplegic migraine. Neurologist. 2009;15(3):147–9.
Carlow TJ. Oculomotor ophthalmoplegic migraine: is it really migraine? J Neuroophthalmol. 2002;22(3):215–21.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2021 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Murchison, A.P., Bilyk, J.R., Savino, P.J. (2021). Ptosis in Neurologic Disease. In: Servat, J.J., Black, E.H., Nesi, F.A., Gladstone, G.J., Calvano, C.J. (eds) Smith and Nesi’s Ophthalmic Plastic and Reconstructive Surgery. Springer, Cham. https://doi.org/10.1007/978-3-030-41720-8_13
Download citation
DOI: https://doi.org/10.1007/978-3-030-41720-8_13
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-41719-2
Online ISBN: 978-3-030-41720-8
eBook Packages: MedicineMedicine (R0)