Progressive supranuclear palsy (PSP) is an example of a condition in which there is no effective treatment for the underlying disease process but in which symptomatic measures can be of some benefit. Because PSP usually affects those in midlife and later, most are wearing bifocals, which do not serve them well as downgaze is progressively limited (see Chapter 9, Chronic progressive external ophthalmoplegia vs. progressive supranuclear palsy). While not a “high-tech” intervention, simply providing separate reading glasses can be extremely helpful for these patients. Because vergence is also impaired, the addition of base-in prism to these reading glasses is often beneficial. An appropriate amount of base-out prism may also be added to distance spectacles to compensate for poor divergence in this condition. Artificial tears can be helpful for the decreased blink rate in PSP as in other parkinsonian syndromes. Providing separate reading glasses is similarly appropriate for any patient with impaired downgaze due to other neurologic disorders.

Most forms of nystagmus and other forms of ocular instability are intractable, but some may show a positive response to medication. Most notably, ocular neuromyotonia typically responds dramatically to carbamazepine and periodic alternating nystagmus often does very well with baclofen. Downbeat nystagmus may dampen with clonazepam but excessive sedation often limits the dose. Certain forms of episodic ataxia with nystagmus respond to acetazolamide. In addition to efforts at pharmacologic treatment, some forms of nystagmus damp with convergence, and in such cases bilateral baseout prism may help stabilize vision by stimulating convergence. In cases with an eccentric null zone (in which nystagmus is absent or decreased with a marked head turn or with chin up or down), bilateral horizontal or vertical prisms or eye muscle surgery can be similarly effective. For intractable cases, botulinum toxin injection into the muscle cone has been used to damp involuntary ocular oscillations by temporarily paralyzing the eye muscles. The downside of such treatment is the resultant loss of the vestibulo-ocular reflex, and the

Chapter 12: Management misadventures 205

need to patch the fellow eye to avoid diplopia. Large recessions of extraocular muscles have also been employed.

Certain persistent pupillary disorders, while not technically “curable”, can be improved with symptomatic measures. The ptosis and miosis of an oculosympathetic palsy (Horner syndrome) can often be reversed with topical application of apraclonidine or dilute phenylephrine. An enlarged pupil that constricts poorly to light may cause photophobia that can be relieved with a contact lens that has a small clear opening. When due to postganglionic parasympathetic denervation (an Adie’s tonic pupil) dilute pilocarpine can provide similar symptomatic relief.

FURTHER READING

Idiopathic intracranial hypertension

G. Bynke, G. Zemack, H. Bynke, B. Romner, Ventriculoperitoneal shunting for idiopathic intracranial hypertension. Neurology, 63 (2004), 1314–16.

K.B. Digre, J. J. Corbett, Idiopathic intracranial hypertension (pseudotumor cerebri): a reappraisal. Neurologist,

7 (2001), 2–67.

H.J. Sugarman, W. F. Felton III, J. B. Salvant Jr., A. Sismanis, J. M. Kellum, Effects of surgically induced weight loss on idiopathic intracranial hypertension in morbid obesity. Neurology, 45 (1995), 1655–9.

M. Thambisetty, P. F. Lavin, N. J. Newman, V. Biousse, Fulminant idiopathic intracranial hypertension. Neurology, 68 (2007), 229–32.

Giant cell arteritis

S. C. Carroll, B. J. Gaskin, H. V. Danesh-Meyer, Giant cell arteritis. Clin Exp Ophthalmol, 334 (2006), 159–73.

J.K. Hall, L. J. Balcer, Giant cell arteritis. Curr Treat Opt Neurol, 6 (2004), 45–53.

S.S. Hayreh, B. Zimmerman, Management of giant cell arteritis. Ophthalmologica, 217 (2003), 239–59.

Non-arteritic anterior ischemic optic neuropathy

A.C. Arnold, L. A. Levin, Treatment of ischemic optic neuropathy. Semin Ophthalmol, 17 (2002), 39–46.

206Chapter 12: Management misadventures

S.Connolly, K. Gordon, J. Horton, Salvage of vision after hypotension-induced ischemic optic neuropathy. Am J

Ophthalmol, 117 (1994), 235–42.

S.S. Hayreh, B. Zimmerman, P. Podhajsky, W. Alward, Nocturnal arterial hypertension and its role in optic nerve

head and ocular ischemic disorders. Am J Ophthalmol, 117 (1994), 603–24.

K. Landau, J. M. Winterkorn, L. U. Mailloux, W. Vetter, B. Napolitano, 24-hour blood pressure monitoring in patients with anterior ischemic optic neuropathy. Arch Ophthalmol, 114 (1996), 570–5.

The Ischemic Optic Neuropathy Decompression Trial Research Group, Optic nerve decompression surgery for nonarteritic anterior ischemic optic neuropathy (NAION) is not effective and may be harmful. JAMA, 273 (1995), 625–32.

Optic neuritis

A.Arnold, Evolving management of optic neuritis and multiple sclerosis. Am J Ophthalmol, 139 (2005), 1101–8.

R.W. Beck, P. A. Clear, J. D. Trobe et al., The effect of corticosteroids for acute optic neuritis on the subsequent development of multiple sclerosis. The Optic Neuritis Study Group. N Engl J Med, 329 (1993), 1764–9.

S.A. Morrow, C. A. Stoian, J. Dmitrovic, S. C. Chan, L. M. Metz, The bioavailability of IV methylprednisolone and

oral prednisone in multiple sclerosis. Neurology, 63 (2004), 1079–80.

J.D. Trobe, P. C. Sieving, K. E. Guire, A. M. Fendrick, The impact of the optic neuritis treatment trial on the practices of ophthalmologists and neurologists. Ophthalmology, 106 (1999), 2047–53.

Ocular myasthenia

P. S. Chavis, D. E. Stickler, A. Walker, Immunosuppression or surgical treatment for ocular myasthenia gravis. Arch Neurol, 64 (2007), 1792–4.

A. Evoli, A. P. Batocchi, C. Minisci, C. DiSchino, P. Tonali, Therapeutic options in ocular myasthenia gravis. Neuromusc Dis, 11 (2001), 208–16.

M. E. Gilbert, E. A. DeSousa, P. J. Savino, Ocular myasthenia gravis treatment. The case against prednisone therapy and thymectomy. Arch Neurol, 64 (2007), 1790–2.

M. Kupersmith, G. Ying, Ocular motor dysfunction and ptosis in ocular myasthenia gravis: effects of treatment. Br J Ophthalmol, 89 (2005), 1330–4.

L.L. Kusner, A. Puwanant, H. Kaminski, Ocular myasthenia. Diagnosis, treatment and pathogenesis. Neurologist, 12

(2006), 231–9.

N. T. Monsul, H. S. Patwa, A. M. Knowrr, R. L. Lesser, J. M. Goldstein, The effect of prednisone on the progression from ocular to generalized myasthenia gravis. J Neurol Sci, 217 (2004), 131–3.

Nystagmus

R.J. Leigh, R. L. Tomsak, Drug treatments for eye movement disorders. J Neurol Neurosurg, 74 (2003), 1–4.

R. McLean, F. Proudlock, S. Thomas, C. Degg, I. Gottlob, Congenital nystagmus: randomized, controlled, doublemasked trial of memantine/gabapentin. Ann Neurol, 61 (2007), 130–8.