Journal of Neuro-Ophthalmology, December 2004, Volume 24, Issue 4

Letters to the Editor

LETTERS TO THE EDITOR Intracranial Hypertension Associated With Transverse Myelitis To the Editor: Raised intracranial pressure has been reported rarely in demyelinating central nervous system disease. We re­cently encountered a case of transverse myelitis with simul­taneous intracranial hypertension. This association of monophasic spinal cord demyelination and concurrently raised intracranial pressure has not, to our knowledge, been reported previously. A 25- year old woman presented with a 12- week his­tory of paresthesias in both hands, upper trunk dysesthesias, Lhermitte phenomenon, postural headaches, and visual obscurations. She had no pertinent history and did not use any drugs. Examination showed a suspended sensory level be­tween C3 and T12. Tone and power were normal. Reflexes were brisk but equal in the four limbs and plantar responses were flexor. Cranial nerve, cerebellar, and sphincter func­tions were unimpaired. Visual acuity was normal but Hum­phrey visual fields showed enlarged blind spots. Fundos-copy revealed bilateral papilledema with several hemorrhages. Her weight was 94 kg with a body mass index of36. Magnetic resonance imaging and venography of the brain were normal. Magnetic resonance imaging of the cer­vical cord showed a longitudinal hyperintensity on sagittal T2- weighted images from C3 to C4, suggestive of a demy­elinating plaque ( Fig. 1). The lumbar puncture opening pressure was 440 mm of water and cerebrospinal fluid ex­amination showed 45 lymphocytes/ uL, a protein of 70 mg/ dL, and a glucose of 2.5 mmol/ L ( serum glucose of 4.0 mmol/ L). Oligoclonal bands were present. Blood count, in­flammatory markers, electrolytes, renal and liver functions, thyroid function, angiotensin- converting enzyme were nor­mal. Serologies for syphilis, herpes, Epstein- Barr, cyto­megalovirus, and Borrelia burgdorferi were negative, as were antinuclear antibodies. Chest x- ray was normal. No corticosteroid treatment was administered and the patient made a complete recovery from the myelitis within 6 months. She was treated with acetazolamide 500 mg/ d and her headaches, visual symptoms, and optic disc edema disappeared within 3 months. In a follow- up of over 3 years, no new clinical findings have appeared. Central nervous system demyelination with raised in­tracranial pressure has been described very rarely ( 1- 4). Some authors ( 1- 3) have speculated that raised intracranial pressure is related to mechanical obstruction of cerebro spi­nal fluid flow by plaques. In an article published in Russian, Skoromets et al in 1991 ( 4) suggested that intrathecal im­munoglobulin synthesis results in osmotic and oncotic cerebrospinal fluid pressure changes, thereby disturbing the blood- brain barrier and elevating intracranial pressure. Contrasting with the scarcity of English language reports, this article surprisingly reported raised intracranial pressure ( 220- 380 mm of water) in 40 patients during an exacerba­tion of multiple sclerosis. All patients were said to be symp­tomatic from the raised pressure, with headaches, nausea, vomiting, and confusion. The appearance of the optic discs was not described. In a later report, Newman et al ( 5) proposed that the association of intracranial hypertension with auto- immune conditions such as systemic lupus erythematosus ( 6) sug­gests immune complex deposition within the arachnoid villi as a potential mechanism. FIG. 1. Sagittal T2- weighted magnetic resonance imaging shows longitudinal hyperintensity at C3- C4 indicative of demyelination. 344 JNeuro- Ophthalmol, Vol. 24, No. 4, 2004 Letters to the Editor JNeuro- Ophthalmol, Vol. 24, No. 4, 2004 A coincidental association is possible, of course, be­cause our patient was a young and obese woman. Our case is distinctive in that she did not, unlike the three patients of Newman et al ( 5), have clinically- definite multiple sclero­sis, but instead had a monophasic illness with imaging ab­normalities limited to the spinal cord and no other manifes­tations 5 years after onset. Such an association has not, to our knowledge, previously been described. Yusuf A. Rajabally, MD Ian F. Pye, MA, MD, FRCP Department of Neurology University Hospitals of Leicester Royal Infirmary Leicester, United Kingdom yusuf. rajabally@ ulil- tr. nhs. uk REFERENCES 1. Butler EG, Gilligan BS. Obstructive hydrocephalus caused by mul­tiple sclerosis. Clin Exp Neurol 1989; 26: 219- 23. 2. Kepes JJ. Large focal tumour- like demyelinating lesions of the brain: intermediate entity between multiple sclerosis and acute dis­seminated encephalomyelitis ? Ann Neurol 1993; 33: 18- 27. 3. David P, Uncini A, Scoppetta C. Intracranial hypertension syn­drome as initial symptom of late onset multiple sclerosis. A case report. Acta Neurol ( Napoli) 1983; 5: 402^ I. 4. Skoromets AA, Ermolenko IN, Barbas IM, et al. [ Efferent methods of the treatment of cerebrospinal fluid hypertension in exacerbation of multiple sclerosis]. Zh Nevropatol Psikhiatr Im S S Korsakova 1991; 91: 23- 6. 5. Newman NJ, Selzer KA, Bell RA. Association of multiple sclerosis and intracranial hypertension. JNeuroophthalmol 1994; 14: 189- 92. 6. Horoshovski D, Amital H, Katz M, et al. Pseudotumor cerebri in SLE. Clin Rheumatol 1995; 14: 708- 10. Persistent Visual Loss from Neurotrophic Corneal Ulceration After Dorsolateral Medullary Infarction ( Wallenberg Syndrome) To the Editor: Neurotrophic keratopathy is a complication of fifth cranial nerve or ganglion dysfunction ( 1). Decreased cor­neal sensitivity can also occur from interruption of the spi­nal fifth nerve complex ( 2). However, few reports have de­scribed ulcerative keratitis complicating loss of corneal sensation associated with lesions of the medulla such as sy­ringomyelia or infarction ( 3,4). We recently encountered a patient in whom this complication developed after a dorso­lateral medullary infarction ( Wallenberg syndrome). A 48- year- old hypertensive, hyperlipidemic man had the abrupt onset of vertigo, imbalance, right facial numb­ness, and weakness of his left arm and leg. The next day, diffusion- weighted and fluid- attenuated inversion recovery magnetic resonance ( MR) imaging ( Fig. 1) revealed infarc­tion of the right dorsolateral medulla. MR angiography showed normal carotid and vertebral arteries. After physi­cal rehabilitation, he was able to walk with a cane, but he had persistently reduced pain and temperature sensation of the right side of his face that was not in an onion- skin pat­tern. He had no lagophthalmos or facial motor dysfunction. Two months after the acute stroke, he had painless inflammation OD with a corneal epithelial defect, stromal edema and infiltration, and a hypopyon ( Fig. 2). He had no sensation of the right cornea to a cotton wisp but could de­tect the gentlest setting of the Cochet- Bonnet esthesiometer applied to the left cornea. Corneal scrapings yielded Pro­teus mirabilis, Enterobacter cloacae, and Staphylococcus epidermidis. After topical therapy with ceftazidime and to­bramycin, a continuous- wear soft contact lens was fit with frequent use of preservative- free lubricants. Because of a persistent corneal epithelial defect, a lateral tarsorrhaphy was later performed. His ocular surface gradually healed, but vision remained counting fingers because of a residual corneal opacity. We are unaware of previous reports of this complica­tion after medullary infarction. Preventive strategies may be needed to avert vision- limiting complications and FIG. 1. Axial fluid- attenuated inversion recovery magnetic resonance imaging showing region of hyperintensity in the right dorsolateral medulla. 345 Letters to the Editor JNeuro- Ophthalmol, Vol. 24, No. 4, 2004 A coincidental association is possible, of course, be­cause our patient was a young and obese woman. Our case is distinctive in that she did not, unlike the three patients of Newman et al ( 5), have clinically- definite multiple sclero­sis, but instead had a monophasic illness with imaging ab­normalities limited to the spinal cord and no other manifes­tations 5 years after onset. Such an association has not, to our knowledge, previously been described. Yusuf A. Rajabally, MD Ian F. Pye, MA, MD, FRCP Department of Neurology University Hospitals of Leicester Royal Infirmary Leicester, United Kingdom yusuf. rajabally@ ulil- tr. nhs. uk REFERENCES 1. Butler EG, Gilligan BS. Obstructive hydrocephalus caused by mul­tiple sclerosis. Clin Exp Neurol 1989; 26: 219- 23. 2. Kepes JJ. Large focal tumour- like demyelinating lesions of the brain: intermediate entity between multiple sclerosis and acute dis­seminated encephalomyelitis ? Ann Neurol 1993; 33: 18- 27. 3. David P, Uncini A, Scoppetta C. Intracranial hypertension syn­drome as initial symptom of late onset multiple sclerosis. A case report. Acta Neurol ( Napoli) 1983; 5: 402^ I. 4. Skoromets AA, Ermolenko IN, Barbas IM, et al. [ Efferent methods of the treatment of cerebrospinal fluid hypertension in exacerbation of multiple sclerosis]. Zh Nevropatol Psikhiatr Im S S Korsakova 1991; 91: 23- 6. 5. Newman NJ, Selzer KA, Bell RA. Association of multiple sclerosis and intracranial hypertension. JNeuroophthalmol 1994; 14: 189- 92. 6. Horoshovski D, Amital H, Katz M, et al. Pseudotumor cerebri in SLE. Clin Rheumatol 1995; 14: 708- 10. Persistent Visual Loss from Neurotrophic Corneal Ulceration After Dorsolateral Medullary Infarction ( Wallenberg Syndrome) To the Editor: Neurotrophic keratopathy is a complication of fifth cranial nerve or ganglion dysfunction ( 1). Decreased cor­neal sensitivity can also occur from interruption of the spi­nal fifth nerve complex ( 2). However, few reports have de­scribed ulcerative keratitis complicating loss of corneal sensation associated with lesions of the medulla such as sy­ringomyelia or infarction ( 3,4). We recently encountered a patient in whom this complication developed after a dorso­lateral medullary infarction ( Wallenberg syndrome). A 48- year- old hypertensive, hyperlipidemic man had the abrupt onset of vertigo, imbalance, right facial numb­ness, and weakness of his left arm and leg. The next day, diffusion- weighted and fluid- attenuated inversion recovery magnetic resonance ( MR) imaging ( Fig. 1) revealed infarc­tion of the right dorsolateral medulla. MR angiography showed normal carotid and vertebral arteries. After physi­cal rehabilitation, he was able to walk with a cane, but he had persistently reduced pain and temperature sensation of the right side of his face that was not in an onion- skin pat­tern. He had no lagophthalmos or facial motor dysfunction. Two months after the acute stroke, he had painless inflammation OD with a corneal epithelial defect, stromal edema and infiltration, and a hypopyon ( Fig. 2). He had no sensation of the right cornea to a cotton wisp but could de­tect the gentlest setting of the Cochet- Bonnet esthesiometer applied to the left cornea. Corneal scrapings yielded Pro­teus mirabilis, Enterobacter cloacae, and Staphylococcus epidermidis. After topical therapy with ceftazidime and to­bramycin, a continuous- wear soft contact lens was fit with frequent use of preservative- free lubricants. Because of a persistent corneal epithelial defect, a lateral tarsorrhaphy was later performed. His ocular surface gradually healed, but vision remained counting fingers because of a residual corneal opacity. We are unaware of previous reports of this complica­tion after medullary infarction. Preventive strategies may be needed to avert vision- limiting complications and FIG. 1. Axial fluid- attenuated inversion recovery magnetic resonance imaging showing region of hyperintensity in the right dorsolateral medulla. 345 JNeuro- Ophthalmol, Vol. 24, No. 4, 2004 Letters to the Editor FIG. 2. Epithelial defect of the lower two- thirds of the right cornea associated with loss of corneal sensation. superinfection of neurotrophic keratopathy in patients who lose corneal sensation in this circumstance. W. Michael Hipps, MD Kirk R. Wilhelmus, MD, PhD Cullen Eye Institute Department of Ophthalmology Baylor College of Medicine Houston, Texas kirkw@ bcm. tmc. edu REFERENCES 1. Pushker N, Dada T, Vajpayee RB, et al. Neurotrophic keratopathy. CLAO J 2001; 21: 100- 1. 2. Ongerboer de Visser BW. Comparative study of corneal and blink reflex latencies in patients with segmental or with cerebral lesions. Adv Neurol 1983; 39: 757- 72. 3. Amalric P, Bessou P, Vergnes H. Syringomyelic et perforation corneenne bilaterale par keratite neurotrophique. Rev Otoneurooph-talmol 1964; 36: 62- 4. 4. Schimmelpfennig B, Baumgartner A. Einige Manifestationen moglicher trophischer Veranderungen des Corneaepithels nach her-abgesetzter Trigeminusfunktion. Klin Monatsbl Augenheilkd 1988; 192: 149- 53. Isolated Granulomatous Uveitis Presenting Twenty- Two Years before Multiple Sclerosis To the Editor: An association exists between granulomatous uveitis and multiple sclerosis ( MS) ( 1- 4). Rarely does the uveitis antedate the other manifestations of MS. We examined a patient who had presented with chronic isolated granuloma­tous anterior uveitis 22 years before she demonstrated the first clinical manifestations of MS, which was confirmed at autopsy 4 years later. Bilateral, granulomatous anterior uveitis and anterior vitreous inflammation developed in a 36- year- old woman in 1975. Angiotensin- converting enzyme, lysozyme, puri­fied protein derivative, syphilis serologies, and chest ro­entgenogram were normal. Gallium scan demonstrated increased uptake in the mediastinum, lacrimal, and parotid glands. Sarcoidosis was presumptively diagnosed. Her uveitis became chronic with intermittent exacerbation of inflammation. In 1997, a spastic right hemiparesis, bladder dysfunc­tion, and gait disturbance developed. Her symptoms remit­ted over the next 2 months. In 2001 she awoke with binocu­lar, horizontal diplopia. She denied headache, blurred vision, dysarthria, or vertigo. Visual acuities were 20/ 100 OD and 20/ 60 OS. Pupils were irregular and poorly reac­tive. Neither eye adducted fully. She had a slow spastic gait, a mild right hemiparesis, pathologically brisk reflexes, and a right extensor plantar response. Cerebrospinal fluid revealed a mild lymphocytic pleocytosis and a markedly elevated cerebrospinal fluid im­munoglobulin G index, immunoglobulin G- to- albumin ra­tio, and immunoglobulin G synthesis rate. Brain magnetic resonance imaging showed multiple ovoid periventricular, subcortical, and infratentorial white matter hyperintensities on T2 and fluid- attenuated inversion recovery sequences ( Fig. 1). Midbrain lesions involved the periaqueductal re­gion and both medial longitudinal fasciculi. Several lesions demonstrated enhancement. Nine months later, she died of urosepsis. At autopsy, multiple microscopic foci of chronic demyelination were present throughout the neuroaxis ( Fig. 1). Uveitis in MS patients usually appears as an interme­diate uveitis sometimes associated with retinal periphlebitis ( 1- 4). Several reports, however, describe patients with clinical MS and granulomatous anterior uveitis, character­ized by large, mutton fat keratic precipitates. Biousse et al ( 2) reported uveitis in 11 patients occurring up to 2 years before the onset of neurologic symptoms of MS. Among six MS patients with chronic granulomatous anterior uveitis re­ported by Acar et al ( 3), the uveitis preceded neurologic symptoms in two patients by 4 and 13 years. However, un­like our case, there was no pathologic confirmation of MS in any of these previously reported patients with uveitis. Therefore, it is conceivable that these patients had neurosarcoidosis rather than MS. The neuroimaging, clini­cal course, and spinal fluid analysis of neurosarcoidosis and MS can be indistinguishable. Each disease can demonstrate 346 © 2004 Lippincott Williams & Wilkins Letters to the Editor JNeuro- Ophthalmol, Vol. 24, No. 4, 2004 FIG. 1. Magnetic resonance fluid- attenuated inversion re­covery imaging shows areas of signal hyperintensity in ce­rebral periventricular white matter ( A) and periaqueductal region in midbrain ( C) that correspond to demyelinated plaques ( arrows) by histologic autopsy examination ( B, D, Luxol fast blue, hematoxylin and eosin). Many of the lesions in the subventricular white matter are partially myelinated shadow plaques ( B, single arrows), suggestive of remyelina-tion. Images B and D were obtained by scanning stained glass slides at 4000- pixel per inch resolution using an ArtixScan 4000tf scanner ( Microtek, Redondo Beach, CA), followed by conversion to grayscale and contrast adjust­ment using Photoshop ( Adobe Systems, San Jose, CA). ovoid periventricular T2 and fluid- attenuated inversion re­covery signal hyperintensities that may enhance. The cere­brospinal fluid analysis in both conditions may reveal intra­thecal immunoglobulin synthesis and oligoclonal bands ( 5,6). There is one report of a patient with autopsy- proven MS and histopathologic evidence of granulomatous uveitis ( 4). However, the authors do not discuss the temporal relationship of the clinical onset of uveitis and neurolo­gic symptoms. Gregory P. Hanes, MD, Susan M. Staugaitis, MD, PhD, David M. Meisler, MD, Michael S. Lee, MD Departments of Neurology, Ophthalmology, and Anatomic Pathology Cleveland Clinic Foundation Cleveland, Ohio leem4@ ccf. org REFERENCES 1. Bachman DM, Rosenthal AR, Beckingsale AB. Granulomatous uveitis in neurological disease. Br J Ophthalmol 1985; 69: 192- 6. 2. Biousse V, Trichet C, Bloch- Michel E, et al. Multiple sclerosis as­sociated with uveitis in two large clinic- based series Neurology 1999; 52: 179- 81. 3. Acar MA, Birch MK, Abbott R, et al. Chronic granulomatous ante­rior uveitis associated with multiple sclerosis. Graefe's Arch Clin Exp Ophthalmolol 1993; 231: 166- 8. 4. Arnold AC, Pepose JS, Hepler RS, et al. Retinal periphlebitis and retinitis in multiple sclerosis. I. Pathologic characteristics. Ophthal­mology 1984; 91: 255- 62. 5. Borucki SJ, Nguyen BV, Ladoulis CT, et al. Cerebrospinal fluid immunoglobulin abnormalities in neurosarcoidosis. Arch Neurol 1989; 46: 270- 3. 6. Smith AS, Meisler DM, Weinstein MA, et al. High signal periven­tricular lesions in patients with sarcoidosis: Neurosarcoidosis or multiple sclerosis? AJR Am J Roentgenol 1989; 153: 147- 52. 347