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Show LETTERS TO THE EDITOR Bilateral Optic Neuritis After Dengue Viral Infection We report a case of optic neuritis after dengue viral infection, a phenomenon not previously reported. A 20- year- old man was referred to Ramathibodi Hospital in February 2001 with a history of bilateral visual loss. Seven days before, the patient had presented to another hospital with a 3- day history of high fever and vomiting. Physical examination revealed high temperature, minimal cervical lymphadenopathy, hepatomegaly, and a positive tourniquet test. Laboratory examination revealed thrombocytopenia ( platelet count 60,000/ mm3). Viral infection with dengue hemorrhagic fever was suspected as the likely diagnosis. Two days later, the fever started to subside and the platelet count decreased to 30,000/ mm3. He was treated conservatively with intravenous fluids, electrolytes, and glucose. No clinical evidence of hypovolemic shock occurred in this patient. Two days after the fever had subsided, the patient experienced gradual loss of vision in his right and OD. Physical examination at our hospital revealed a well- developed man without signs of acute distress. Temperature, blood pressure, and pulse rate were normal. The liver was palpable and a positive convalescent rash on extremities was found. Visual acuity was counting fingers OU. Pupils measured 3 mm in dim illumination and reacted normally to light. Extraocular motility was normal without pain. Ophthalmoscopy revealed mild bilateral optic disc hyperemia with a flame- shaped hemorrhage at the fovea OD. Goldmann visual field tests demonstrated bilateral cecocentral scotomas ( Fig. 1). Retinal fluorescein angiography demonstrated no disc leakage. Laboratory results included hemoglobin 15.3 g/ dL, white blood count 6,310/ mm3 ( 30% polymorphonuclear cells, 52% lymphocytes, 14% monocytes, and 2% atypical lymphocytes), platelets 238,000/ mm3, and elevated liver enzymes ( glutamic- oxaloacetic transaminase 402 U/ L, glutamic- pyruvic transaminase 529 U/ L, 7- glutamyltransfer-ase 280 U/ L). Coagulation studies were normal. Serologic analyses for syphilis ( venereal disease research laboratory and fluorescent treponemal antibody absorption test) were negative. Lumbar puncture revealed clear and colorless cerebrospinal fluid with normal glucose, protein, cell count, and differential. A brain and orbit magnetic resonance imaging study demonstrated enhancement of both optic nerves. The diagnosis of dengue infection was confirmed after IgM and IgG antibodies for dengue virus serotype I were detected in the serum. The patient was treated with intravenous methyl-prednisolone 250 mg four times per day for 3 days, FIG. 1. Humphrey visual fields ( gray scale, top; pattern deviation, bottom) at presentation show cecocentral scotomas OU. followed by prednisolone 60 mg/ d slowly tapered over 4 weeks. Visual acuity improved gradually, being 20/ 200 OD and 10/ 200 OS after 1 month, and returning to 20/ 20 OU after 8 months. Over 18 months of follow- up, he has had no further ocular or neurologic symptoms. Visual acuity after 26 months remains 20/ 20 OU and color vision testing with Ishihara plates is normal. Dengue or dengue hemorrhagic fever is a mosquito-transmitted viral disease that is endemic mainly in Southeast Asia and the Western Pacific region. The ocular manifestations include photophobia, retrobulbar pain, conjunctival congestion, subconjunctival hemorrhage, retinal vessel engorgement, and accommodative weakness ( 1,2). Retinal hemorrhages and maculopathy are rare but have been reported to cause visual loss ( 3). Optic nerve ischemia can occur in survivors of dengue hemorrhagic fever after severe hypotension and circulatory failure ( 4). To our knowledge, this is the first reported case of bilateral optic neuritis associated with a dengue viral infection. As in many parainfectious cases with optic neuritis, profound loss of visual function occurred with nearly complete recovery. Pisit Preechawat, MD Anuchit Poonyathalang, MD Department of Ophthalmology Ramathibodi Hospital Mahidol University Bangkok, Thailand E- mail: akepisit@ hotmail. com J Neuro- Ophthalmol, Vol. 25, No. 1, 2005 51 J Neuro- Ophthalmol, Vol. 25, No. 1, 2005 Letters to the Editor REFERENCES Beck RW. Optic neuritis. In: Miller NR, Newman NJ, eds. Walsh & Hoyt's Clinical Neuro- Ophthalmology, 5th ed. Baltimore: Williams & Wilkins, 1999: 628- 9. Gill WD. Ocular symptoms in dengue based on analysis of 1241 cases. Arch Ophthalmol. 1928; 57: 628- 38. Haritoglou C, Scholz F, Bialasiewicz A, et al. Ocular manifestation in dengue fever. Ophthalmologe. 2000; 97: 433- 6. Hendarto SK, Hadinegoro SR. Dengue encephalopathy. Acta Paediatr Jpn. 1992; 34: 350- 7. One- and- a- Half Syndrome With Facial Diplegia: The 15V2 Syndrome? We recently examined a 67- year- old man who demonstrated findings consistent with a unilateral one- and-a- half syndrome and a bilateral lower motor neuron facial palsy ( IVi + 7 + 7 = \ 5Vi). The patient had the sudden onset of dizziness, nausea, and blurring of vision. He smoked heavily and had a history of diabetes mellitus. Examination disclosed a complete right horizontal conjugate gaze palsy and a right adduction deficit. Oculocephalic stimulation did not improve the defective eye movements. Convergence was relatively spared. Vertical ocular movements and alignment were normal and ptosis was absent. The pupils were equal in size and normally reactive to light and a near stimulus. The remainder of the neurologic examination was normal. One day later, a bilateral lower motor neuron facial palsy abruptly developed, including bilateral flattening of the nasolabial fold and absent forehead wrinkling. Brain computed tomography was normal. FLAIR and T2- weighted brain magnetic resonance imaging disclosed high signal in the lower pontine tegmentum, findings considered typical of stroke ( Fig. 1). A facial nerve conduction and blink reflex study confirmed a bilateral lower motor neuron facial palsy. When last examined 2 months after the onset of symptoms, the patient showed only a minimal deficit in right horizontal gaze. Horizontal gaze palsy in one- and- a- half syndrome may result from lesions in the abducens nucleus, in the ab-ducens nerve and the medial longitudinal fasciculus, or in the pontine paramedian reticular formation ( PPRF) ( 1,2). Isolated PPRF lesions are associated with retained vestibulo-ocular movement. If the vestibulo- ocular movement is abolished, the lesion lies rostral or caudal to the PPRF ( 5,6). Because our patient showed limitation of oculocephalic abduction toward the right side, we presume that the lesion lies in the abducens nucleus or fascicle rather than in the PPRF ( 2- 7). More rapid resolution of ipsilateral adduction compared with abduction favors involvement of the abducens fascicle rather than the nucleus. An alternative hypothesis is that these findings reflect the relative vulnerability of abducens motor neurons innervating the lateral rectus ( 2). The one- and- a- half syndrome is rarely an isolated finding ( 7), being usually associated with cranial nerve palsies ( facial in 75%), hemiplegia ( 30%), or hemihypesthesia ( 35%) ( 3,4). A lower motor neuron facial nerve palsy is the most commonly associated finding, resulting from involvement of the genu of the intraaxial fasciculus of the facial nerve in the pontine tegmentum ( 2,4). Although the FIG. 1. Axial FLAIR ( A), axial T2- weighted ( B), sagittal T2- weighted ( C), and diffusion- weighted ( D) magnetic resonance images show increased signal { arrow) consistent with ischemic stroke in the paramedian dorsal pontine tegmentum bilaterally. 52 2005 Lippincott Williams & Wilkins Letters to the Editor J Neuro- Ophthalmol, Vol. 25, No. 1, 2005 FIG. 2. Schematic axial section through the caudal pons. The shaded area represents the site of the lesion that involves the right abducens nucleus, both medial longitudinal fasciculi, and both seventh ( facial) nerve fasciculi. combination of a one- a- a- half syndrome and an ipsilateral seventh nerve palsy has been described (" eight- and- a- half syndrome" ( 2)), we are unaware of a previous description of a one- and- a- half syndrome with bilateral seventh nerve palsies, as our patient had. We attribute these findings to a bilateral tegmental pontine lesion involving the right abducens nucleus or fasciculus, bilateral or right medial longitudinal fasciculus, and bilateral intraaxial fasciculi of the facial nerve ( Fig. 2). The histopathologic studies of Fisher ( 8) showing terminal bifurcation of a single paramedian artery to supply the dorsal pontine tegmentum bilaterally could explain why occlusion of a single vessel would produce these findings. Jong Seok Bae, MD Hong Ki Song, MD Department of Neurology Seoul Medical Center and College of Medicine, Hallym University Seoul, Korea E- mail: hksong@ hallym. or. kr REFERENCES 1. Fisher CM. Some neuro- ophthalmological observations. J Neurol Neurosurg Psychiatry. 1967; 30: 383- 92. 2. Eggenberger E. Eight- and- a- half syndrome: one- and- a- half syndrome plus cranial nerve VII palsy. JNeuroophthalmol. 1998; 18: 114- 6. 3. de Seze J, Lucas C, Leclerc X, et al. One- and- a- half syndrome in pontine infarcts: MRI correlates. Neuroradiology. 1999; 41: 666- 9. 4. Wall M, Wray SH. The one- and- a- half syndrome- a unilateral disorder of the pontine tegmentum: a study of 20 cases and review of the literature. Neurology. 1983; 33: 971- 80. 5. Bogousslavsky J, Miklossy J, Regli J, et al. One- and- a- half syndrome in ischemic locked- in state. J Neurol Neurosurg Psychiatry. 1984; 47: 927- 35. 6. Johnston JL, Sharpe JA. Sparing of the vestibuo- ocular reflex with lesions of the paramedian pontine reticular formation. Neurology. 1989; 39: 876. 7. Pierrot- Deseilligny C. Circuits oculomoteurs centraux. Rev Neurol. 1985; 141: 349- 70. 8. Fisher CM. Neuroanatomic evidence to explain why bilateral internuclear ophthalmoplegia may result from occlusion of a unilateral pontine branch artery. J Neuroophthalmol. 2004; 24: 39- 41. Unilateral Involuntary Eyelid Closure Induced by Diplopia That Did Not Remit With Contact Lens Occlusion We report an unusual case of unilateral blepharospasm secondary to diplopia that did not remit even with contact lens occlusion. A 50- year- old woman had diplopia on right gaze for 3 years. Examination revealed a right face turn, a complete right abduction deficit, and a partial deficit of right adduction and supraduction. The eyelids and pupils were normal. Magnetic resonance imaging revealed a right intracavernous meningioma. During follow- up, diminished supraduction of the OD and synkinetic elevation of the lid on adduction and infraduction developed, as well as absence of right incyclotorsion on attempted downward gaze, attributed to aberrant regeneration of a palsied third J Neuro- Ophthalmol, Vol. 25, No. 1, 2005 Letters to the Editor FIG. 1. Right third cranial nerve palsy. The patient's lids have been elevated and she has been asked to direct her gaze up, down, left, and right ( see arrows). There is limitation principally of supraduction and abduction of the OD. cranial nerve ( Fig. 1). The face- turn increased and involuntary unilateral closure of the OD lids developed that was attributed to a desire to eliminate diplopia ( Fig. 2). Botulinum toxin injected into the right medial rectus failed to relieve primary position diplopia, so a right occlusive contact lens was fitted. Despite relief of the diplopia, the involuntary OD lid closure persisted. When the occlusive contact lens was removed and she occluded her OS, the OD lids opened briefly, but she experienced vertigo. A right upper lid crutch in conjunction with the right occlusive lens was unsuccessful. She was finally treated with borulinum toxin injection into the right orbicularis muscle. With the occluding contact lens in place in the OD, the OD lids remained open. Several triggers of blepharospasm have been reported. These include bright lights, air, and cold ( 1). Elston ( 2) found that 57% of 272 blepharospasm patients had ophthalmological symptoms at the onset, mostly irritation and photophobia, but there was no evidence of ocular disease on examination. Jankovic and Orman ( 3) reported the lack of associated ocular signs in patients with blepharospasm who described ocular symptoms. Ocular irritation as a result of lagophthalmos from Bell's palsy is thought also to be a precipitating factor in those predisposed to blepharospasm ( 4). Precipitating triggers have been noted in other dystonias, such as local hand injury in those with writer's cramp ( 2). It is possible that diplopia- activated brain stem mechanisms subserving unilateral eye closure in our patient resulted in involuntary eye closure that persisted despite contact lens occlusion and eventually required borulinum toxin treatment. FIG. 2. Involuntary blepharospasm, right. The left panel shows the patient at rest with involuntary closure of the OD lids. This occurred when she was awake and also persisted when the OD was occluded with a contact lens. The right panel shows that she could, with effort, open her OD for short periods. There is moderate ptosis caused by partial weakness of the levator palpebrae superioris. 54 © 2005 Lippincott Williams & Wilkins Letters to the Editor J Neuro- Ophthalmol, Vol. 25, No. 1, 2005 Eugene Tay MSc, MRCSEd, MRCOphth Gordon T. Plant MA, MD, FRCP Moorfields Eye Hospital and Royal National Hospital for Neurology and Neurosurgery London, England E- mail: dr_ eugenetay@ yahoo. com REFERENCES 1. Jankovic J, Havins WE, Wilkins RB. Blinking and blepharospasm. Mechanism, diagnosis, and management. JAMA. 1982; 248: 316( Mk 2. Elston JS, Marsden CD, Grandas F, Quin NP. The significance of ophthalmological symptoms in idiopathic blepharospasm. Eye. 1988; 2: 435- 9. 3. Jankovic J, Orman J. Blepharospasm: demograohic and clinical survey of 520 patients. Ann Ophthalmol. 1984; 16: 371- 6. 4. Miwa H, Kondo T, Mizuno Y. Bell's palsy induced blepharospasm. J Neurol. 2002; 249: 452^ k Progressive External Ophthalmoplegia With Hereditary Sensory Neuropathy: A Rare Association We describe a patient with chronic progressive external ophthalmoplegia and hereditary sensory neuropathy, an association not previously reported. A 7- year- old girl presented with drooping of both eyelids and inability to look up since age 4 years. There was no history of trauma or swelling of eyelids. From age 3 years, the child had had recurrent ulcers on her fingertips, toes, and the dorsum of her hands and feet without pain. No family member had a similar problem. The child was of below- average intelligence. We noted auto- amputation of the great and small toes, mutilated right nail beds on the other toes, multiple scar marks on the dorsum of both feet, mild pes- cavus on the right, and mutilated nail beds and multiple scar marks on the dorsal surfaces of both hands ( Fig. 1). We also found distal weakness of both upper and lower extremities, absent knee and ankle jerks bilaterally, and a decrease in all modalities of sensation, greatest distally Cranial nerve examination was normal. There were no hypopigmented anesthetic skin patches or thickened nerves. Visual acuity was finger counting OU. Visual fields were normal bilaterally. She had mild ptosis with good levator function bilaterally. Ocular movements were markedly reduced in upgaze and downgaze but full in horizontal gaze with nystagmus in extreme lateral gaze ( Fig. 2). An alter- FIC. 1. The dorsum of hands and feet show auto-amputation of fingertips and toes, mutilation, and multiple scar marks. nating 20- prism- diopter exotropia was present without diplopia. Corneal sensation was mildly decreased. Pupils were normal in size and shape and reacted briskly to light. Ultrasound of both orbits was normal. The neostigmine test was negative. Complete blood profile, erythrocyte sedimentation rate, serum protein electrophoresis, thyroid profile, and brain magnetic resonance imaging were normal. A nerve conduction study showed absent sensory conduction in median, ulnar, and sural nerves and decreased motor conduction in median, ulnar, and common peroneal nerves. These findings favored a diagnosis of hereditary motor sensory neuropathy type 1. No other siblings or parents had this condition. However, the parents were consanguineous. In a 1980 study of 228 patients of hereditary sensory neuropathy, Harding et al ( 1) did not find a single case of external ophthalmoplegia. Pramod Kumar Sahu, MD, FRCS ( Edin) V. A. Rao, MD Renuka Srinivasan, MS S. Thanikachalam, MD Department of Ophthalmology, Jawaharlal Institute of Postgraduate Medical Education & Research Center Pondicherry, India E- mail: pujapramod@ hotmail. com 55 J Neuro- Ophthalmol, Vol. 25, No. 1, 2005 Letters to the Editor FIG. 2. Mild bilateral ptosis, reduced upgaze and downgaze, and nearly normal horizontal gaze. REFERENCES 1. Harding AE, Thomas PK. The clinical features of hereditary motor and sensory neuropathy Types I and II. Brain. 1980; 103: 259- 80. See- Saw Nystagmus as the Presenting Sign in Multiple Sclerosis Although see- saw nystagmus has been reported in multiple sclerosis ( MS) ( 1), there is no previous report of a patient presenting with this finding. A 3 3- year- old woman reported a 2- day history of oscillopsia. She had no other neurologic or systemic symptoms. On examination, the eyes were aligned with full ductions. The patient had characteristic pendular seesaw nystagmus that increased on left gaze. The velocity of the nystagmus was equal in both directions. The OD ascended and intorted, whereas the OS descended and extorted; then the OD descended and extorted, whereas the OS ascended and intorted. Otherwise, the ophthalmologic and neurologic examination was unremarkable. Total blood count, erythrocyte sedimentation rate, glucose, urea, electrolytes, and a collagen vascular disease profile were normal. Lumbar puncture revealed a normal FIG. 1. A. Axial T2- weighted magnetic resonance imaging shows linear high signal in the white matter adjacent to the body of the left lateral ventricle ( arrow). B. Coronal FLAIR magnetic resonance imaging shows high signal in the deep white matter of the left frontal lobe ( arrow). 56 © 2005 Lippincott Williams & Wilkins Letters to the Editor J Neuro- Ophthalmol, Vol. 25, No. 1, 2005 opening pressure and cerebrospinal fluid formula, but isoelectric focusing showed three positive IgG oligoclonal bands; none was detected from a simultaneous serum sample. Brain magnetic resonance imaging showed a linear area of signal change in white matter adjacent to the body of left lateral ventricle ( Fig. 1A) and in the deep white matter of the left frontal lobe ( Fig. IB). The combination of clinical, cerebrospinal fluid, and imaging findings suggested a diagnosis of multiple sclerosis. The exact mechanism of see- saw nystagmus is still not known. Its frequent association with chiasmal and parasellar lesions initially led to speculation that see- saw nystagmus was related to a disturbance in visual input. Recent evidence suggests that see- saw nystagmus is caused by dysfunction of the rostral brainstem ( 2). A disturbance in the interstitial nucleus of Cajal, a small collection of neurons located adjacent to medial longitudinal fasciculus in the midbrain tegmentum, is often implicated ( 2). Soupramanien Sandramouli, FRCS, FRCOphth Hani T. Benamer, MRCP, PhD Mark Mantle, MRCP, FRCR Randhir Chavan, MS Wolverhampton and Midland Counties Eye Infirmary and Departments of Neurology and Radiology New Cross Hospital Wolverhampton, England E- mail: Samouli@ aol. com REFERENCES 1. Samkoff LM, Smith CR. See- saw nystagmus in a patient with clinically definite MS. Eur Neurol. 1994; 34: 228- 9. 2. Miller NR. Nystagmus and related ocular motility disorders. In: Miller NR, ed. Walsh and Hoyt's Clinical Neuro- Ophthalmology, 4th ed. Baltimore: Williams & Wilkins, 1988: 907- 8. 57 |