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Show journal of Neuro- Ophthahnology 16( 4): 302- 303, 1996. © 1996 Lippincott- Raven Publishers, Philadelphia Letter to the Editor Predominant Downgaze Ophthalmoparesis in Anti- Hu Encephalomyelitis To the Editor: In a recent article, Crino et al. ( 1) reported the cases of three patients with paraneoplastic brainstem encephalitis and ophthalmoparesis, two of them associated with the presence of anti- Hu antibodies in the serum. We report another case of paraneoplastic anti- Hu- associated encephalopathy with midbrain signs and predominant downgaze paresis. An active 62- year- old woman experienced difficulty in reading and walking downstairs together with daytime sleepiness, episodes of slurred speech, staring, and unresponsiveness, which worsened over several weeks. Her medical and family histories were unremarkable except for a 35- pack-year history of cigarette smoking with no complaints of headaches, double vision, gastrointestinal problems, rheumatologic symptoms, insect bites, diabetes mellitus, or hypertension. When alert and cooperative, she was oriented to time and intermittently to place, but her short- and long- term memory were impaired. She made errors in calculation, and her speech was dysarthric. She was hy-persomnolent, and within moments she would lapse into inattention and mutism, during which time she followed no directions and was unable to stand up or walk. A grasp reflex was present, but startle myoclonus was absent. Upper- extremity tone was mildly increased without axial rigidity; trace left- upper- extremity dysdiadochokinesis was noted, and ankle jerks were absent. Her visual acuity was 20/ 20 for individual letters, but she was unable to read. Her visual fields were intact, and her pupils reacted sluggishly to light but without relative afferent defects. Fixation was interrupted by square- wave jerks. No vertical OKN could be elicited, and horizontal OKN was poorly formed. On attempted downgaze, the patient moved her chin downward, and paradoxically rolled her eyes up. On attempted upgaze, the palpebral fissure widened, with an occasional beat of convergence retraction nystagmus. No other nystagmus or rhythmic movements of the mouth or palate were observed. Horizontal pursuit was jerky with slowed hypo-metric saccades. Bilateral ptosis was present, which did not improve with rest. Blood tests, including a complete blood count with smear, chemistries, coagulation profile, TFTs, acetylcholine receptor antibody, antinuclear antibody, WESR, VDRL, and Lyme titer, showed normal results. Magnetic resonance imaging and angiography, transesophageal echocardiography, carotid duplex scan, cerebral angiography, pelvic/ abdominal computed tomography ( CT), and jejunal biopsy also gave normal results. Repeated spinal taps showed elevated white cells ( 9- 20/ mm3) with normal glucose and protein levels and negative cytology. A 24- h continuous EEG showed results that had no correlation with behavioral disturbances, and during an Amytal interview the patient was more organized, without lapses in contact but with no improvement in memory, calculation, speech, or eye movements. A temporal lobe biopsy showed no evidence of Creutzfeld- Jacob disease, lymphoma or periodic- acid- Schiff- positive macrophages. A brief therapeutic trial of steroids yielded no improvement. A 2- week course of intravenous ceftriaxone as empiric treatment for Whipple's disease was not effective. A screen for paraneoplastic antibodies in the patient's cerebrospinal fluid ( CSF) and serum found high titers of anti- Hu antibodies. Subsequent CT scan of the chest showed two enlarged mediastinal lymph nodes, which on CT- guided biopsy proved to be invaded by small- cell carcinoma of the lung. Anti- Hu antibodies in CSF or serum are diagnostic of a paraneoplastic syndrome most frequently associated with small- cell carcinoma of the lung ( 2,3). Women are affected more commonly than men. Often the diagnosis of malignancy is not made until after the onset of neurologic symptoms. Multifocal neurologic symptoms are found in 75% of anti- Hu- positive patients, resulting in sensory neuropathy, limbic encephalitis, cerebellar degeneration, motor neuropathy, autonomic dysfunction, visual loss, and brain- stem encephalitis. Paraneoplastic brain- stem encephalitis may mimic a variety of syndromes, including focal lesions of the midbrain, pons, or medulla. The differential diagnosis in this case consisted of Whipple's disease, primary central nervous system lymphoma, Creutzfeldt- Jakob LETTER TO THE EDITOR 303 disease, and progressive supranuclear palsy. The patient's clinical features of vertical gaze paresis with absent downgaze, poor fixation, and hypomet-ric horizontal saccades were suggestive of progressive supranuclear palsy; however, the lack of axial rigidity together with the subacute temporal course of the symptoms was atypical. While many patients with anti- Hu syndrome have had eye movement abnormalities reflecting midbrain pathology, predominance of downgaze paresis has not previously been described. Nicholas D. Schiff, M. D. David F. Moore, M. D., Ph. D. Jacqueline M. S. Winterkorn, M. D., Ph. D. Departments of Neurology and Ophthalmology Cornell University Medical College New York, NY 10021 References 1. Crino PB, Galetta SL, Sater RA, et al. Clinicopathologic study of paraneoplastic brainstem encephalitis and ophthalmo-paresis. / Neuro- Ophthalmol 1996; 16: 44- 8. 2. Dalmau J, Graus F, Rosenblum M, Posner J. Anti- Hu associated paraneoplastic encephalomyelitis and sensory neuropathy: a clinical study of 71 patients. Medicine 1992; 71: 59- 72. 3. Posner JB. Neurologic complications of cancer. Philadelphia: F. A. Davis, 1995: 368- 75. / Neuro- Ophthalmol Vol. 16, No. 4, 1996 |
