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Show IOllm"f (If ClilliCfll NI'lIn)-ol'''''",flllol~y 6(2): 1J6-1l9, 1986. Metastatic Adenocarcinoma Presenting as a One-and-a-Half Syndrome Clinical, Radiological, and Pathological Correlations Roy A. Jackel, M.D., John W. Gittinger, Jr., M.D., Thomas W. Smith, M.D., and Cynthia B. Passarelli, M.D. © 1986 Raven Press, New York The one-and-a-half syndrome, produced by a lesion in the tegmentum at the level of the pons, rarely has been reported as the result of a solitary metastasis. We report a case of adenocarcinoma of the lung metastatic to the pontine tegmentum with clinical, radiological, and pathological confirmation of the anatomy of this syndrome. From the Departments of Neurology (R.A.J.), Surgery (Ophthalmology) (J.W.G.), and Pathology (Neuropathology) (T. W.S.), St. Vincent Hospital and University of Massachusetts Medical School, and Fallon Clinic (C.B.P.), Worcester, Massachusetts. Address correspondence and reprint requests to Roy A. Jackel, M.D., The Medical College of Pennsylvania, Department of Neurology, 3300 Henry Avenue, Philadelphia, PA 19129. This case was presented before the 17th Annual Frank B. Walsh Society Meeting. February 22-23. 1985, Baltimore. Maryland. Paralysis of conjugate gaze and an internuclear ophthalmoplegia as the result of a lesion in the brainstem tegmentum at the level of the pons are known as the one-and-a-half syndrome (1). Its pathophysiology has been reviewed recently (2,3). Brainstem infarction and hemorrhage and multiple sclerosis are common etiologies (2). We report here the fourth case of a one-and-a-half syndrome from metastatic disease, only the second with pathological confirmation. CASE REPORT A 75-year-old white male complained of double vision beginning 10 months prior to his death. He had a history of hypertension, glaucoma, diabetes mellitus, and angina. An episode of hemoptysis was attributed to bronchitis, and an atypical mycobacterium (Mycobacterium intracellularis) was cultured, but not treated. In March 1984, he experienced oblique diplopia when looking to the right, and a diagnosis of a partial left third nerve palsy was considered. He was hospitalized in April for a workup of this condition that included a negative unenhanced and enhanced computed tomographic (CT) scan of the head, normal brainstem auditory-evoked potentials, and a negative edrophonium test. Chest roentgenogram showed a reticular- nodular pattern with biapical pleural thickening. He was discharged and initially had mild improvement of his diplopia, which then worsened, particularly on right lateral gaze. The patient denied other complaints, with the exception of brief, intermittent, sharp pains on the right side of his head, a productive cough, and a 10-lb. weight loss. He was admitted in June 1984, at which time his examination revealed a left abducens nerve palsy JJ6 ONE-AND-A-HALF SYNDROME Jl7 as well as right-beating nystagmus on right lateral gaze. A left peripheral facial palsy also was noted. Work-up during that admission consisted of a normal lumbar puncture, including cytology and AFB stains and cultures. A CT scan was interpreted as showing no definite abnormalities. Westergren erythrocyte sedimentation rate was 82 mrn/h (nl < 15), with a negative antinuclear antibody. A left temporal artery biopsy was negative, but the patient was given a trial of steroids without improvement. He was readmitted 1 month later with progression of his left facial palsy, such that he was unable to close his left eye and was drooling from the left side of his mouth. In addition to diplopia with right lateral gaze, he now complained of diplopia on left lateral gaze. Examination revealed paresis of conjugate gaze to the left and a left internuclear ophthalmoplegia. There was coarse nystagmus of the abducting right eye. Convergence was intact. The findings pointed to a lesion at the level of the facial coUiculus on the left also affecting the facial nerve at its genu. CT scan of the head, after injection of metrizamide into the subarachnoid space, revealed asymmetry of the fourth ventricle with a fullness at the level of the left pons. Review of the earlier CT scans revealed a small enhancing lesion just anterior to the fourth ventricle on the left (Fig. 1). In view of his abnormal chest roentgenogram, a skin test was applied for tuberculosis that was positive to 21 mm. Because of the possibility of a brainstem tuberculoma, bronchoscopy with washings and biopsy was performed. No evidence of tuberculosis was found, and the patient was taken to open-lung biopsy, which revealed nonspecific fibrosis and chronic inflammation without evidence of tuberculosis or tumor. He was started on isoniazid. A neoplasm was considered, either a pontine glioma or metastatic lesion, but no other evidence for tumor could be found. The patient was given a course of radiation, a total of 4,000 rad over 20 treatments. Over the ensuing 4 months, the patient's pulmonary status gradually deteriorated without significant change in neurological findings. He died in November 1984. PATHOLOGIC FINDINGS General Autopsy A moderately differentiated adenocarcinoma was present in the periphery of the upper lobe of the right lung. Carcinomatosis of the lungs with FIG. 1. Enhanced 5 mm CT scan at the level of the facial colliculus. demonstrating lesion subjacent to the fourth ventricle. fibrosis, as well as metastases to the hilar and mediastinal lymph nodes, was found. Small metastases were present in the pericardium, myocardium, serosal surface of the stomach, and peripancreatic lymph nodes. Other findings included bilateral pleural adhesions, pulmonary emphysema, moderate generalized atherosclerosis, and mild fatty change in the liver. Neuropathology The brain weighed 1,460 g. Coronal sections of the cerebral hemispheres showed a small solitary focus of metastatic adenocarcinoma within the cortex of the medial left frontal lobe (Fig. 2). The brainstem sections revealed a small necrotic lesion within the left superior medial tegmentum of the lower pons, immediately subadjacent to the fourth ventricle (Fig. 3). The lesion was partially cavitated and consisted histologically of fibrillary gliosis, fibrosis particularly around blood vessels, focal deposition of fibrinoid material, and scattered macrophages (postirradiation changes). Occasional bizarre- appearing multinucleated cells were present, compatible with metastatic tumor cells. This lesion completely destroyed the left abducens nucleus and part of the paramedian pontine reticular formation. 1Clin Neuro-ophthallllol, Vol. 6, No.2, 1986 //8 R. A. JACKEL ET AL. FIG. 2. Section of left frontal cortex showing metastatic adenocarcinoma. (Hematoxylin and eosin stain. x 64.) Sections of the rostral pons and midbrain showed degenerative changes (vacuolization, macrophage infiltration) in the left mediallongitudinal fasciculus; mild degeneration was also present in the right medial longitudinal fasciculus. The pontine sections at and just caudal to the necrotic lesion showed secondary degeneration of the left abducens and left facial nerves. Some brainstem and spinal cord sections showed clumps of tumor cells within the subarachnoid space. COMMENT In this patient, the one-and-a-half syndrome was the first manifestation of an occult neoplasm. CT scans confirmed the presence of a lesion in the tegmentum at the level of the facial colliculus. Autopsy disclosed a metastasis destroying the abducens nucleus and involving the paramedian pontine reticular formation and the medial longitudinal fasciculus. Brainstem infarction and hemorrhage, multiple sclerosis, arteriovenous malformation-other reported etiologies of the one-and-a-half syndrome -did not fit the clinical picture in our patient. The differential lay between neoplasm-primary or metastatic-and infection. Tuberculoma of the brainstem has been reported recently by several authors (4-6). Boghen et al. (4) described a patient who was found eventually to have multiple cerebral tuberculomas, including one in the pons resulting in facial myokymia and a spastic, paretic facial contracture. Monteiro and Coppeto (5) reported a case of gaze palsy caused by a pontine tuberculoma, whereas I 0", :Vcun"0l'hthallllol. Vol. 6. No.2. 1986 AG. 3. Sequential transverse sections of lower pons. showing necrotic lesion in left superior-medial tegmentum subjacent to fourth ventricle. Inocencio and Ballecer (6) presented a case of bilateral internuclear ophthalmoplegia with loss of convergence secondary to a midbrain tuberculoma. Our patient had a history of a bronchoscopy isolating an atypical mycobacterium with positive skin testing and an abnormal chest roentgenogram. With repeat bronchoscopy and open-lung biopsy being negative and the original culture revealing only an atypical mycobacterium, this possibility was considered less likely, but a course of isoniazid was given. We were led to treatment on the basis of a presumed diagnosis of neoplasm. Weiss and Richardson (7) stated that while gliomas tend to infiltrate slowly into the brainstem and metastases grow in an expansile fashion, the assertion that gliomas produce a slower neurologic deterioration does not aid in the differential. There are no reliable ONE-AND-A-HALF SYNDROME 119 clinical criteria to distinguish between a brainstem metastasis and a glioma (7-13), but brainstem gliomas are rare over the age of 50 (12). In approximately 10% of patients with central nervous system metastases, the metastasis is the first clinical evidence of the neoplasm (14,15). Adenocarcinoma of the lung characteristically has a slow growth rate with a high metastatic potential, and is frequently the primary tumor in these cases (16). Brainstem involvement from tumors with multiple central nervous system metastases is present in approximately 8% of cases (7,9). Solitary brainstem metastases, however, are less common, e.g., only five out of Weiss and Richardson's 574 cases of central nervous system metastases (7). Wall and Wray (2) reviewed 29 cases of oneand- a-half syndrome in the literature, as well as 20 cases of their own, including examples of primary tumors such as pontine gliomas, cerebellar astrocytomas, and ependymomas of the fourth ventricle. In addition, Gonyea and Collins (17) reported a case of one-and-a-half syndrome with facial nerve involvement as a result of primary reticulum cell sarcoma of the nervous system. Wall and Wray (2) noted only one case as the result of metastatic disease, Le., that of PierrotDeseilligny et al. (3). Two additional cases (those of Meienberg et al. (18) and Spector and Stark (19)], although not described as such, have oneand- a-half syndrome secondary to brainstem metastases. Only Meienberg's case (18) had pathologic correlation: the tumor destroyed the right abducens nucleus, right facial nucleus and nerve, the right medial longitudinal fasciculus, the caudal part of the paramedian pontine reticular formation, and the right upper medulla-extending over the midline, damaging the left medial longitudinal fasciculus and the rootlets of the left abducens nerve. We report a case of adenocarcinoma with a pontine metastasis, only the fourth reported case of metastatic disease presenting as a one-and-a-half syndrome. Our case confirms clinically, radiologically, and pathologically the anatomy of this syndrome. Acknowledgment: The authors thank Dr. M. Weiner, Department of Radiology, and Dr. E. Marcus, Depart-ment of Neurology, St. Vincent Hospital, Worcester, Massachusetts, for their help and cooperation; Dr. D. 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