prednisone was unsuccessful.9 In view of our patient's excellent re¬ sponse to treatment, we believe that the diagnosis of infiltrative polyneu¬ ropathy should be aggressively pur¬ sued in patients with hématologie ma- lignancy and clinically significant pe¬ ripheral neuropathy of unknown cause. Because this process can be patchy, and because negative sural nerve biopsies have been reported,214 we believe that biopsy of a clinically involved sensory nerve is advisable. Empiric treatment for recurrent ma¬ lignancy may be justified in some cases. References 1. Barron KD, Rowland LP, Zimmerman HM. Neuropathy with malignant tumor metastases. J Nerv Ment Dis. 1960;131:10-31. 2. Borit A, Altrocchi PH. Recurrent polyneuropathy and neurolymphomatosis. Arch Neurol. 1971;24:40-49. 3. Gherardi R, Gaulard P, Prost C, et al. T-cell lymphoma revealed by a peripheral neuropathy. Cancer. 1986;58:2710-2716. 4. Henson RA, Urich H. Diffuse infiltration by lymphoma and leukemia. In: Henson RA, Urich H, eds. Cancer and the Nervous System. Boston, Mass: Blackwell Scientific Publications Inc; 1982: 227-267. 5. Hubert D, Gepner P, Baudrimont M, et al. Neuropathies periph\l=e`\riquesspecifiques au cours des lymphomes non-Hodgkiens: \l=a`\propos de deux observations. Semin Hop Paris. 1984;60:2797\x=req-\ 2801. 6. Ince PG, Shaw PJ, Fawcett PR, Bates D. De\x=req-\ myelinating neuropathy due to primary IgM kapa B cell lymphoma of peripheral nerve. Neurology. 1987;37:1231-1235. 7. Kohot H. Unusual involvement of the nervous system in generalization lymphoblastoma. J Nerv Ment Dis. 1946;103:9-120. 8. Moore RY, Oda Y. Malignant lymphoma with diffuse involvement of the peripheral nervous system. Neurology. 1962;12:186-192. 9. Shoenfeld Y, Aderka D, Saudbank V, Gadot N, Santo M, Pinkhas J. Fatal neurolymphomato- sis after remission of histiocytic lymphoma. Neu- rology. 1983;33:243-245. 10. Sumi S, Farrell D, Knauss T. Lymphoma and leukemia manifested by steroid-responsive polyneuropathy. Arch Neurol. 1983;40:577-582. 11. Zuber M, Gherardi R, Imbert M, Garland P, Kuentz M, Poirier J. Peripheral neuropathy with distal nerve infiltration revealing a diffuse pleomorphic malignant lymphoma. J Neurol. 1987; 235:61-62. 12. Alajouanine T, Thurel R, Castaigue P, Lhermitte F. Leucemie aligue avec syndrome polynervitique et infiltration leucosique des nerfs. Rev Neurol. 1949;81:249-261. 13. Harris W. A case of leukemic polyneuritis. Lancet. 1921;1:122. 14. Krendel D, Albright R, Graham D. Infiltrative polyneuropathy due to acute monoblastic leukemia in hematologic remission. Neurology. 1987;37:474-477. 15. Kuroda Y, Nakata H, Kakigi R, Oda K, Shibasaki H, Nakashiro H. Human neurolymphomatosis by adult T-cell leukemia. Neurology. 1989;38:144-146. 16. Klimo P, Conners JM. MACOP-B chemotherapy for the treatment of diffuse large-cell lymphoma. Ann Intern Med. 1985;102:596-602. 17. Sweet DL, Golomb HM, Ultmann JE, et al. Cyclophosphamide, vincristine, methotrexate with leucovorin rescue, and cytarabine (COMLA): combination sequential chemotherapy for advanced diffuse histiocytic lymphoma. Ann Intern Med. 1980;92:785-790. 18. Jellinger K, Radaszkiewiez T. Involvement of the central nervous system in malignant lymphomas. Virchows Arch A. 1976;370:345-362. 19. Olsson Y. Topographic differences in the vascular permeability of the peripheral nervous system. Acta Neuropathol. 1968;10:26-33. 20. Jacobs SA, Bleyer WA, Chabner BA, Johns DG. Altered plasma pharmacokinetics of methotrexate administered intrathecally. Lancet. 1975;1:465-466. 21. Skarin AT, Zuckerman KS, Pitman SW, et al. High-dose methotrexate with folinic acid in the treatment of advanced non-Hodgkin's lymphoma including CNS involvement. Blood. 1977; 50:1039-1047. 22. Canellos GP, Skarin AT, Rosenthal DS, Moloney WL, Frei E. Methotrexate as a single agent and in combination chemotherapy for treatment of non-Hodgkin's lymphoma of unfavorable histology. Cancer Treat Rep. 1981;65 (suppl 1):125-129. 23. Ho DHW, Frei E III. Clinical pharmacology of 1-B-D-arabinofuranosyl cytosine. Clin Pharmacol Ther. 1971;12:944-954. Homer's Syndrome From Hypothalamic Infarction Christopher P. Austin, MD, Simmons Lessell, MD \s=b\ We report a case of Horner's syndrome due to ipsilateral posterior hypothalamic infarction, occurring in the absence of other signs of hypothalamic dysfunction. Associated symptoms of contralateral faciobrachial weakness and dysarthria correlated with the extension of the infarct into the posterior limb of the internal capsule seen by magnetic resonance imaging. The likely vascular anatomy of this lesion is discussed. (Arch Neurol. 1991;48:332-334) Accepted for publication July 20, 1990. From the Departments of Neurology (Dr Austin) and Ophthalmology (Dr Lessell), Harvard Medical School, Massachusetts General Hospital and Massachusetts Eye and Ear Infirmary, Bos- ton. Reprint requests to Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA 02114 (Dr Lessell). -J orner's syndrome can result from interruption of the sympathetic pathway at any point from the hypo¬ thalamus to the orbit. The central neuron is involved in as many as 63% of cases,13 most of them at the level of the brain stem; few are caused by lesions that implicate the sympathetic cell bodies in the hypothalamus. We are reporting a case of oculosympathetic paresis from focal, ipsilateral, posterior hypothalamic infarction. REPORT OF A CASE A 62-year-old woman with hypertension, diabetes mellitus, and coronary artery dis¬ ease presented with left-sided occipital headache, left-sided ptosis, and right-sided face and arm weakness of 8 hours' duration. On the day before admission, she was Downloaded From: http://archneur.jamanetwork.com/ by a University of Calgary User on 05/27/2015 mildly dysarthric. She was "sleepy" on the day of admission, and, at 6 pm, her left up¬ per eyelid drooped and she complained of left-sided occipital headache. Examination 8 hours later revealed an obese woman with a regular pulse rate of 63 beats per minute, a blood pressure of 135/90 mm Hg, and a crescendo-decrescendo systolic cardiac murmur of grade 3/6. Her mental status was normal, and there was no aphasia. The right pupil measured 4.0 mm and the left 2.0 mm. There was dilatation lag of the left pupil that accentuated the anisocoria when the lights were dimmed, but pupillary re¬ actions were otherwise normal. She had left-sided ptosis, elevation of the left lower eyelid, and decreased sweating over the left side of the forehead. There was slight lin¬ gual dysarthria, mild right-sided upper mo¬ tor neuron facial weakness, right pronator drift, and right arm weakness. Computed tomographic scan of the head, performed 120 msec) demonstrating Magnetic resonance images in the sagittal (left) and axial (right) planes (TR 2000 msec; TE an area of high signal intensity consistent with infarction in the left posterior hypothalamus (arrows). = without contrast, demonstrated a small cavitated infarction in the left corona radi¬ ata, lateral to the head of the caudate nucleus. The laboratory test results were as follows: blood glucose level, 19 mmol/L; fasting cholesterol level, 8.25 mmol/L; and triglycéride level, 4.89 mmol/L. An electro¬ cardiogram showed evidence of an old anteroseptal myocardial infarction, left ven¬ tricular hypertrophy with strain, and firstdegree atrioventricular block. The patient was admitted with a provi¬ sional diagnosis of carotid artery dissec¬ tion, but arteriography gave unremarkable results; a persistent fetal pattern was not present. Neuro-ophthalmologic examina¬ tion the next day showed normal visual acuity, color vision, and anterior segments. Applanation intraocular pressure was 18 mm Hg bilaterally. The eyelids and pupils were as on admission. Both pupils dilated after instillation of hydroxyamphetamine; however, the next day, neither pupil dilated after 4% cocaine was instilled. Magnetic resonance imaging of the brain showed an 8 X 10-mm lesion in the left pos¬ terior hypothalamus, extending into the genu and posterior limb of the internal capsule. The lesion was slightly hypointense on ,-weighted images and hyperin¬ tense on T2-weighted images, consistent with recent infarction (Figure). The only other abnormality was the left frontal co¬ rona radiata lesion seen on computed to¬ mographic scan. There were no lesions in the brain stem. There were no other signs of hypothalamic dysfunction, and thyroid function and prolactin levels were normal. The patient was discharged, remained on a regimen of aspirin, and was asymptom¬ atic thereafter. On examination 4 weeks later, her pupils were unremarkable, there was a slight narrowing of the left palpebrai fissure, but no face or arm weakness. COMMENT Pharmacological tests localized this patient's lesion to the central or preganglionic sympathetic neuron. Al¬ though neither pupil dilated with co¬ caine, the clinical signs of Homer's syndrome were unequivocal and present only on the left pupil; we can¬ not explain why cocaine failed to dilate the right pupil. Most cases of hypothalamic Horner's syndrome are caused by large diencephalic tumors,4 hemorrhages,5 or, less commonly, infarction.6·7 In these cases, localization to the hypo¬ thalamus is obvious from symptoms and signs that reflect involvement of the thalamus or other adjacent areas. Isolated hypothalamic infarction is an uncommon event owing to the rich vascular supply of this region, eg, an¬ teromedial arteries arising from the anterior cerebral artery and supplying the anterior hypothalamus, posteromedial (thalamostriate) perforators arising from the proximal portion of the posterior cerebral artery at the bi¬ furcation of the basilar artery and supplying the posterior hypothalamus, and short hypothalamic arteries off the posterior communicating artery.8 In the case of a persistent fetal circu¬ lation pattern, which occurs in up to 30% of individuals,9 the blood supply to the hypothalamus can derive solely from the carotid system, and, thus, be more liable to embolie infarction. The patient's symptoms of faciobrachial weakness, dysarthria, and Downloaded From: http://archneur.jamanetwork.com/ by a University of Calgary User on 05/27/2015 = drowsiness provide clues to localiza¬ tion of this lesion. Bogousslavsky and Regli10 reported a case of faciobrachial paresis and dysarthria as an imcom¬ plete "psuedo-opercular" syndrome from a small lenticulocapsular he¬ matoma and emphasized that the genu of the internal capsule was the critical site for lesions that produced this clin¬ ical picture. Rascol et al" reported a' case, similar to others reported by Fisher,12 of dysarthria and transitory confusion without paresis, which was attributed to a lesion in the genu of the internal capsule. Infarctions in the distribution of the anterior choroidal artery, which frequently involve the genu and posterior limb, also often cause dysarthria and lethargy.1314 Our patient's magnetic resonance imaging showed that the hypothalamic lesion responsible for the central Horner's syndrome extended into the genu and posterior limb of the internal capsule. Thus, the symptoms and signs in our case appear to be explainable by in¬ volvement of the posterior hypothala¬ mus, as well as the overlying genu and rostral segment of the posterior limb of the internal capsule. Occlusion of a hypothalamic branch of the posterior cerebral or posterior communicating artery is the likeliest cause of the in¬ farction, probably from an embolus of cardiac origin to the posterior circula¬ tion. Although lacunar infarcts of the hypothalamus are exceedingly rare (C. M. Fisher, MD, oral communication), given the patient's history of chronic hypertension and the presence of a small infarction in the frontal white matter, the hypothalamic lesion may 4. Tori D, Schisano G, Liljequist B. Primary tumors of the region of the thalamus. J Neuro- 1. Giles CL, Henderson JW. Horner's syndrome: an analysis of 216 cases. Am J Opthalmol. surg. 1961;18:730-740. 5. Crill WE. Horner's syndrome secondary to deep cerebral lesions. Neurology. 1966;16:325. 6. Schiffter R, Reinhart K. The telodiencephalic ischemic syndrome. J Neurol. 1980;222:265-274. 7. Stone WM, deToledo J, Romanul FCA. Horner's syndrome due to hypothalamic infarc- 1958;46:289-296. 2. Grimson BS, Thompson HS. Drug testing in Horner's syndrome. In: Glaser JS, Smith JL, eds. Neuro-Ophthalmology. St Louis, Mo: CV Mosby Co; 1975;8. 721-724. 9. Fisher CM. The circle of Willis: anatomical also have been lacunar. References 3. Keane JR. Oculosympathetic paresis: analysis of 100 hospitalized patients. Arch Neurol. 1979;36:13-15. tion. Arch Neurol. 1986;43:199-200. 8. Carpenter MB, Sutin J. Human Neuroanatomy. Baltimore, Md: Wililams & Wilkins; 1983; variations. Vase Dis. 1965;2:99-105. 10. Bogousslavsky J, Regli F. H\l=e'\miparesieavec atteinte linguale: h\l=e'\matom\l=e'\du genou de la cap- sule interne. Rev Neurol (Paris). 1984;140:587-590. 11. Rascol A, Clanet M, Manelfe C, Guiraud B, Bonafe A. Pure motor hemiplegia: CT study of 30 cases. Stroke. 1982;13:11-17. 12. Fisher CM. Capsular infarcts: the underlying vascular lesions. Arch Neurol. 1979;36:65-73. 13. Graff-Radford NR, Damasio H, Yamada T, Eslinger PJ, Damasio, AR. Nonhemorrhagic thalamic infarction: clinical, neuropsychological and electrophysiological findings in four anatomical groups defined by computerized tomography. Brain. 1985;108:485-516. 14. Helgason C, Caplan LR, Goodwin J, Hedges T. Anterior choroidal artery territory infarction: report of cases and review. Arch Neurol. 1986; 43:681-686. Successful Treatment of Pure Myopathy, Associated With Complex I Deficiency, With Riboflavin and Carnitine Pieter L. J. A. Bernsen, MD; Fons J. M. Gabre\l=e"\ls,MD, PhD; Wim Ruitenbeek, PhD; Rob C. A. Sengers, MD, PhD; Ad M. Stadhouders, MD, PhD; Willie O. Renier, MD, PhD \s=b\ We describe a 6-year-old boy who presented with progressive muscle weakness. Additional investigations revealed the existence of a myopathy and a pure motor neuropathy. Biochemical studies in muscle tissue showed a defect of NADH dehydrogenase (complex I). The patient dramatically improved on treatment with riboflavin and l-carnitine. Seven months after the start of the treatment, complex I activity was determined again and appeared to be normalized. Normalization of the enzymatic defect at this level has not been reported before. We provide a survey of nine patients with pure myopathy, associated with complex I deficiency and onset of symptoms in childhood. (Arch Neurol. 1991;48:334-338) rPhe primary mitochondrial myopathies form a clinically, morpholog¬ ically, and biochemically heteroge¬ group of inborn errors of me¬ tabolism affecting the energy-gener¬ ating pathway.1·2 The underlying bio¬ chemical defects can be classified into neous Accepted for publication June 29, 1990. From the Institute of Neurology, Department of Child Neurology (Drs Bernsen, Gabre\l=e"\ls,and Renier), the Institute of Pediatrics (Drs Ruitenbeek and Sengers), and the Institute of Cell Biology (Dr Stadhouders), St Radboud University Hospital, Nijmegen, the Netherlands. Presented in part at the 14th World Congress of Neurology, New Delhi, India, October 24, 1989. Reprints not available. four groups3 as follows: (1) defects of substrate transport; (2) defects of sub¬ strate utilization; (3) defects of the respiratory chain; and (4) defects of energy conservation and transduction. There is no correlation between the clinical picture and the biochemical defect. Patients with a defect of the respiratory chain may present from early infancy to adulthood. A de¬ creased activity of NADHQ, oxidoreductase (NADH dehydrogenase or complex I) is frequently found. Only nine patients with a combination of symptoms and onset at an infantile age and a clinically pure myopathy, associated with a complex I deficiency, have been described to date.410 In these patients the diagnosis of complex I de¬ ficiency has been established by indi¬ rect estimation of NADH-dependent substrate oxidation rates. Effective treatment of mitochondrial myopa¬ thies remains extremely limited. We describe a 6-year-old boy who presented with a progressive myopa¬ thy and motor neuropathy in whom complex I activity was found to be de¬ ficient. The defect could be demon¬ strated by indirect estimation of the NADH oxidation rate and by direct measurement of NADH:Qi oxidoreductase activity. His condition dramatically improved during treat¬ ment with riboflavin and L-carnitine. Downloaded From: http://archneur.jamanetwork.com/ by a University of Calgary User on 05/27/2015 REPORT OF A CASE The patient was the first child of healthy, unrelated parents. Pregnancy, delivery, and motor development were normal. His family history was unremarkable. He had been completely well until August 1987. At that time he started to complain about weakness in his legs, and it was noticed that he was subject to sudden falls. He became unable to run and play games at school. He had difficulty in climbing stairs. Two months later, he developed weakness in his hands to such an extent that he became un¬ able to hold a cup. His gait became waddling and he needed help with his daily activities. Three months after the start of his com¬ plaints he was referred to our hospital. On examination, the patient was an alert and cooperative 6-year-old boy. Results of general physical and ophthalmologic exam¬ ination revealed no abnormalities. On neu¬ rologic examination the cranial nerves were normal. There was a mild weakness of neck flexion, an increased lumbar lordosis, and winging of the scapulae. He had a waddling gait and was unable to sit up from the su¬ pine position without using his arms. Gow¬ ers' sign was positive. Muscles were gener¬ ally thin, with a moderate wasting of the proximal musculature. Muscle tone was impaired. There was a diffuse weakness (shoulder girdle, Medical Research Council [1] MRC grade 3; forearms, grade 4; intrin¬ sic hand muscles, grade 3%; pelvic girdle, quadriceps muscle, and hamstring muscle, grade 3; and ankle flexion and extension, grade 4) of all muscle groups that was more pronounced in the proximal musculature.