CLINICAL EXPERIENCES Lacunar Infarction of the Basal Ganglia as a Complication of Hemolytic-uremic Syndrome MRI and Clinical Correlations Francis J. DiMario, Jr., MD,* Helen Brönte-Stewart, MD,‡ Joseph Sherbotie, MD,† Mary Ellen Turner, MD† Central nervous system (CNS) complications of hemolytic-uremic syndrome (HUS) commonly consist of alterations in mental status, seizures, and rarely 1,2 The authors report the clinical evolution of left hemiparesis and hemiparesis. later choreo-athetoid movements in a patient who sustained a right lacunar infarction as a complication of HUS. The infarction is demonstrated on magnetic resonance imaging (MRI). CEREBRAL cerebral INFARCTION as a complication of hemolytic-uremic syndrome (HUS), is uncommon.’ Although the pathophysiology of this syndrome involves small vessel occlusions, cerebral occlusive syndromes have been reported infrequently. We report the clinical and magnetic resonance imaging (MRI) findings of a patient who developed a lacunar infarction of the basal ganglia as a complication of HUS. Case Report A previously healthy 5.5-year-old right-handed girl was admitted after a 7-day evolution of dehydration and bloody diarrhea. On the day of admission she complained of headache and light headedness. Family history was significant for a maternal first cousin who suffered a grand-mal seizure during an episode of hemolytic-uremic syndrome (HUS). Her general examination was notable for parched mucous membranes. She had a normal neurological exam except for mild encephalopathy manifested by From the Divisions of *Pediatric Neurology, †Nephrology and Pediatrics, The Children’s Hospital of Philadelphia ; and ‡The Department of Neurology, The Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania. Correspondence to: Francis J. DiMario, Jr., MD, Division of Neurology, The Children’s Hospital of Philadelphia, 34th Street & Civic Center Blvd, Philadelphia, PA 19104. Received for publication May 1987, revised July 1987, and accepted August 1987. inattention and disorientation. Laboratory data upon admission revealed thrombocytopenia, hemolytic anemia, and azotemia consistent with a diagnosis of HUS (Table 1). Urinalysis was positive for proteinuria, hematuria, and granular casts. No stool culture was done. She remained hospitalized for 7 days after receiving an infusion of fresh frozen plasma (FFP) and washed, packed, red blood cells (PRBC). Her mental status gradually returned to normal with improvement of her metabolic and hematologic abnormalities. She was discharged home on day 15 of illness. Two days after discharge she was noted to have difficulty climbing stairs and noticeable clumsiness of her left hand. There were no complaints of vertigo, visual changes, or difficulty swallowing. Examination on readmission revealed a left hemiparesis, predominantly involving the distal anti-gravity musculature of the leg more than the arm and not involving the face. Mental status, cranial nerve, and sensory exams were normal. She was treated with infusions of FFP as well as receiving physical and occupational therapy. A head computerized tomography scan with and without contrast 3 days after the onset of weakness was normal. She was discharged home 27 days later still demonstrating a mild left hemiparesis, from a probable right hemispheric lacunar infarction. Three days following her second discharge the patient was noted to be &dquo;speaking out of the left side of her mouth.&dquo; Examination subsequently revealed an increased left hemiparesis with new findings of left facial droop, left palatal paresis and tongue deviation 586 Downloaded from cpj.sagepub.com at The University of Auckland Library on June 29, 2015 Mental status and the remainder of her cranial nerves and sensory exams were normal. She again received infusions of FFP with continued physical and occupational therapy. An MRI of the brain and brainstem 30 days after the initial onset of weakness revealed a subacute hemorrhage of the right caudate and lentiform nuclei without demonstrable brain stem lesions (Fig. 1). She was discharged home with a complete resolution of palatal weakness and partial resolution of her left hemiparesis, facial droop, and tongue deviation. Subsequently, she has developed an involuntary movement disorder. This is predominantly characterized by choreo-athetoid movements of the left arm. to the left. Discussion A E £ at c C v c q 0 >, C) z 0 oJ::> C -4 &dquo;Ci 2 b-. -5iH L 8 II &< w - C o C w G 1J ...... w Il e0 < N i I E § c P ¡:.; m r-: &< I E (~5 fa0 v m I G :Ern E§ v !3 ~b .E I week and < 1 month old), or chronic (> I month old).9 The MRI in our patient is a subacute lesion because of its hyperintensity on both the T1 and T2 weighted images. A lesion 1 week to 1 month of age conformed well to the clinical history. This case provides both clinical and neuroimaging evidence for the microvascular thrombotic complications of HUS within the central nervous system. Neither lacunar infarction in HUS nor MRI of this lesion in HUS, to our knowledge, has been reported staged as acute (< previously. Acknowledgments We express our appreciation to Dr. Larissa T. Bilaniuk for neuro-imaging interpretation, Dr. Samuel Tucker for reviewing the manuscript, and Linda Cella for preparing the manuscript. 588 Downloaded from cpj.sagepub.com at The University of Auckland Library on June 29, 2015 FIG. 1. (Continued) 589 Downloaded from cpj.sagepub.com at The University of Auckland Library on June 29, 2015 References 1. 1980;134:869-72. 2. Gianantonio CA, Vitacco M, Mendilaharzu F, Gallo GE, Sojo ET. The hemolytic-uremic syndrome. Nephron 1973;11:174-92. 3. Gianantonio CA, Vitacco M, Mendilaharzu F, Rutty A, Mendilaharzu J. The hemolytic-uremic syndrome.J Pediatr 1964;64:478-91. 4. Upadhyaya K, Barwick K, Fishaut M, Kashgarian M, Siegel N. The importance of non-renal involvement in hemolytic-uremic syndrome. Pediatrics 1980;65:115-20. Rodney JC, Anderson R McD, Hopkins IJ. Clinical and pathological aspects of central nervous system involvement in the hemolytic-uremic syndrome. Aust PaediatrJ 1971;7:28. 6. Crisp DE, Siegler RL, Bale JF, Thompson JA. 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