CASE REPORT intracranial bleed; systemic lupus erythematosus
Systemic Lupus Erythematosus Presenting as an Intracranial Bleed A previously healthy i2-year-old girl presented with fever and mental status changes without focal neurologic deficit. A CBC and computed tomography scan revealed thrombocytopenia and an intracranial bleed. Further laboratory studies were consistent with systemic lupus erythematosus. This case emphasizes the need to maintain a high index of suspicion for atypical presentations of an uncommon childhood illness. [Cunningham S, Conway EE Jr: Systemic lupus erythematosus presenting as an intracranial bleed. Aim Emerg Med July i991;20:810-812.] INTRODUCTION Systemic lupus erythematosus (SLE) is u n c o m m o n in childhood, with an estimated incidence in the United States of 0.6 per 100,000 children. 1 At presentation, children with SLE frequently have m u l t i s y s t e m disease, which usually includes rash, arthritis, and fever. Initial involvement of the central nervous system is uncommon, occurring in fewer than 15% of children. Possible manifestations include seizures, psychiatric disorders, personality changes, headache, hemiplegia, paraplegia, chorea, papilledema, aseptic meningitis, cranial nerve palsies, pseudotumor cerebri, and movement disorders.l-3 However, SLE presenting as an intracranial bleed is extremely rare, with only two cases reported in the English literature.4, s We report the case of a previously healthy 12-year-old girl with an acute subarachnoid and intraparenchymal hemorrhage as her initial manifestation of SLE.
Sandra Cunningham, MD*IEdward E Conway, Jr, MD*:~ Bronx, New York From the Department of Pediatrics, Albert Einstein College of Medicine;* Division of Ambulatory Care, Bronx Municipal Hospital Center;t and Division of Critical Care, Montefiore Medical Center,:~ Bronx, New York. Receded for publication August 27, 1990. Accepted f.or publication November 29, 1990. Address for reprints: Sandra Cunningham, MD, lW20 Jacobi Hospital, Pelham Parkway and Eastchester Road, Bronx, New York 10461.
CASE REPORT A 12-year-old girl was brought to the emergency department because of a two-hour history of behavioral changes and intermittent unresponsiveness. She had been well until the day of admission, when she awoke in the morning, complained of mild headache, and vomited once. She was given one 500-rag dose of acetaminophen, slept throughout most of the day, and vomited once again, but she did not complain of recurrent or persistent headache. Several hours later, she was noted to be lethargic, nonverbal, and only intermittently responsive to commands. In the ED, her mother reported that one to two weeks before admission she had a self-limited, generalized, red rash on the extremities. There was no history of head trauma, ingestion, or chronic illness. On physical examination, the patient was nonverbal but awake and making purposeful movements. Her Glasgow Coma Score was 11. The vital signs were blood pressure of 120/70 m m Hg; pulse, 84; respirations, 24; and temperature, 39.5 C. The pupils were equal and reactive to light, and the optic discs were sharp bilaterally. Nuchal rigidity was present. The skin was without petechiae or rash. The lungs were clear, and a grade I/VI systolic ejection m u r m u r was noted. The abdomen was benign. The extremities were normal without joint swelling or tenderness. The rectal examination was normal and stool was gnaiac-negative. The patient was Tanner stage 3. Neurological examination revealed no focal deficits. An IV line was started, and lactated Ringer's solution was infused at the rate of t w o - t h i r d s m a i n t e n a n c e . T h e p a t i e n t was g i v e n a 650-mg acetaminophen suppository and 2 mg naloxone IV with no change in mental status.
158/810
Annals of Emergency Medicine
20:7 July 1991
LUPUS Cunningham & Conway
T h e p a t i e n t ' s p r e s e n t a t i o n and physical findings were c o n s i s t e n t with meningitis, and a lumbar puncture was performed. T h e opening pressure was more than 300 mm. Microscopic examination revealed white blood cells of 28/ram 3 (28 × 106/L) with 75% polymorphonuclear cells; erythrocytes, 30,000/mm 3 (30 x 109/L); total protein, 284 mg/dL (2.84 g/L); and glucose, 97 mg/dL (5.4 mmol/L) with a concomitant serum glucose of 233 mg/dL (13 mmol/L). IV ampicillin and chloramphenicol were given. An arterial blood gas while b r e a t h i n g 30% F~o 2 was pH 7.44; Paco2, 21 m m Hg; and Pao2, 130 m m Hg. Serum toxicologic screen for aspirin, acetaminophen, and barbiturates was negative. Serum electrolytes were sodium of 134 mEq/L (mmol/L); potassium, 4.0 mEq/L (mmol/L); chloride, 100 mEq/ L (retool/L); bicarbonate, 20.5 mEq/L (mmol/L); blood urea nitrogen, 14 mg/dL (5 mmol/L); and creatinine, 0.9 mg/dL (80 ~mol/L). Urinalysis with microscopic examination was negative. CBC revealed white blood cells of 12,000/mm 3 (12 x 109/L) with 85% (0.85) polymorphonuclear cells, 11% (0.11) l y m p h o c y t e s , and 4% (0.04) monocytes; hemoglobin and hematocrit, 9.1 g/dL (91 g/L} and 27.1% (0.27); platelet count, 8 x 103/mm 3 (8 × 109/L); reticulocyte count, 1.9% {0.002}. Liver function tests revealed total bilirubin of 1.5 mg/dL (26 p~mol/ L); SGOT, 42 units/L (0.70 ~kat/L); SGPT, 10 units/L (O.17/~kat/L); ammonia, 19 m m / L (normal for laboratory is less than 35 ram/L). Erythrocyte sedimentation rate was 19 m m / hr. Prothrombin time was 12.2 s/13.0 s, partial t h r o m b o p l a s t i n t i m e was 29.1 s/34.0 s, and the fibrinogen was 280 mg/dL (2.8 g/L). During the next hour, the patient's neurologic status deteriorated with the d e v e l o p m e n t of a right h e m i paresis and bilaterally upgoing toes. Computed tomography of the head d e m o n s t r a t e d a left frontal hemorrhage with a significant midline shift (Figure). She was given mannitol 1 g/kg IV, intubated, and hyperventilate& During the computed tomography scan, the patient had a brief, generalized, tonic-clonie seizure and was given 15 m g / k g IV diphenylhydantoin over 20 minutes. She was transferred to the pediatric ICU, where she was continued on IV mannitol 20:7: July 1991
FIGURE. Computed tomography
scan showing left frontal hemorrhage with midline shift. and hyperventilation. A bone m a r r o w aspirate showed megakaryocytes and normal cellularity interpreted as consistent with i d i o p a t h i c t h r o m b o c y t o p e n i a purpura. She was given 8 units of platelets, 1 g/kg IV ~/-globulin, and 5 mg/ kg/day IV solumedrol. The patient was taken to the operating room where s~e underwent a left frontal eraniotomy, partial frontal l o b e c t o m y , r e m o v a l of a large clot, and p l a c e m e n t of an intraventricular catheter to measure the intracranial pressure. Neuropathologic examination revealed an acute subarachnoid and intraparenchymal hemorrhage without evidence of v a s c u l i t i s or v a s c u l a r malformation. Subsequent l a b o r a t o r y tests revealed fluorescent antinuclear antibody 3 + in a speckled pattern, total c o m p l e m e n t CH50 of 139 (normal, 150 to 250), positive anti-ENA, 2 + Coombs, and a negative lupus anticoagulant. The platelet count was normalized on the second hospital day, and she was extubated on day 13. On hospital day 36, the patient underwent bilateral common carotid angiogram with normal findings. At discharge on hospital day 58, she was able to initiate conversation appropriate for age and was ambulating unassisted. She was noted to have a persistent right-sided weakness and was transferred to a rehabilitative center for further therapy. At follow-up, the patient continued to make progress and was doing well. DISCUSSION Neurologic manifestations of SLE have been recognized since Kaposi first described the disease in 1872. 6 Osler noted neurologic involvement in lupus patients when he described the systemic nature of the disease in the 1890s. 7 N e u r o l o g i c m a n i f e s t a tions are an important feature of SLE, occurring at some t i m e in 15% to 75% of patients J-3, 8-1o The nature and time of onset of the central nervous system (CNS} involvement is variable. The most c o m m o n neurologic complication is seizures, occurring in 20% to 54% of SLE patients with CNS involvement, s-l° Seizures Annals of Emergency Medicine
generally occur early in the disease, but they can also occur as a terminal or preterminal event.9, n Rarely, neurologic dysfunction has been reported to be the sole presentation of SLE, but it is usually in conjunction with o t h e r m a n i f e s t a t i o n s of the disease.2, 9 In studies involving children with SLE, there are varied reports of CNS involvement. In a recent review of 23 children w i t h SLE, none presented with neurologic disease. 12 King et al found that CNS i n v o l v e m e n t was present in only 13% of 108 children at the time of diagnosisJ 3 Initially, cerebral vasculitis was thought to be the major pathologic entity responsible for CNS events. Johnson and Richardson reviewed 24 patients, 75% of w h o m had CNS involvement, and found that cerebral vasculitis (inflammatory cells within the v a s c u l a r wall) was present in o n l y t h r e e cases. 9 In s u b s e q u e n t studies, cerebral vasculitis continues to be a r a r e e v e n t . 3,8,10,]4 The neurop a t h o l o g y of lupus p a t i e n t s w i t h CNS fnvolvement is generally widespread cerebral infarcts and hemor~ rhage. Casey and Symon reported the case of a 13-year-old with headache, nausea, vomiting, and a butterfly skin rash who was found to have a subarachnoid hemorrhage and was subsequently diagnosed with SLE on the basis of a skin biopsy, s Silverstein reported five patients ranging in age from 27 to 63 years who presented with cerebrovascular accidents as the initial manifestation of SLE.4 One patient was found to have a probable 811/159
LUPUS Cunningham & Conway
subarachnoid hemorrhage and t h r o m b o c y t o p e n i a . Silverstein reported five other cases from the literature of patients who sustained cerebrovascular accidents before the diagnosis of SLE; however, they all had non-neurologic antecedent signs or symptoms compatible with SLE. The lupus anticoagulant occurs in approximately 10% of SLE patients and rarely is the cause of clinical bleeding, s The lupus anticoagulant was not present in our patient. Thrombocytopenia is estimated to occur in 75% of SLE patients and can significantly increase the risk of clinical bleeding. In a 1982 study of 62 patients with h e m a t o l o g i c abnormalities, 35 had idiopathic thrombocytopenia purpura is and approximately 25% of that subgroup developed SLE within one to six years. In 1983, Glidden et al retrospectively reviewed 55 children with SLE.16 Although the majority presented in a manner similar to children in other studies, they found that an atypical presentation, such as an isolated hematologic abnormality, may be the sole presentation of SLE.
SUMMARY We report the case of a 12-year-old girl with an intracranial hemorrhage and i d i o p a t h i c t h r o m b o c y t o p e n i a
160/812
purpura whose laboratory examinations were c o n s i s t e n t w i t h SLE. Spontaneous cerebral hemorrhage is a rare complication of childhood idiopathic thrombocytopenia purpura, with a prevalence ranging from less than 1% to 2%. 17 This case illustrates that an intracranial hemorrhage should always be considered in the differential diagnosis of acute mental status changes, even if the initial neurologic findings are nonfocal. In addition, an intracranial hemorrhage may be the sole presentation of SLE. We recommend that a workup for SLE be considered for any patient with an intracranial h e m o r r h a g e s e c o n d a r y to t h r o m bocytopenia. The authors thank Drs Ellen Crain and Jeffrey Gershel for their careful reading of the manuscript.
REFERENCES i. E m e r y H: C l i n i c a l a s p e c t s of s y s t e m i c lupus erythematosus in childhood. Pediatr Clin North A m 1986;33:1177-1190. 2. Delaney P: Neurologie complications of systemic lupus erythematous. A m Faro Physician 1983128: 191-193.
5. Casey E, Symou L: Systemic lupus erythematosus presenting as subarachnoid haemorrhage and space occupying lesion. Br J Dermatoi 1971;84:157-160. 6. Kaposi M: Neue beitrage zur k e n n m i s des lupus erythematosus. Arch Dermal U Svph 1872~4:36-78. 7. Osier W: On the visceral complications of erythema e x u d a t i v u m multiforme. A m J Med Sci 1895;110: 629-646. 8. Bresnihan B: CNS lupus. Clin Rhe~lm Dis 1982; 8:183-195. 9. Johnson R, Richardson E: The neurological manifestations of systemic lupus erythematosus. Medicine 1968;47:337-369. 10. Ellis 8, Verity M: Central nervous system involvement in systemic lupus erythematosus: A review of neuropathologic findings in 57 cases, [955-1977. Semin Arthritis Rheum 1979;8:212-22L i1. Adelman D, Sahiel E, Klinenberg J: The neuropsychiatric manifestations of systemic lupus erythematosus: An o v e r v i e w . Semin A r t h r i t i s Rhe~zm I986; 15:185-199. 12. Lehman T, McCurdy D, Bernstein B, et ah Systemic lupus erythematosus in the first decade of life. Pediatrics 1989;83:235-238. 13. King K, Kornreich H, Bernstein B, et al: The clinical spectrum of systemic lupus erythematosus in childhood. Arthritis Rheum 1977;20:287-294. 14. Devinsky O, PetJto C, Alonso D: Clinical and neuropathologieal findings in systemic lupus erythematosus: The role of vasculitis, heart emboli and thrombotic thrombocytopenia. Ann Netzrol 1988;23:380-384. 15. Lavalle C, Hurtado R, Quezada J: Hemocytopenia Initial manifestation of systemic lupus erythematosus: Prognostic significance. Clin Rheum 1982;2:227-232.
3. Gibson T, Myers A: Nervous system involvement in systemic lupus erythematous. Ann Rheum Dis 1976; 35:398-406.
16. Glidden R, Mantzouranis E, Borel Y: Systemic lupus erythematosus in childhood: Clinical manifestations and improved survival in fifty-five patients. Clin lmmLtno] hnmlznopathol 1983~29:196-210.
4. Silverstein A: Cerebrovascular accidents as the initial major manifestation of lupus erythematosus. N Y State J Med 1963;63:2942 2948.
17. Woerner S, Abildgaard C, French B: Intracranial h e m o r r h a g e in c h i l d r e n w i t h i d i o p a t h i c t h r o m bocytopenic purpura. Pediatrics 1981;67:453-460.
Annals of Emergency Medicine
20:7 July 1991
Systemic Lupus Erythematosus Presenting as an Intracranial Bleed A previously healthy i2-year-old girl presented with fever and mental status changes without focal neurologic deficit. A CBC and computed tomography scan revealed thrombocytopenia and an intracranial bleed. Further laboratory studies were consistent with systemic lupus erythematosus. This case emphasizes the need to maintain a high index of suspicion for atypical presentations of an uncommon childhood illness. [Cunningham S, Conway EE Jr: Systemic lupus erythematosus presenting as an intracranial bleed. Aim Emerg Med July i991;20:810-812.] INTRODUCTION Systemic lupus erythematosus (SLE) is u n c o m m o n in childhood, with an estimated incidence in the United States of 0.6 per 100,000 children. 1 At presentation, children with SLE frequently have m u l t i s y s t e m disease, which usually includes rash, arthritis, and fever. Initial involvement of the central nervous system is uncommon, occurring in fewer than 15% of children. Possible manifestations include seizures, psychiatric disorders, personality changes, headache, hemiplegia, paraplegia, chorea, papilledema, aseptic meningitis, cranial nerve palsies, pseudotumor cerebri, and movement disorders.l-3 However, SLE presenting as an intracranial bleed is extremely rare, with only two cases reported in the English literature.4, s We report the case of a previously healthy 12-year-old girl with an acute subarachnoid and intraparenchymal hemorrhage as her initial manifestation of SLE.
Sandra Cunningham, MD*IEdward E Conway, Jr, MD*:~ Bronx, New York From the Department of Pediatrics, Albert Einstein College of Medicine;* Division of Ambulatory Care, Bronx Municipal Hospital Center;t and Division of Critical Care, Montefiore Medical Center,:~ Bronx, New York. Receded for publication August 27, 1990. Accepted f.or publication November 29, 1990. Address for reprints: Sandra Cunningham, MD, lW20 Jacobi Hospital, Pelham Parkway and Eastchester Road, Bronx, New York 10461.
CASE REPORT A 12-year-old girl was brought to the emergency department because of a two-hour history of behavioral changes and intermittent unresponsiveness. She had been well until the day of admission, when she awoke in the morning, complained of mild headache, and vomited once. She was given one 500-rag dose of acetaminophen, slept throughout most of the day, and vomited once again, but she did not complain of recurrent or persistent headache. Several hours later, she was noted to be lethargic, nonverbal, and only intermittently responsive to commands. In the ED, her mother reported that one to two weeks before admission she had a self-limited, generalized, red rash on the extremities. There was no history of head trauma, ingestion, or chronic illness. On physical examination, the patient was nonverbal but awake and making purposeful movements. Her Glasgow Coma Score was 11. The vital signs were blood pressure of 120/70 m m Hg; pulse, 84; respirations, 24; and temperature, 39.5 C. The pupils were equal and reactive to light, and the optic discs were sharp bilaterally. Nuchal rigidity was present. The skin was without petechiae or rash. The lungs were clear, and a grade I/VI systolic ejection m u r m u r was noted. The abdomen was benign. The extremities were normal without joint swelling or tenderness. The rectal examination was normal and stool was gnaiac-negative. The patient was Tanner stage 3. Neurological examination revealed no focal deficits. An IV line was started, and lactated Ringer's solution was infused at the rate of t w o - t h i r d s m a i n t e n a n c e . T h e p a t i e n t was g i v e n a 650-mg acetaminophen suppository and 2 mg naloxone IV with no change in mental status.
158/810
Annals of Emergency Medicine
20:7 July 1991
LUPUS Cunningham & Conway
T h e p a t i e n t ' s p r e s e n t a t i o n and physical findings were c o n s i s t e n t with meningitis, and a lumbar puncture was performed. T h e opening pressure was more than 300 mm. Microscopic examination revealed white blood cells of 28/ram 3 (28 × 106/L) with 75% polymorphonuclear cells; erythrocytes, 30,000/mm 3 (30 x 109/L); total protein, 284 mg/dL (2.84 g/L); and glucose, 97 mg/dL (5.4 mmol/L) with a concomitant serum glucose of 233 mg/dL (13 mmol/L). IV ampicillin and chloramphenicol were given. An arterial blood gas while b r e a t h i n g 30% F~o 2 was pH 7.44; Paco2, 21 m m Hg; and Pao2, 130 m m Hg. Serum toxicologic screen for aspirin, acetaminophen, and barbiturates was negative. Serum electrolytes were sodium of 134 mEq/L (mmol/L); potassium, 4.0 mEq/L (mmol/L); chloride, 100 mEq/ L (retool/L); bicarbonate, 20.5 mEq/L (mmol/L); blood urea nitrogen, 14 mg/dL (5 mmol/L); and creatinine, 0.9 mg/dL (80 ~mol/L). Urinalysis with microscopic examination was negative. CBC revealed white blood cells of 12,000/mm 3 (12 x 109/L) with 85% (0.85) polymorphonuclear cells, 11% (0.11) l y m p h o c y t e s , and 4% (0.04) monocytes; hemoglobin and hematocrit, 9.1 g/dL (91 g/L} and 27.1% (0.27); platelet count, 8 x 103/mm 3 (8 × 109/L); reticulocyte count, 1.9% {0.002}. Liver function tests revealed total bilirubin of 1.5 mg/dL (26 p~mol/ L); SGOT, 42 units/L (0.70 ~kat/L); SGPT, 10 units/L (O.17/~kat/L); ammonia, 19 m m / L (normal for laboratory is less than 35 ram/L). Erythrocyte sedimentation rate was 19 m m / hr. Prothrombin time was 12.2 s/13.0 s, partial t h r o m b o p l a s t i n t i m e was 29.1 s/34.0 s, and the fibrinogen was 280 mg/dL (2.8 g/L). During the next hour, the patient's neurologic status deteriorated with the d e v e l o p m e n t of a right h e m i paresis and bilaterally upgoing toes. Computed tomography of the head d e m o n s t r a t e d a left frontal hemorrhage with a significant midline shift (Figure). She was given mannitol 1 g/kg IV, intubated, and hyperventilate& During the computed tomography scan, the patient had a brief, generalized, tonic-clonie seizure and was given 15 m g / k g IV diphenylhydantoin over 20 minutes. She was transferred to the pediatric ICU, where she was continued on IV mannitol 20:7: July 1991
FIGURE. Computed tomography
scan showing left frontal hemorrhage with midline shift. and hyperventilation. A bone m a r r o w aspirate showed megakaryocytes and normal cellularity interpreted as consistent with i d i o p a t h i c t h r o m b o c y t o p e n i a purpura. She was given 8 units of platelets, 1 g/kg IV ~/-globulin, and 5 mg/ kg/day IV solumedrol. The patient was taken to the operating room where s~e underwent a left frontal eraniotomy, partial frontal l o b e c t o m y , r e m o v a l of a large clot, and p l a c e m e n t of an intraventricular catheter to measure the intracranial pressure. Neuropathologic examination revealed an acute subarachnoid and intraparenchymal hemorrhage without evidence of v a s c u l i t i s or v a s c u l a r malformation. Subsequent l a b o r a t o r y tests revealed fluorescent antinuclear antibody 3 + in a speckled pattern, total c o m p l e m e n t CH50 of 139 (normal, 150 to 250), positive anti-ENA, 2 + Coombs, and a negative lupus anticoagulant. The platelet count was normalized on the second hospital day, and she was extubated on day 13. On hospital day 36, the patient underwent bilateral common carotid angiogram with normal findings. At discharge on hospital day 58, she was able to initiate conversation appropriate for age and was ambulating unassisted. She was noted to have a persistent right-sided weakness and was transferred to a rehabilitative center for further therapy. At follow-up, the patient continued to make progress and was doing well. DISCUSSION Neurologic manifestations of SLE have been recognized since Kaposi first described the disease in 1872. 6 Osler noted neurologic involvement in lupus patients when he described the systemic nature of the disease in the 1890s. 7 N e u r o l o g i c m a n i f e s t a tions are an important feature of SLE, occurring at some t i m e in 15% to 75% of patients J-3, 8-1o The nature and time of onset of the central nervous system (CNS} involvement is variable. The most c o m m o n neurologic complication is seizures, occurring in 20% to 54% of SLE patients with CNS involvement, s-l° Seizures Annals of Emergency Medicine
generally occur early in the disease, but they can also occur as a terminal or preterminal event.9, n Rarely, neurologic dysfunction has been reported to be the sole presentation of SLE, but it is usually in conjunction with o t h e r m a n i f e s t a t i o n s of the disease.2, 9 In studies involving children with SLE, there are varied reports of CNS involvement. In a recent review of 23 children w i t h SLE, none presented with neurologic disease. 12 King et al found that CNS i n v o l v e m e n t was present in only 13% of 108 children at the time of diagnosisJ 3 Initially, cerebral vasculitis was thought to be the major pathologic entity responsible for CNS events. Johnson and Richardson reviewed 24 patients, 75% of w h o m had CNS involvement, and found that cerebral vasculitis (inflammatory cells within the v a s c u l a r wall) was present in o n l y t h r e e cases. 9 In s u b s e q u e n t studies, cerebral vasculitis continues to be a r a r e e v e n t . 3,8,10,]4 The neurop a t h o l o g y of lupus p a t i e n t s w i t h CNS fnvolvement is generally widespread cerebral infarcts and hemor~ rhage. Casey and Symon reported the case of a 13-year-old with headache, nausea, vomiting, and a butterfly skin rash who was found to have a subarachnoid hemorrhage and was subsequently diagnosed with SLE on the basis of a skin biopsy, s Silverstein reported five patients ranging in age from 27 to 63 years who presented with cerebrovascular accidents as the initial manifestation of SLE.4 One patient was found to have a probable 811/159
LUPUS Cunningham & Conway
subarachnoid hemorrhage and t h r o m b o c y t o p e n i a . Silverstein reported five other cases from the literature of patients who sustained cerebrovascular accidents before the diagnosis of SLE; however, they all had non-neurologic antecedent signs or symptoms compatible with SLE. The lupus anticoagulant occurs in approximately 10% of SLE patients and rarely is the cause of clinical bleeding, s The lupus anticoagulant was not present in our patient. Thrombocytopenia is estimated to occur in 75% of SLE patients and can significantly increase the risk of clinical bleeding. In a 1982 study of 62 patients with h e m a t o l o g i c abnormalities, 35 had idiopathic thrombocytopenia purpura is and approximately 25% of that subgroup developed SLE within one to six years. In 1983, Glidden et al retrospectively reviewed 55 children with SLE.16 Although the majority presented in a manner similar to children in other studies, they found that an atypical presentation, such as an isolated hematologic abnormality, may be the sole presentation of SLE.
SUMMARY We report the case of a 12-year-old girl with an intracranial hemorrhage and i d i o p a t h i c t h r o m b o c y t o p e n i a
160/812
purpura whose laboratory examinations were c o n s i s t e n t w i t h SLE. Spontaneous cerebral hemorrhage is a rare complication of childhood idiopathic thrombocytopenia purpura, with a prevalence ranging from less than 1% to 2%. 17 This case illustrates that an intracranial hemorrhage should always be considered in the differential diagnosis of acute mental status changes, even if the initial neurologic findings are nonfocal. In addition, an intracranial hemorrhage may be the sole presentation of SLE. We recommend that a workup for SLE be considered for any patient with an intracranial h e m o r r h a g e s e c o n d a r y to t h r o m bocytopenia. The authors thank Drs Ellen Crain and Jeffrey Gershel for their careful reading of the manuscript.
REFERENCES i. E m e r y H: C l i n i c a l a s p e c t s of s y s t e m i c lupus erythematosus in childhood. Pediatr Clin North A m 1986;33:1177-1190. 2. Delaney P: Neurologie complications of systemic lupus erythematous. A m Faro Physician 1983128: 191-193.
5. Casey E, Symou L: Systemic lupus erythematosus presenting as subarachnoid haemorrhage and space occupying lesion. Br J Dermatoi 1971;84:157-160. 6. Kaposi M: Neue beitrage zur k e n n m i s des lupus erythematosus. Arch Dermal U Svph 1872~4:36-78. 7. Osier W: On the visceral complications of erythema e x u d a t i v u m multiforme. A m J Med Sci 1895;110: 629-646. 8. Bresnihan B: CNS lupus. Clin Rhe~lm Dis 1982; 8:183-195. 9. Johnson R, Richardson E: The neurological manifestations of systemic lupus erythematosus. Medicine 1968;47:337-369. 10. Ellis 8, Verity M: Central nervous system involvement in systemic lupus erythematosus: A review of neuropathologic findings in 57 cases, [955-1977. Semin Arthritis Rheum 1979;8:212-22L i1. Adelman D, Sahiel E, Klinenberg J: The neuropsychiatric manifestations of systemic lupus erythematosus: An o v e r v i e w . Semin A r t h r i t i s Rhe~zm I986; 15:185-199. 12. Lehman T, McCurdy D, Bernstein B, et ah Systemic lupus erythematosus in the first decade of life. Pediatrics 1989;83:235-238. 13. King K, Kornreich H, Bernstein B, et al: The clinical spectrum of systemic lupus erythematosus in childhood. Arthritis Rheum 1977;20:287-294. 14. Devinsky O, PetJto C, Alonso D: Clinical and neuropathologieal findings in systemic lupus erythematosus: The role of vasculitis, heart emboli and thrombotic thrombocytopenia. Ann Netzrol 1988;23:380-384. 15. Lavalle C, Hurtado R, Quezada J: Hemocytopenia Initial manifestation of systemic lupus erythematosus: Prognostic significance. Clin Rheum 1982;2:227-232.
3. Gibson T, Myers A: Nervous system involvement in systemic lupus erythematous. Ann Rheum Dis 1976; 35:398-406.
16. Glidden R, Mantzouranis E, Borel Y: Systemic lupus erythematosus in childhood: Clinical manifestations and improved survival in fifty-five patients. Clin lmmLtno] hnmlznopathol 1983~29:196-210.
4. Silverstein A: Cerebrovascular accidents as the initial major manifestation of lupus erythematosus. N Y State J Med 1963;63:2942 2948.
17. Woerner S, Abildgaard C, French B: Intracranial h e m o r r h a g e in c h i l d r e n w i t h i d i o p a t h i c t h r o m bocytopenic purpura. Pediatrics 1981;67:453-460.
Annals of Emergency Medicine
20:7 July 1991
