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Pediatric eosinophilic fasciitis: About 2 cases
e184
Abstracts
clinical presentation, 86.04% of patients showed epistaxis and 81.47% telangiectases (no significative differences considering genetic type for both cases), 42.80% presented with pulmonary arteriovenous malformations (significative higher prevalence in HHT1 patients), 9.22% had brain arteriovenous malformations (significative higuer in HHT1) and 62.73% showed liver infection (significative higuer in HHT2). Discussion: Prevalence of HHT in Spain is calculated to be in the highest levels compared with international series, however it remains in the range of rare diseases. As in other mediterranean countries, mutations in ALK1 (HHT2) seem to be more frequent. Percentages of epistaxis are slightly lower probably due to a younger population. Pulmonary and liver infections are in the same range of other series and although neurological screening is always performed, brain involvement is much lower.
doi:10.1016/j.ejim.2013.08.472
ID: 385 Forearm ischemic exercise test (FIET). Experience in an Internal Medicine Department C. Cardozo, J.C. Milisenda, P.J. Moreno-Lozano, V. Lazo, A. Jérez, R.A. Losno, J.M. Grau Muscle Research Unit, Internal Medicine Department, Hospital Clinic, CIBERER, Barcelona, Spain
Objective: The objective of this study is to describe the patients's characteristics and the diagnosis we made during a ten-year period performing the FIET in a Muscle Research Unit from an Internal Medicine Department. Methods: A total of 107 patients were evaluated during a ten-year period. The main (non-excluding) complaints of such patients were: cramps, exercise intolerance, abnormal fatigue, iterative rhabdomyolysis or unexplained hyperCKemia. In some cases an additional muscle biopsy was also performed in order to confirm or to rule out the suspected disease. A descriptive analysis of the results was performed. Results: In this 10-year period, 56% of the patients were male, with a mean age of 41.7 (range 13– 72 years). The most frequent symptoms were exercise intolerance and abnormal fatigue. From 107 patients, in 29 (27.1%) pathological curves were obtained: 9 patients received the diagnosis of McArdle's disease, 17 of Myoadenylate deaminase deficiency and 3 cases nonMcArdle glucogenosis (phosphofructokinase deficiency one case). Both curves were plane only in 8 cases, representing a non-proper exercise in the test. In any case, no complication was observed during the test. Conclusions: FIET was a safe and very useful test, and it allowed us to achieve a diagnosis of metabolic myopathy in 27.1% of the tested patients. FIET is a test that must be performed when a metabolic myopathy is suspected. It's important to stand out that, in some patients, it was no longer necessary to perform a muscle biopsy because of the results in the FIET. doi:10.1016/j.ejim.2013.08.473
ID: 429 A delayed diagnosis of tuberous sclerosis C. Oliveira, R. Real, G. Nadais, C. Garret Neurology, Centro Hospitalar São João, Porto, Portugal
Introduction: Tuberous sclerosis is an autosomal dominant genetic disorder with multisystem clinical manifestations. The expanded
phenotype is now called the tuberous sclerosis complex (TSC), to emphasize the multiorgan involvement, characterized by the formation of hamartomatous lesions in the brain, heart, skin, kidney, lung and other organs. The incidence is approximately 1:6000 newborns and the diagnosis typically occurs during childhood, with most patients presenting with seizures or cutaneous involvement. Vascular manifestations of TS are now being more frequently acknowledged, and although significant vascular lesions are rare, they can be life threatening. Because of the wide range of organs affected, a multidisciplinary team approach is ideal and produces the best outcomes for patients. Treatment options for TSC have been limited, focusing primarily on the management of symptoms. Case report: A 38-yearold woman was referred to the Neurology clinic due to a family history of TSC (the daughter had been diagnosed with TSC at the age of 8 months, in the context of epilepsy) and suspicious skin lesions. The patient denied a history of seizures or other neurological symptoms, although she reported a mild learning disability in school. The patient had a background of arterial hypertension and aortic arch aneurysm that had been surgically corrected two years before. On physical examination, several skin lesions were present (hypomelanotic macules, angiofibromas and hyperpigmented nevus) on the face, neck, legs and arms; according to the patient, these lesions had been present since childhood. The neurological examination was unremarkable. The brain MRI revealed the presence of cortical and subcortical tubers, a number of small subependymal nodules in the walls of the lateral ventricles and a nodular lesion with intense gadolinium enhancement located on the left lateral ventricle near the foramen of Monro, suggestive of a subependymal giant cell astrocytoma. The renal ultrasound showed multiple hyperechoic nodular formations, likely angiomyolipomas. Abdominal MRI further identified multiple angiolipomas and cystic formations in the kidney, and a liver hemangioma. The chest CT did not show significant changes in lung interstitium, only ground-glass opacities in the middle lobe adjacent to the large fissure. An ophthalmological evaluation excluded retinal lesions and a dentistry workup revealed gingival fibromas and a tongue lesion that the patient refused to biopsy. Conclusions: The patient fulfills clinical criteria for definite TSC. Although the patient has multisystem involvement, the diagnosis was delayed until adulthood, a fact that underscores the importance of recognizing the full spectrum of signs and symptoms of this condition. Also, this case is atypical for its significant vascular involvement (aortic arch aneurysm), which is a rare manifestation of TSC, especially in adults. To conclude, it is important that physicians recognize the full spectrum of manifestations of TSC, including the most unusual, so not to miss the diagnosis. doi:10.1016/j.ejim.2013.08.474
ID: 436 Pediatric eosinophilic fasciitis: About 2 cases R. Lihioua, O. Harzallaha, A. Boukera, A. Jelladb, S. Hammamic, M. Kechidaa, S. Mahjouba a
Internal Medicine Department, Fattouma Bourguiba Hospital, Monastir, Tunisia b Rehabilitation and Physical Therapy Department, Fattouma Bourguiba Hospital, Monastir, Tunisia c Pediatrics Department, Fattouma Bourguiba Hospital, Monastir, Tunisia
Introduction: Eosinophilic fasciitis is an uncommon entity characterized by a scleroderma-like induration of the extremities skin and connective tissue and by blood eosinophilia. This singular syndrome is very rare in children with almost 30 cases described in literature. Methods: We report two cases of childhood eosinophilic fasciitis.
Abstracts
Results: Case report 1: A 13 year old girl was seen for skin induration of the abdomen and limbs and massive weight loss. Physical examination noted a limited abduction of shoulders and irreducible flexion contracture of knees and elbows. Laboratory tests revealed eosinophilia at 1700 E/mm3 and hypergammaglobulinemia. A deep tissue biopsy noted a dermal thickening and fibrosis with perivascular nonspecific inflammatory infiltration, a fibrotic fascia with abundant infiltration by lymphocytes, plasma cells and numerous eosinophils. Muscular specimen shows no abnormalities. Magnetic resonance imaging showed fasciitis involving the different muscular compartments of the thigh predominant in the posterior compartment associated to muscular atrophy without significant anomalies of intra-muscular or subcutaneous fat signal intensity. Our two patients were treated with prednisone (1 mg/kg/day) and physical therapy with insufficient response. Methotrexate was associated at 10 mg/week in addition to prolonged physical rehabilitation. The follow-up was marked by partial improvement of skin induration and joint stiffness. Case report 2: A 15 year old girl with a history of Hashimoto disease was seen for painless thickening of legs and forearms skin with joint stiffness and proximal muscular deficit. Physical examination noted also flexion contractures of the fingers and muscular atrophy of triceps and quadriceps. Laboratory testing revealed a peripheral eosinophilia at 1100 E/mm3, hypergammaglobulinemia, anemia and inflammatory biologic syndrome. Muscular enzymes were at normal level. A deep tissue biopsy revealed subcutaneous nonspecific inflammatory infiltration with fibrosis of the derma. Muscular biopsy showed a nonspecific myositis. Magnetic resonance imaging redescribed myositis involving pelvic muscles and fasciitis of the muscular lodges at the thigh backside. The evolution was characterized by a steroid's resistance indicating the association of methotrexate (12 mg per week). Conclusion: Eosinophilic fasciitis is an uncommon disease in childhood that must be considered in any patient with skin induration and painless joint contractures. The majority of patients are steroid responsive, but recourse to more aggressive treatment may be necessary. Interdisciplinary approach, including physical therapy, is crucial to prevent long-term morbidity. doi:10.1016/j.ejim.2013.08.475
ID: 440 Diagnosis of Erdheim–Chester disease following the workup of multiple falls I.R. Bistreanua, T. Nguyenb, S. Daensc, R. Attoua a
Internal Medicine Department, Brugman University Hospital, Brussels, Belgium b Internal Medicine Department, Erasme University Hospital, Brussels, Belgium c Rheumatology Department, Brugman University Hospital, Brussels, Belgium
Observation: A 65 year old woman presents to the Emergency Department for multiple falls and she is complaining of right ankle and left shoulder pain. She has had lower limb pain and gait difficulties for a few months. Recent memory losses were reported by family members. Past medical history includes appendectomy and cholecystectomy. X-rays reveal a left trochiter fracture and an old fracture of the proximal phalanx of the third right toe. Management is conservatory. The right tibia x-ray also demonstrates an osseous condensation in the medullo-metaphyseal region that warrants further investigation. Bone MRI shows heterogeneous focal lesions in the femurs and tibias. Whole body MRI is performed and shows also medullary lesions of both humerus without involvement of the spine and the pelvis. The distribution of lesions is suggestive of Erdheim–
e185
Chester disease. A bone biopsy is performed and confirms the diagnosis of Erdheim–Chester histiocytosis. Cerebral MRI and chest CT find disseminated lesions in the pons, cerebellum and pulmonary infiltrates, respectively. With such disseminated disease, the therapeutic decision is to start the interferon alpha. Discussion: We report a case of Erdheim–Chester disease, a rare non-Langerhans' cell histiocytosis, with characteristic radiological and histological features. The most common characteristic is bilateral and symmetric osteosclerotic lesions in long bones. Usually, patients complain of bone pain, as in our case. Interestingly, our patient initially consulted because of multiple falls and gait difficulties. Only several hundred cases have been reported in the medical literature. About half of the patients have extraskeletal manifestations and the prognosis depends on it. Imaging and clinical biology assess the extent of the disease. Neurological involvement includes ataxia, paresis, and diabetes insipidus. The differential diagnosis includes Langerhans' cell histiocytosis, metabolic disorders and malignancies. Treatment of Erdheim–Chester disease must be decided on a case-to-case basis taking into account patient's symptoms. Interpheron is the first-intention therapy, but corticosteroids, chemotherapy, surgical resection and radiotherapy have been used also. Conclusion: Erdheim–Chester disease is a rare systemic nonLangerhans' cell histiocytosis of the middle-aged adult. Bone pain is the most frequent of all symptoms and mainly affects the lower limbs, knees and ankles. The combination of multisystemic symptoms with typical radiological findings can direct the diagnosis. However, a histological examination is crucial for the diagnosis of Erdheim–Chester disease. doi:10.1016/j.ejim.2013.08.476
ID: 483 Catastrophic antiphospholipid syndrome: The aftermath H. Casimiro, P. Carreira, B. Lobão, B. Navarro, S. Marques, M. Parreira, E. Pedroso Internal Medicine, Centro Hospitalar de Setúbal, E.P.E. — Hospital de S. Bernardo, Setúbal, Portugal
Catastrophic antiphospholipid syndrome (CAPS) is a rare autoimmune disease that is associated with high mortality that can reach 50%. In most cases there is an underlying causative event such as an infection or inflammation. The authors report the case of a 34-year-old woman who was recently diagnosed with probable CAPS. Although she had a progressive and almost complete recovery, she developed extensive distal necrotic lesions in both feet that she refused being amputated. Two weeks after discharge, the patient was readmitted to the emergency room after being found unconscious at home. At admission, she wasn't alert, only responsive to painful stimuli, with left hemiplegia, and total gaze paralysis with gaze fixed to the right. She also maintained the feet lesions, with no evolution or external infection signs. Cranioencephalic computed tomography (CT) revealed a large hypodensity in the middle cerebral artery territory consistent with a stroke, despite being under warfarin therapy with international normalized ratio (INR) within the therapeutic range. The laboratory exams revealed elevation of the leukocyte count and C-reactive protein and methicillinresistant Staphylococcus aureus was isolated in the blood cultures. It was decided to maintain warfarin therapy with a target INR of 2.0–3.0, to which was added aspirin (100 mg daily). The patient also started treatment with vancomycin plus gentamicin. Fibrinolysis treatment was not considered due to the elevated INR. Despite an early significant recovery, a week after admission there was a clinical deterioration with a sudden drop in the level of consciousness followed by periods of bradypnea. Cranioencephalic CT was repeated and revealed the presence of subarachnoid hemorrhage. The neurologic examination showed no
Abstracts
clinical presentation, 86.04% of patients showed epistaxis and 81.47% telangiectases (no significative differences considering genetic type for both cases), 42.80% presented with pulmonary arteriovenous malformations (significative higher prevalence in HHT1 patients), 9.22% had brain arteriovenous malformations (significative higuer in HHT1) and 62.73% showed liver infection (significative higuer in HHT2). Discussion: Prevalence of HHT in Spain is calculated to be in the highest levels compared with international series, however it remains in the range of rare diseases. As in other mediterranean countries, mutations in ALK1 (HHT2) seem to be more frequent. Percentages of epistaxis are slightly lower probably due to a younger population. Pulmonary and liver infections are in the same range of other series and although neurological screening is always performed, brain involvement is much lower.
doi:10.1016/j.ejim.2013.08.472
ID: 385 Forearm ischemic exercise test (FIET). Experience in an Internal Medicine Department C. Cardozo, J.C. Milisenda, P.J. Moreno-Lozano, V. Lazo, A. Jérez, R.A. Losno, J.M. Grau Muscle Research Unit, Internal Medicine Department, Hospital Clinic, CIBERER, Barcelona, Spain
Objective: The objective of this study is to describe the patients's characteristics and the diagnosis we made during a ten-year period performing the FIET in a Muscle Research Unit from an Internal Medicine Department. Methods: A total of 107 patients were evaluated during a ten-year period. The main (non-excluding) complaints of such patients were: cramps, exercise intolerance, abnormal fatigue, iterative rhabdomyolysis or unexplained hyperCKemia. In some cases an additional muscle biopsy was also performed in order to confirm or to rule out the suspected disease. A descriptive analysis of the results was performed. Results: In this 10-year period, 56% of the patients were male, with a mean age of 41.7 (range 13– 72 years). The most frequent symptoms were exercise intolerance and abnormal fatigue. From 107 patients, in 29 (27.1%) pathological curves were obtained: 9 patients received the diagnosis of McArdle's disease, 17 of Myoadenylate deaminase deficiency and 3 cases nonMcArdle glucogenosis (phosphofructokinase deficiency one case). Both curves were plane only in 8 cases, representing a non-proper exercise in the test. In any case, no complication was observed during the test. Conclusions: FIET was a safe and very useful test, and it allowed us to achieve a diagnosis of metabolic myopathy in 27.1% of the tested patients. FIET is a test that must be performed when a metabolic myopathy is suspected. It's important to stand out that, in some patients, it was no longer necessary to perform a muscle biopsy because of the results in the FIET. doi:10.1016/j.ejim.2013.08.473
ID: 429 A delayed diagnosis of tuberous sclerosis C. Oliveira, R. Real, G. Nadais, C. Garret Neurology, Centro Hospitalar São João, Porto, Portugal
Introduction: Tuberous sclerosis is an autosomal dominant genetic disorder with multisystem clinical manifestations. The expanded
phenotype is now called the tuberous sclerosis complex (TSC), to emphasize the multiorgan involvement, characterized by the formation of hamartomatous lesions in the brain, heart, skin, kidney, lung and other organs. The incidence is approximately 1:6000 newborns and the diagnosis typically occurs during childhood, with most patients presenting with seizures or cutaneous involvement. Vascular manifestations of TS are now being more frequently acknowledged, and although significant vascular lesions are rare, they can be life threatening. Because of the wide range of organs affected, a multidisciplinary team approach is ideal and produces the best outcomes for patients. Treatment options for TSC have been limited, focusing primarily on the management of symptoms. Case report: A 38-yearold woman was referred to the Neurology clinic due to a family history of TSC (the daughter had been diagnosed with TSC at the age of 8 months, in the context of epilepsy) and suspicious skin lesions. The patient denied a history of seizures or other neurological symptoms, although she reported a mild learning disability in school. The patient had a background of arterial hypertension and aortic arch aneurysm that had been surgically corrected two years before. On physical examination, several skin lesions were present (hypomelanotic macules, angiofibromas and hyperpigmented nevus) on the face, neck, legs and arms; according to the patient, these lesions had been present since childhood. The neurological examination was unremarkable. The brain MRI revealed the presence of cortical and subcortical tubers, a number of small subependymal nodules in the walls of the lateral ventricles and a nodular lesion with intense gadolinium enhancement located on the left lateral ventricle near the foramen of Monro, suggestive of a subependymal giant cell astrocytoma. The renal ultrasound showed multiple hyperechoic nodular formations, likely angiomyolipomas. Abdominal MRI further identified multiple angiolipomas and cystic formations in the kidney, and a liver hemangioma. The chest CT did not show significant changes in lung interstitium, only ground-glass opacities in the middle lobe adjacent to the large fissure. An ophthalmological evaluation excluded retinal lesions and a dentistry workup revealed gingival fibromas and a tongue lesion that the patient refused to biopsy. Conclusions: The patient fulfills clinical criteria for definite TSC. Although the patient has multisystem involvement, the diagnosis was delayed until adulthood, a fact that underscores the importance of recognizing the full spectrum of signs and symptoms of this condition. Also, this case is atypical for its significant vascular involvement (aortic arch aneurysm), which is a rare manifestation of TSC, especially in adults. To conclude, it is important that physicians recognize the full spectrum of manifestations of TSC, including the most unusual, so not to miss the diagnosis. doi:10.1016/j.ejim.2013.08.474
ID: 436 Pediatric eosinophilic fasciitis: About 2 cases R. Lihioua, O. Harzallaha, A. Boukera, A. Jelladb, S. Hammamic, M. Kechidaa, S. Mahjouba a
Internal Medicine Department, Fattouma Bourguiba Hospital, Monastir, Tunisia b Rehabilitation and Physical Therapy Department, Fattouma Bourguiba Hospital, Monastir, Tunisia c Pediatrics Department, Fattouma Bourguiba Hospital, Monastir, Tunisia
Introduction: Eosinophilic fasciitis is an uncommon entity characterized by a scleroderma-like induration of the extremities skin and connective tissue and by blood eosinophilia. This singular syndrome is very rare in children with almost 30 cases described in literature. Methods: We report two cases of childhood eosinophilic fasciitis.
Abstracts
Results: Case report 1: A 13 year old girl was seen for skin induration of the abdomen and limbs and massive weight loss. Physical examination noted a limited abduction of shoulders and irreducible flexion contracture of knees and elbows. Laboratory tests revealed eosinophilia at 1700 E/mm3 and hypergammaglobulinemia. A deep tissue biopsy noted a dermal thickening and fibrosis with perivascular nonspecific inflammatory infiltration, a fibrotic fascia with abundant infiltration by lymphocytes, plasma cells and numerous eosinophils. Muscular specimen shows no abnormalities. Magnetic resonance imaging showed fasciitis involving the different muscular compartments of the thigh predominant in the posterior compartment associated to muscular atrophy without significant anomalies of intra-muscular or subcutaneous fat signal intensity. Our two patients were treated with prednisone (1 mg/kg/day) and physical therapy with insufficient response. Methotrexate was associated at 10 mg/week in addition to prolonged physical rehabilitation. The follow-up was marked by partial improvement of skin induration and joint stiffness. Case report 2: A 15 year old girl with a history of Hashimoto disease was seen for painless thickening of legs and forearms skin with joint stiffness and proximal muscular deficit. Physical examination noted also flexion contractures of the fingers and muscular atrophy of triceps and quadriceps. Laboratory testing revealed a peripheral eosinophilia at 1100 E/mm3, hypergammaglobulinemia, anemia and inflammatory biologic syndrome. Muscular enzymes were at normal level. A deep tissue biopsy revealed subcutaneous nonspecific inflammatory infiltration with fibrosis of the derma. Muscular biopsy showed a nonspecific myositis. Magnetic resonance imaging redescribed myositis involving pelvic muscles and fasciitis of the muscular lodges at the thigh backside. The evolution was characterized by a steroid's resistance indicating the association of methotrexate (12 mg per week). Conclusion: Eosinophilic fasciitis is an uncommon disease in childhood that must be considered in any patient with skin induration and painless joint contractures. The majority of patients are steroid responsive, but recourse to more aggressive treatment may be necessary. Interdisciplinary approach, including physical therapy, is crucial to prevent long-term morbidity. doi:10.1016/j.ejim.2013.08.475
ID: 440 Diagnosis of Erdheim–Chester disease following the workup of multiple falls I.R. Bistreanua, T. Nguyenb, S. Daensc, R. Attoua a
Internal Medicine Department, Brugman University Hospital, Brussels, Belgium b Internal Medicine Department, Erasme University Hospital, Brussels, Belgium c Rheumatology Department, Brugman University Hospital, Brussels, Belgium
Observation: A 65 year old woman presents to the Emergency Department for multiple falls and she is complaining of right ankle and left shoulder pain. She has had lower limb pain and gait difficulties for a few months. Recent memory losses were reported by family members. Past medical history includes appendectomy and cholecystectomy. X-rays reveal a left trochiter fracture and an old fracture of the proximal phalanx of the third right toe. Management is conservatory. The right tibia x-ray also demonstrates an osseous condensation in the medullo-metaphyseal region that warrants further investigation. Bone MRI shows heterogeneous focal lesions in the femurs and tibias. Whole body MRI is performed and shows also medullary lesions of both humerus without involvement of the spine and the pelvis. The distribution of lesions is suggestive of Erdheim–
e185
Chester disease. A bone biopsy is performed and confirms the diagnosis of Erdheim–Chester histiocytosis. Cerebral MRI and chest CT find disseminated lesions in the pons, cerebellum and pulmonary infiltrates, respectively. With such disseminated disease, the therapeutic decision is to start the interferon alpha. Discussion: We report a case of Erdheim–Chester disease, a rare non-Langerhans' cell histiocytosis, with characteristic radiological and histological features. The most common characteristic is bilateral and symmetric osteosclerotic lesions in long bones. Usually, patients complain of bone pain, as in our case. Interestingly, our patient initially consulted because of multiple falls and gait difficulties. Only several hundred cases have been reported in the medical literature. About half of the patients have extraskeletal manifestations and the prognosis depends on it. Imaging and clinical biology assess the extent of the disease. Neurological involvement includes ataxia, paresis, and diabetes insipidus. The differential diagnosis includes Langerhans' cell histiocytosis, metabolic disorders and malignancies. Treatment of Erdheim–Chester disease must be decided on a case-to-case basis taking into account patient's symptoms. Interpheron is the first-intention therapy, but corticosteroids, chemotherapy, surgical resection and radiotherapy have been used also. Conclusion: Erdheim–Chester disease is a rare systemic nonLangerhans' cell histiocytosis of the middle-aged adult. Bone pain is the most frequent of all symptoms and mainly affects the lower limbs, knees and ankles. The combination of multisystemic symptoms with typical radiological findings can direct the diagnosis. However, a histological examination is crucial for the diagnosis of Erdheim–Chester disease. doi:10.1016/j.ejim.2013.08.476
ID: 483 Catastrophic antiphospholipid syndrome: The aftermath H. Casimiro, P. Carreira, B. Lobão, B. Navarro, S. Marques, M. Parreira, E. Pedroso Internal Medicine, Centro Hospitalar de Setúbal, E.P.E. — Hospital de S. Bernardo, Setúbal, Portugal
Catastrophic antiphospholipid syndrome (CAPS) is a rare autoimmune disease that is associated with high mortality that can reach 50%. In most cases there is an underlying causative event such as an infection or inflammation. The authors report the case of a 34-year-old woman who was recently diagnosed with probable CAPS. Although she had a progressive and almost complete recovery, she developed extensive distal necrotic lesions in both feet that she refused being amputated. Two weeks after discharge, the patient was readmitted to the emergency room after being found unconscious at home. At admission, she wasn't alert, only responsive to painful stimuli, with left hemiplegia, and total gaze paralysis with gaze fixed to the right. She also maintained the feet lesions, with no evolution or external infection signs. Cranioencephalic computed tomography (CT) revealed a large hypodensity in the middle cerebral artery territory consistent with a stroke, despite being under warfarin therapy with international normalized ratio (INR) within the therapeutic range. The laboratory exams revealed elevation of the leukocyte count and C-reactive protein and methicillinresistant Staphylococcus aureus was isolated in the blood cultures. It was decided to maintain warfarin therapy with a target INR of 2.0–3.0, to which was added aspirin (100 mg daily). The patient also started treatment with vancomycin plus gentamicin. Fibrinolysis treatment was not considered due to the elevated INR. Despite an early significant recovery, a week after admission there was a clinical deterioration with a sudden drop in the level of consciousness followed by periods of bradypnea. Cranioencephalic CT was repeated and revealed the presence of subarachnoid hemorrhage. The neurologic examination showed no