Case Reports / Journal of Clinical Neuroscience 21 (2014) 699–701
previous reports. On T2-weighted images, low signal intensity (SI) areas correspond to abundant collagen fibers. High SI areas showing marked enhancement on contrast T1-weighted images correspond to areas consisting of fibroblasts and loose collagen fibers. On T1-weighted images, low SI areas represent areas with low cellularity and abundant collagen fibers. These reports suggest that MRI findings vary among and within individual tumors because of the variable cellularity [7,8]. Histopathological differentials include spindle cell tumors of the bone-like desmoid tumor, low grade fibrosarcoma, fibrous dysplasia, low grade intra-osseous osteosarcoma and intra-osseous meningioma [9]. In comparison to DF, desmoid are more cellular, infiltrative, and less circumscribed with a sprinkling of lymphocytes. Low grade fibrosarcoma show more cellularity with a herring bone arrangement of cells, less collagen and some mitotic activity. Fibrous dysplasia on other hand show cellular spindle cell proliferation with metaplastic bone formation without osteoblastic rimming. Intra-osseous meningioma show meningothelial cells with whorl formation and psammoma bodies. Low grade osteosarcoma is composed of hypocellular spindle cell proliferation with well formed trabeculae of bone. In the present patient only a focal presence of bone formation was seen with the predominant component being collagenous stroma and hypocellular areas composed of spindle and stellate cells unlike fibrous dysplasia or low grade osteosarcoma. The lesion is considered to be neoplastic in origin because of its association with 11q12 breakpoints, lack of inciting events, and the initiation of reactive fibrous proliferation [10]. Bridge et al. [11] in a review of 22 specimens from 19 patients diagnosed with desmoid tumor, DF, periosteal desmoid tumor, osteofibrous dysplasia, or fibrous dysplasia, performed cytogenetic analysis of short-term cultures and bi-color fluorescence in situ hybridization of cytological touch preparations or paraffin-embedded tissue with centromeric probes for chromosomes 8 and 20. They found that trisomy 8 and trisomy 20 are non-random aberrations in histologically similar, but clinically distinct, benign fibrous lesions of bone.
699
To conclude, we describe to our knowledge the first patient with giant DF of the calvarium in a 20-year-old woman whose tumor first appeared at the age of 5 years. Although extremely rare, DF should be considered in the differential diagnosis of large calvarial lesions. A staged surgical approach with a multi-disciplinary team including plastic, facial and reconstructive surgeons should be considered in giant DF. Conflicts of interest/disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Nielsen GP, O’Connell JX, Dickersin GR, et al. Collagenous fibroma (desmoplastic fibroblastoma): a report of seven cases. Mod Pathol 1996;9:781–5. [2] Beggs I, Salter DS, Dorfman HD. Synovial desmoplastic fibroblastoma of hip joint with bone erosion. Skeletal Radiol 1999;28:402–6. [3] Nishio J, Iwasaki H, Nishijima T, et al. Collagenous fibroma (desmoplastic fibroblastoma) of the finger in a child. Pathol Int 2002;52:322–5. [4] Fong F, Odell E, Simo R. Collagenous fibroma (desmoplastic fibroblastoma) of the neck presenting with neurological symptoms. Head Neck Pathol 2009;3:47–50. [5] Osipov V, Carrera GF. Collagenous fibroma (desmoplastic fibroblastoma) with vertebral body erosion. Sarcoma 2009;2009:682687. [6] Merriman DJ, Deavers MT, Czerniak BA, et al. Massive desmoplastic fibroblastoma with scapular invasion. Orthopedics 2010;33. http:// dx.doi.org/10.3928/01477447-20100625-23. [7] Shuto R, Kiyosue H, Hori Y, et al. CT and MR imaging of desmoplastic fibroblastoma. Eur Radiol 2002;12:2474–6. [8] Yamamoto A, Abe S, Imamura T, et al. Three cases of collagenous fibroma with rim enhancement on postcontrast T1-weighted images with fat suppression. Skeletal Radiol 2013;42:141–6. [9] Miettinen M, Fetsch JF. Collagenous fibroma (desmoplastic fibroblastoma): a clinicopathologic analysis of 63 cases of a distinctive soft tissue lesion with stellate-shaped fibroblasts. Hum Pathol 1998;29:676–82. [10] Bernal K, Nelson M, Neff JR, et al. Translocation (2;11)(q31;q12) is recurrent in collagenous fibroma (desmoplastic fibroblastoma). Cancer Genet Cytogenet 2004;149:161–3. [11] Bridge JA, Swarts SJ, Buresh C, et al. Trisomies 8 and 20 characterize a subgroup of benign fibrous lesions arising in both soft tissue and bone. Am J Pathol 1999;154:729–33.
http://dx.doi.org/10.1016/j.jocn.2013.07.032
Brainstem encephalitis caused by Coxiella burnetii Jung-Ah Lim a, Jeong-Min Kim b, Soon-Tae Lee a, Keun-Hwa Jung a, Young Su Kim c, Sang Kun Lee a, Kon Chu a,⇑ a
Department of Neurology, Seoul National University Hospital, 101 Daehangno, Chongro-Gu, 110-744 Seoul, South Korea Department of Neurology, Chung-Ang University Medical Center, Seoul, South Korea c Department of Neurology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, South Korea b
a r t i c l e
i n f o
Article history: Received 1 May 2013 Accepted 26 May 2013
Keywords: Brainstem encephalitis Coxiella burnetii Q fever
a b s t r a c t Coxiella burnetii is the causative agent of Q fever, which rarely causes neurologic symptoms. We describe a patient who presented with progressive sensory changes, dizziness, and motor weakness. The cerebrospinal fluid revealed pleocytosis, and MRI indicated the presence of a diffuse brain stem lesion. An indirect immunofluorescence test revealed antibodies against C. burnetii. The symptoms improved with antibiotics against C. burnetii. Q fever should be considered as a possible etiology of brainstem encephalitis. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction ⇑ Corresponding author. Tel.: +82 2 2072 1878; fax: +82 2 3672 4949. E-mail address: [email protected] (K. Chu).
Q fever is a disease that is caused by Coxiella burnetii infection. A flu-like illness is the most common presentation, and pneumonia,
700
Case Reports / Journal of Clinical Neuroscience 21 (2014) 699–701
hepatitis, and endocarditis may also occur [1]. Although neurological manifestation is rare, various neurological complications including meningitis, encephalitis, encephalomyelitis and peripheral neuropathy have been reported [1–3]. However, brainstem involvement or rhombencephalitis has not been reported to our knowledge. We report a patient with brainstem encephalitis that was found to be associated with Q fever. 2. Case report A 29-year-old man was referred to our hospital because of right leg weakness that had started 5 days previously. Two months earlier he had developed parestheisa of the right leg. One month later, he began to experience vertigo, hiccups, nausea and was vomiting. Ten days later, the parestheisa progressed to his right arm and left face. His previous medial history was unremarkable. On admission, his vital signs were stable and he had a normal body temperature. Neurologic examination revealed horizontal diplopia with left eye abduction limitation combined with horizontal and vertical gaze palsy that was accompanied by nystagmus, hemiparesis and paresthesia of the right extremities, and left face hypesthesia. Laboratory examination indicated elevated C-reactive protein levels (14.62 mg/dL; normal range, 0–0.5), but otherwise the results of the examination were unremarkable. Brain MRI revealed a high signal intensity lesion involving the bilateral lower pons, medulla, and cervicomedullary junction on both the diffusion weighted images and fluid attenuation inversion recovery (FLAIR) images (Fig. 1A–C).
Three days after admission the patient deteriorated rapidly with quadriplegia and respiratory failure. He was transferred to the intensive care unit and mechanical ventilation was initiated. On post-admission day 7, lumbar puncture analysis revealed elevated leukocytes (18 leukocytes/mm [3], lymphocyte-dominant) and normal protein (33 mg/dL) and glucose (87 mg/dL). Repeat MRI showed an increase in the signal change in the brainstem (Fig. 1D–F). The patient began treatment with empirical intravenous antibiotics (vancomycin, ampicillin and ceftriaxone) and an antiviral agent (acyclovir) to treat the presumed infectious encephalitis. The patient was also given corticosteroids. Blood and cerebrospinal fluid (CSF) cultures were negative. Polymerase chain reactions using CSF were negative for herpes simplex virus, Epstein-Barr virus, cytomegalovirus, varicella zoster virus and enterovirus. Serologic tests for the above viruses and Mycoplasma, Borrelia, Rickettsia, Brucella, Leptospira, Legionella, measles, mumps, rubella and Japanese encephalitis were all negative. Specific antibodies to C. burnetii were detected using an indirect immunofluorescence assay with serum immunoglobulin (Ig) G titer to phase II antigen 1:512 and an IgM titer less than 1:16. Antinuclear antibody, anticardiolipin antibody and anticoagulant were unremarkable. The echocardiogram was normal and no cardiac effects due to C. burnetii were observed. Levofloxacin, an agent to be active against C. burnetii, was administered for 4 weeks, followed by doxycyclin, and the neurological symptoms slowly improved. MRI 18 months after initial symptom onset showed that the extent of the lesion had decreased (Fig. 1G–I), but the antibodies to C.
Fig. 1. Axial T2-weighted fluid attenuated inversion recovery brain MRI showing an asymmetrical high signal intensity lesion involving the lower pons, medulla and cervicomedullary junction in initial images (A–C). The lesion increased when the patient’s neurological status deteriorated after admission (D–F). Follow-up images 2 years after antibiotic treatment showing a decrease in the size of the lesion (G–I).
Case Reports / Journal of Clinical Neuroscience 21 (2014) 701–704
burnetii against phase II were still high (1:128). Antibiotics were continued for another 6 months and the serum IgG titer decreased to 1:32. Most of the neurological symptoms resolved except for intermittent diplopia. 3. Discussion Brainstem encephalitis can be caused by a group of inflammatory diseases that result in cranial nerve paresis, ataxia, and long tract signs with CSF pleocytosis, sometimes with abnormal imaging findings. The etiologic categories include infectious, autoimmune, and paraneoplastic causes [4,5]. We diagnosed our patient based on clinical manifestations and laboratory findings. The patient had brainstem manifestations. The CSF showed pleocytosis, and MRI revealed an increased signal intensity lesion in the brainstem on T2-weighted and FLAIR images. Multiple tests for detecting diverse pathogens were negative except for C. burnetii. After initiating antibiotic treatment against C. burnetii, the patient’s neurologic symptoms slowly improved. Neurological complications in the course of Q fever are rare. In a report of 1269 patients with acute Q fever, neurologic involvement was documented in only 29 patients (2.2%) [2]. In another series of 121 patients with Q fever, 40.5% suffered from headaches, 4.1% complained of confusion, and meningitis was confirmed in 0.8% [3]. Meningitis and meningoencephalitis are the most common clinical syndromes in regards to the neurologic effects of Q fever, but brain imaging findings are usually normal or nonspecific, such as diffuse cerebral edema or bilateral periventricular edema [1,6,7]. One patient with acute cerebellitis caused by C. burnetii was previously reported to have a high signal intensity on T2-weighted images in the right cerebellar hemisphere [8]. This report describes brainstem encephalitis presumably caused by Coxiella burnettii in an otherwise healthy man. The
701
patient had a positive response to treatment with antibiotics against C. burnetii, which was administered based on the serologic tests. To the best of our knowledge, this is the first reported patient with brainstem encephalitis associated with Q fever. Brain MRI showed a characteristic asymmetrical high signal intensity brainstem lesion involving the pons, medulla, and upper cervical cord. Therefore, Q fever should be considered a possible etiology of brainstem encephalitis. Conflicts of interest/disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. Acknowledgment This study was supported by Ministry of Health and Welfare, Republic of Korea (A111393–1202–0000100). References [1] Parker N, Barralet J, Bell A. Q fever. Lancet 2006;367:679–88. [2] Bernit E, Pouget J, Janbon F, et al. Neurological involvement in acute Q fever: a report of 29 cases and review of the literature. Arch Intern Med 2002;162:693–700. [3] Kofteridis DP, Mazokopakis EE, Tselentis Y, et al. Neurological complications of acute Q fever infection. Eur J Epidemiol 2004;19:1051–4. [4] Jubelt B, Mihai C, Li TM, et al. Rhombencephalitis/brainstem encephalitis. Curr Neurol Neurosci Rep 2011;11:543–52. [5] Moragas M, Martinez-Yelamos S, Majos C, et al. Rhombencephalitis: a series of 97 patients. Medicine 2011;90:256–61. [6] Schuil J, Richardus JH, Baarsma GS, et al. Q fever as a possible cause of bilateral optic neuritis. Br J Ophthalmol 1985;69:580–3. [7] Gomez-Aranda F, Pachon Diaz J, Romero Acebal M, et al. Computed tomographic brain scan findings in Q fever encephalitis. Neuroradiology 1984;26:329–32. [8] Sawaishi Y, Takahashi I, Hirayama Y, et al. Acute cerebellitis caused by Coxiella burnetii. Ann Neurol 1999;45:124–7.
http://dx.doi.org/10.1016/j.jocn.2013.05.021
Temozolomide-related acute lymphoblastic leukemia with translocation (4;11)(q21;q23) in a glioblastoma patient Kuan-Nien Chou a, Yu-chieh Lin b, Ming-Ying Liu a, Ping-Ying Chang c,⇑ a
Department of Neurological Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, ROC Department of Pathology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, ROC c Department of Internal Medicine, Division of Hematology & Oncology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, ROC b
a r t i c l e
i n f o
Article history: Received 26 April 2013 Accepted 12 July 2013
Keywords: Glioblastoma multiforme Secondary acute lymphoblastic leukemia Temozolomide Therapy-related acute lymphoblastic leukemia
a b s t r a c t Temozolomide (TMZ), an alkylating agent, is widely used for treating high-grade gliomas. TMZ has been reported to cause secondary myelodysplastic syndrome and acute myeloid leukemia. However, TMZrelated acute lymphoblastic leukemia is rare. Here we describe a 54-year-old woman with glioblastoma multiforme, who developed precursor-B acute lymphoblastic leukemia with translocation (4;11)(q21;q23) after 15 months of TMZ treatment. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction ⇑ Corresponding author. Tel.: +886 8792 7177; fax: +886 8792 7178. E-mail address: [email protected] (P.-Y. Chang).
Temozolomide (TMZ) has been increasingly reported to cause secondary myelodysplastic syndrome (MDS) and acute myeloid
previous reports. On T2-weighted images, low signal intensity (SI) areas correspond to abundant collagen fibers. High SI areas showing marked enhancement on contrast T1-weighted images correspond to areas consisting of fibroblasts and loose collagen fibers. On T1-weighted images, low SI areas represent areas with low cellularity and abundant collagen fibers. These reports suggest that MRI findings vary among and within individual tumors because of the variable cellularity [7,8]. Histopathological differentials include spindle cell tumors of the bone-like desmoid tumor, low grade fibrosarcoma, fibrous dysplasia, low grade intra-osseous osteosarcoma and intra-osseous meningioma [9]. In comparison to DF, desmoid are more cellular, infiltrative, and less circumscribed with a sprinkling of lymphocytes. Low grade fibrosarcoma show more cellularity with a herring bone arrangement of cells, less collagen and some mitotic activity. Fibrous dysplasia on other hand show cellular spindle cell proliferation with metaplastic bone formation without osteoblastic rimming. Intra-osseous meningioma show meningothelial cells with whorl formation and psammoma bodies. Low grade osteosarcoma is composed of hypocellular spindle cell proliferation with well formed trabeculae of bone. In the present patient only a focal presence of bone formation was seen with the predominant component being collagenous stroma and hypocellular areas composed of spindle and stellate cells unlike fibrous dysplasia or low grade osteosarcoma. The lesion is considered to be neoplastic in origin because of its association with 11q12 breakpoints, lack of inciting events, and the initiation of reactive fibrous proliferation [10]. Bridge et al. [11] in a review of 22 specimens from 19 patients diagnosed with desmoid tumor, DF, periosteal desmoid tumor, osteofibrous dysplasia, or fibrous dysplasia, performed cytogenetic analysis of short-term cultures and bi-color fluorescence in situ hybridization of cytological touch preparations or paraffin-embedded tissue with centromeric probes for chromosomes 8 and 20. They found that trisomy 8 and trisomy 20 are non-random aberrations in histologically similar, but clinically distinct, benign fibrous lesions of bone.
699
To conclude, we describe to our knowledge the first patient with giant DF of the calvarium in a 20-year-old woman whose tumor first appeared at the age of 5 years. Although extremely rare, DF should be considered in the differential diagnosis of large calvarial lesions. A staged surgical approach with a multi-disciplinary team including plastic, facial and reconstructive surgeons should be considered in giant DF. Conflicts of interest/disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Nielsen GP, O’Connell JX, Dickersin GR, et al. Collagenous fibroma (desmoplastic fibroblastoma): a report of seven cases. Mod Pathol 1996;9:781–5. [2] Beggs I, Salter DS, Dorfman HD. Synovial desmoplastic fibroblastoma of hip joint with bone erosion. Skeletal Radiol 1999;28:402–6. [3] Nishio J, Iwasaki H, Nishijima T, et al. Collagenous fibroma (desmoplastic fibroblastoma) of the finger in a child. Pathol Int 2002;52:322–5. [4] Fong F, Odell E, Simo R. Collagenous fibroma (desmoplastic fibroblastoma) of the neck presenting with neurological symptoms. Head Neck Pathol 2009;3:47–50. [5] Osipov V, Carrera GF. Collagenous fibroma (desmoplastic fibroblastoma) with vertebral body erosion. Sarcoma 2009;2009:682687. [6] Merriman DJ, Deavers MT, Czerniak BA, et al. Massive desmoplastic fibroblastoma with scapular invasion. Orthopedics 2010;33. http:// dx.doi.org/10.3928/01477447-20100625-23. [7] Shuto R, Kiyosue H, Hori Y, et al. CT and MR imaging of desmoplastic fibroblastoma. Eur Radiol 2002;12:2474–6. [8] Yamamoto A, Abe S, Imamura T, et al. Three cases of collagenous fibroma with rim enhancement on postcontrast T1-weighted images with fat suppression. Skeletal Radiol 2013;42:141–6. [9] Miettinen M, Fetsch JF. Collagenous fibroma (desmoplastic fibroblastoma): a clinicopathologic analysis of 63 cases of a distinctive soft tissue lesion with stellate-shaped fibroblasts. Hum Pathol 1998;29:676–82. [10] Bernal K, Nelson M, Neff JR, et al. Translocation (2;11)(q31;q12) is recurrent in collagenous fibroma (desmoplastic fibroblastoma). Cancer Genet Cytogenet 2004;149:161–3. [11] Bridge JA, Swarts SJ, Buresh C, et al. Trisomies 8 and 20 characterize a subgroup of benign fibrous lesions arising in both soft tissue and bone. Am J Pathol 1999;154:729–33.
http://dx.doi.org/10.1016/j.jocn.2013.07.032
Brainstem encephalitis caused by Coxiella burnetii Jung-Ah Lim a, Jeong-Min Kim b, Soon-Tae Lee a, Keun-Hwa Jung a, Young Su Kim c, Sang Kun Lee a, Kon Chu a,⇑ a
Department of Neurology, Seoul National University Hospital, 101 Daehangno, Chongro-Gu, 110-744 Seoul, South Korea Department of Neurology, Chung-Ang University Medical Center, Seoul, South Korea c Department of Neurology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, South Korea b
a r t i c l e
i n f o
Article history: Received 1 May 2013 Accepted 26 May 2013
Keywords: Brainstem encephalitis Coxiella burnetii Q fever
a b s t r a c t Coxiella burnetii is the causative agent of Q fever, which rarely causes neurologic symptoms. We describe a patient who presented with progressive sensory changes, dizziness, and motor weakness. The cerebrospinal fluid revealed pleocytosis, and MRI indicated the presence of a diffuse brain stem lesion. An indirect immunofluorescence test revealed antibodies against C. burnetii. The symptoms improved with antibiotics against C. burnetii. Q fever should be considered as a possible etiology of brainstem encephalitis. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction ⇑ Corresponding author. Tel.: +82 2 2072 1878; fax: +82 2 3672 4949. E-mail address: [email protected] (K. Chu).
Q fever is a disease that is caused by Coxiella burnetii infection. A flu-like illness is the most common presentation, and pneumonia,
700
Case Reports / Journal of Clinical Neuroscience 21 (2014) 699–701
hepatitis, and endocarditis may also occur [1]. Although neurological manifestation is rare, various neurological complications including meningitis, encephalitis, encephalomyelitis and peripheral neuropathy have been reported [1–3]. However, brainstem involvement or rhombencephalitis has not been reported to our knowledge. We report a patient with brainstem encephalitis that was found to be associated with Q fever. 2. Case report A 29-year-old man was referred to our hospital because of right leg weakness that had started 5 days previously. Two months earlier he had developed parestheisa of the right leg. One month later, he began to experience vertigo, hiccups, nausea and was vomiting. Ten days later, the parestheisa progressed to his right arm and left face. His previous medial history was unremarkable. On admission, his vital signs were stable and he had a normal body temperature. Neurologic examination revealed horizontal diplopia with left eye abduction limitation combined with horizontal and vertical gaze palsy that was accompanied by nystagmus, hemiparesis and paresthesia of the right extremities, and left face hypesthesia. Laboratory examination indicated elevated C-reactive protein levels (14.62 mg/dL; normal range, 0–0.5), but otherwise the results of the examination were unremarkable. Brain MRI revealed a high signal intensity lesion involving the bilateral lower pons, medulla, and cervicomedullary junction on both the diffusion weighted images and fluid attenuation inversion recovery (FLAIR) images (Fig. 1A–C).
Three days after admission the patient deteriorated rapidly with quadriplegia and respiratory failure. He was transferred to the intensive care unit and mechanical ventilation was initiated. On post-admission day 7, lumbar puncture analysis revealed elevated leukocytes (18 leukocytes/mm [3], lymphocyte-dominant) and normal protein (33 mg/dL) and glucose (87 mg/dL). Repeat MRI showed an increase in the signal change in the brainstem (Fig. 1D–F). The patient began treatment with empirical intravenous antibiotics (vancomycin, ampicillin and ceftriaxone) and an antiviral agent (acyclovir) to treat the presumed infectious encephalitis. The patient was also given corticosteroids. Blood and cerebrospinal fluid (CSF) cultures were negative. Polymerase chain reactions using CSF were negative for herpes simplex virus, Epstein-Barr virus, cytomegalovirus, varicella zoster virus and enterovirus. Serologic tests for the above viruses and Mycoplasma, Borrelia, Rickettsia, Brucella, Leptospira, Legionella, measles, mumps, rubella and Japanese encephalitis were all negative. Specific antibodies to C. burnetii were detected using an indirect immunofluorescence assay with serum immunoglobulin (Ig) G titer to phase II antigen 1:512 and an IgM titer less than 1:16. Antinuclear antibody, anticardiolipin antibody and anticoagulant were unremarkable. The echocardiogram was normal and no cardiac effects due to C. burnetii were observed. Levofloxacin, an agent to be active against C. burnetii, was administered for 4 weeks, followed by doxycyclin, and the neurological symptoms slowly improved. MRI 18 months after initial symptom onset showed that the extent of the lesion had decreased (Fig. 1G–I), but the antibodies to C.
Fig. 1. Axial T2-weighted fluid attenuated inversion recovery brain MRI showing an asymmetrical high signal intensity lesion involving the lower pons, medulla and cervicomedullary junction in initial images (A–C). The lesion increased when the patient’s neurological status deteriorated after admission (D–F). Follow-up images 2 years after antibiotic treatment showing a decrease in the size of the lesion (G–I).
Case Reports / Journal of Clinical Neuroscience 21 (2014) 701–704
burnetii against phase II were still high (1:128). Antibiotics were continued for another 6 months and the serum IgG titer decreased to 1:32. Most of the neurological symptoms resolved except for intermittent diplopia. 3. Discussion Brainstem encephalitis can be caused by a group of inflammatory diseases that result in cranial nerve paresis, ataxia, and long tract signs with CSF pleocytosis, sometimes with abnormal imaging findings. The etiologic categories include infectious, autoimmune, and paraneoplastic causes [4,5]. We diagnosed our patient based on clinical manifestations and laboratory findings. The patient had brainstem manifestations. The CSF showed pleocytosis, and MRI revealed an increased signal intensity lesion in the brainstem on T2-weighted and FLAIR images. Multiple tests for detecting diverse pathogens were negative except for C. burnetii. After initiating antibiotic treatment against C. burnetii, the patient’s neurologic symptoms slowly improved. Neurological complications in the course of Q fever are rare. In a report of 1269 patients with acute Q fever, neurologic involvement was documented in only 29 patients (2.2%) [2]. In another series of 121 patients with Q fever, 40.5% suffered from headaches, 4.1% complained of confusion, and meningitis was confirmed in 0.8% [3]. Meningitis and meningoencephalitis are the most common clinical syndromes in regards to the neurologic effects of Q fever, but brain imaging findings are usually normal or nonspecific, such as diffuse cerebral edema or bilateral periventricular edema [1,6,7]. One patient with acute cerebellitis caused by C. burnetii was previously reported to have a high signal intensity on T2-weighted images in the right cerebellar hemisphere [8]. This report describes brainstem encephalitis presumably caused by Coxiella burnettii in an otherwise healthy man. The
701
patient had a positive response to treatment with antibiotics against C. burnetii, which was administered based on the serologic tests. To the best of our knowledge, this is the first reported patient with brainstem encephalitis associated with Q fever. Brain MRI showed a characteristic asymmetrical high signal intensity brainstem lesion involving the pons, medulla, and upper cervical cord. Therefore, Q fever should be considered a possible etiology of brainstem encephalitis. Conflicts of interest/disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. Acknowledgment This study was supported by Ministry of Health and Welfare, Republic of Korea (A111393–1202–0000100). References [1] Parker N, Barralet J, Bell A. Q fever. Lancet 2006;367:679–88. [2] Bernit E, Pouget J, Janbon F, et al. Neurological involvement in acute Q fever: a report of 29 cases and review of the literature. Arch Intern Med 2002;162:693–700. [3] Kofteridis DP, Mazokopakis EE, Tselentis Y, et al. Neurological complications of acute Q fever infection. Eur J Epidemiol 2004;19:1051–4. [4] Jubelt B, Mihai C, Li TM, et al. Rhombencephalitis/brainstem encephalitis. Curr Neurol Neurosci Rep 2011;11:543–52. [5] Moragas M, Martinez-Yelamos S, Majos C, et al. Rhombencephalitis: a series of 97 patients. Medicine 2011;90:256–61. [6] Schuil J, Richardus JH, Baarsma GS, et al. Q fever as a possible cause of bilateral optic neuritis. Br J Ophthalmol 1985;69:580–3. [7] Gomez-Aranda F, Pachon Diaz J, Romero Acebal M, et al. Computed tomographic brain scan findings in Q fever encephalitis. Neuroradiology 1984;26:329–32. [8] Sawaishi Y, Takahashi I, Hirayama Y, et al. Acute cerebellitis caused by Coxiella burnetii. Ann Neurol 1999;45:124–7.
http://dx.doi.org/10.1016/j.jocn.2013.05.021
Temozolomide-related acute lymphoblastic leukemia with translocation (4;11)(q21;q23) in a glioblastoma patient Kuan-Nien Chou a, Yu-chieh Lin b, Ming-Ying Liu a, Ping-Ying Chang c,⇑ a
Department of Neurological Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, ROC Department of Pathology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, ROC c Department of Internal Medicine, Division of Hematology & Oncology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, ROC b
a r t i c l e
i n f o
Article history: Received 26 April 2013 Accepted 12 July 2013
Keywords: Glioblastoma multiforme Secondary acute lymphoblastic leukemia Temozolomide Therapy-related acute lymphoblastic leukemia
a b s t r a c t Temozolomide (TMZ), an alkylating agent, is widely used for treating high-grade gliomas. TMZ has been reported to cause secondary myelodysplastic syndrome and acute myeloid leukemia. However, TMZrelated acute lymphoblastic leukemia is rare. Here we describe a 54-year-old woman with glioblastoma multiforme, who developed precursor-B acute lymphoblastic leukemia with translocation (4;11)(q21;q23) after 15 months of TMZ treatment. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction ⇑ Corresponding author. Tel.: +886 8792 7177; fax: +886 8792 7178. E-mail address: [email protected] (P.-Y. Chang).
Temozolomide (TMZ) has been increasingly reported to cause secondary myelodysplastic syndrome (MDS) and acute myeloid
