CASE REPORT/CASE SERIES
Acute Longitudinal and Hemorrhagic Myelitis Caused by Varicella-Zoster Virus in an Immunocompetent Adolescent Yu Wang, MD, PhD, Chuan-Yong Yu, MD, Lin Huang, MD, Yuan-Yuan Han, MD, Yi-Min Liu, MD, and Jin Zhu, MD
Objectives: In immunocompetent person, varicella-zoster virus (VZV)-induced myelitis is rare and the lesion usually comprise focal spinal cord segment. VZV-induced hemorrhagic myelitis with lesions comprising longitudinal spinal cord has never been reported. Methods: We report a 15-year-old male patient who developed acute flaccid quadriplegia, days after a common cold. One week after the quadriplegia developed, he presented a typical herpes zoster on the lateral side of his left waist. Results: IgG antibody for VZV was detected in cerebrospinal fluid and the spinal cord magnetic resonance imaging findings were consistent with that of small vessel vasculitis. Treatment with acyclovir and corticosteroids resulted in no significant clinical improvement. Conclusions: In this report, we wanted to emphasize the hemorrhage, the extensiveness of inflammatory changes induced by VZV in spinal cord. It is suggested that VZV should be considered as a possible cause of a severe hemorrhagic myelitis even in immunocompetent adolescent. Key Words: varicella-zoster virus, myelitis, hemorrhage, immunocompetence, pathogenesis
(The Neurologist 2015;19:93–95)
T
he presentations of neurological complications of varicellazoster virus (VZV) infection or reactivation depend on the immune setting of the patients. Severe and diffuse multifocal lesions in the central nervous system (CNS) are usually observed in immunocompromised patients,1,2 in contrast, focal lesion, for example, transverse myelitis, usually in immunocompetent patients.3,4 Myelitis is rarely associated with VZV infection in immunocompetent patients and VZV-induced myelitic lesions comprising extensive spinal cord with hemorrhage has never been reported in immunocompetent patients. Treatment with high doses of steroids and acyclovir usually has significant recovery, though fatal cases have been reported.5 To the best of our knowledge, we describe for the first time a case of hemorrhagic myelitis with lesions comprising extensive spinal cord and this severe myelitis occurred before the appearance of cutaneous zoster rash. Combined From the Department of Neurology, The First Hospital of Anhui Medical University, Hefei, China. Supported in part by Natural Science grants from the National Natural Science Foundation of China (grant number 30970997) and from Natural Science Foundation of Anhui Province (grant number 09020103008). The authors declare no conflict of interest. Reprints: Yu Wang, MD, PhD, Department of Neurology, The First Hospital of Anhui Medical University, Jixi Road 218, Hefei 230022, China. E-mail: [email protected]. Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 1074-7931/15/1904-0093 DOI: 10.1097/NRL.0000000000000016
The Neurologist
Volume 19, Number 4, April 2015
treatment with high doses of steroids and acyclovir failed to achieve significant clinical improvement.
CLINICAL REPORT A 15-year-old, previously healthy boy was presented to our department with the complaints of acute and progressive paresthesia and weakness in both legs with the starting symptom of bilateral radiating pain band at T10 level for 1 day. Three days before the onset of this neurological symptom he had a common cold and fever. In a few days he became paraplegic with T4 sensory level and urinary incontinence. On admission, the neurological examination showed intact consciousness, cooperation, orientation, as well as normal cranial nerve and cerebellar function. Motor examination revealed severely reduced tone of lower limbs and mildly reduced tone of upper limbs. Power was grade 4/5 in upper limbs, and grade 0/5 in lower limbs. Deep tendon jerks and bilateral plantar responses were absent. Algesthesia examination showed diminished sensation to pain and light touch below the T2 dermatome. Other neurological examinations revealed no positive sign. Physical examination also revealed that the patient was eupneic, with heart rate at 82 bpm and blood pressure at 110/75 mm Hg. Abdomen observations showed distended with hydroaerial sounds. Lung examinations were uneventful. Cardiac auscultation revealed normal valve click. Laboratory findings for blood cell count, liver enzymes, BUN, Creactive protein, erythrocyte sedimentation rate, and urinalysis were normal. Serological tests were negative for hepatitis B and C, cytomegalovirus, herpes simplex virus, Epstein-Barr virus, PPD, and human immunodeficiency virus but positive in IgM for VZV. Lumbar puncture revealed crystal CSF with pressure of 110 mm H2O. CSF protein was 3.9 g/L (normal range, 0.15 to 0.45 g/L), glucose level 3.0 mmoL/L (normal range, 2.5 to 4.5 mmoL/L), and RBC count 150 cells/mL with 15 polymorphs and 2 lymphocytes. CSF viral antibody test revealed negative for all the familiar virus, including hepatitis B and C, cytomegalovirus, and Epstein-Barr virus, but positive in IgG for VZV. The serum/CSF ratio for anti-VZV IgG was reduced compared with that for total IgG as well as for albumin. An immediate magnetic resonance imaging (MRI) study showed multifocal low-signal intensity lesions throughout the long segments of the spinal cord (T1-T12) on T2-weighted (Fig. 1) and T1-weighted images. Apart from the multifocal low-signal intensity lesions, the spinal cord was swelling with mildly high-signal intensity on T2weighted images (Fig. 1) and mildly low-signal intensity on T1weighted images. Cranial MRI examination was normal. The patient was initially diagnosed with spinal cord hemorrhage from possible vascular malformation and treatment was started with 20% mannitol and hemostatic. At the seventh day after he was afflicted with paraplegia, he presented a typical herpes zoster on the lateral side of his left waist, which reached peak on the ninth day (Fig. 2). The diagnosis was then adjusted to acute longitudinal and hemorrhagic myelitis caused by VZV. Further inquiry of the past history indicated that he had varicella 10 years ago and had never accepted vaccination against varicella. He was treated with intravenous (IV) acyclovir 10 mg/kg/d divided by every 8 hours for 2 weeks and IV methylprednisolone 1.0 g every day for 5 days and subsequent IV dexamethasone 20 mg/d for 2 weeks. Within 6 months, he had not recovered from paraplegia, sensory deficits, and voiding dysfunction.
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The Neurologist
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Volume 19, Number 4, April 2015
FIGURE 1. T2-weighted MR images of the spinal cord. MRI shows extensive T2-hyperintense changes along the spinal cord from T1 level to T12. Multiple central cavitations (hemorrhage) indicated by the arrowheads can be observed along the longitudinal spinal cord. (C) is the axial image in section indicated by arrows in (A) and (B).
DISCUSSION We describe a longitudinal and hemorrhagic myelitis caused by VZV in an immunocompetent adolescent. Virological verification was provided by the detection of anti-VZV IgG antibody and intrathecal production of anti-VZV IgG antibody,6 which was indicated by the reduced serum/CSF ratios compared with normal serum/CSF ratios of total IgG and
FIGURE 2. Herpes zoster in the lumbar region.
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albumin in our patient. This report illustrates 3 notable features. First, it is the first case of myelitis with widespread multifocal hemorrhagic lesions due to VZV infection. Second, this disastrous myelitis was in an immunocompetent adolescent but not in an immunocompromised patient or in an elderly person. Third, the cutaneous herpes zoster appeared lagging behind the onset of myelitis in an immunocompetent patient. The pathogenesis of neurological complications associated with VZV infection is largely unclear. Direct viral invasion has been supposed in the pathogenesis of VZV myelitis, because VZV particles were found in glial cells. Hogan and Krigman7 presented evidence of direct invasion of VZV in a patient with herpes zoster transverse myelitis after a postmortem examination of the spinal cord. This supposed direct viral invasion pathogenesis is further supported by the clinical findings that VZV myelitis was usually monofocal lesions succeeding a zoster at the corresponding dermatome.3,8 In our case, the zoster dermatome was at T9-T10 levels of which the corresponding spinal cord segments were also most severely lesioned (biggest hemorrhagic lesion), and at the same levels the patient experienced the initial bilateral radiating pain band. Allergic mechanisms after VZV infection are also suggested.9 Pathologic examinations in a large series of patients with VZV myelitis showed that vasculitis, necrosis, and demyelination coexisted in the spinal cord.10 VZV infection can cause large vessel vasculitis as well as small vessel vasculitis. Large
Copyright
r
2015 Wolters Kluwer Health, Inc. All rights reserved.
The Neurologist
Volume 19, Number 4, April 2015
vessel vasculitis is characterized by acute onset of focal deficits with brain or spine MRI findings congruent of focal infarction, whereas small vessel vasculitis is characterized by multifocal lesions at the grey-white matter junction and in the deep white matter.11 The findings in our case supported the pathologic changes of probable VZV small vessel vasculitis with clinical evidence of acute onset and MRI evidence of diffused (longitudinal) and multifocal lesions. Another finding of the central cavitation of the lesion areas was also consistent with the characteristics of small vessel vasculitis.10 Detection of anti-VZV IgG antibody seemed also not against the speculative involvement of vasculopathy, as anti-VZV IgG antibody is the most valued sensitive indicator of VZV vasculopathy.6,8 Differential diagnosis of other causes of vasculitis should be taken into account. Spinal cord involvement was documented in 5% of patients with primary CNS vasculitis and the clinical characteristics were similar to those of the patients without spinal cord involvement.12 While primary angiitis of the CNS with pure spinal cord involvement is rare with only 1 case report in the literature.13 This is different from the pure involvement of spinal cord in our case. Other inflammatory vasculitis of the spinal cord were reported associated with immunologic disease including systemic lupus erythematosus14 and Behcet disease.15,16 This is different from the normal immunologic profile in our case. The convincing findings of no detectable HIV, no evidence of solid or hematological neoplasms, and normal immunologic profile led us to consider our patient as immunocompetent, though acute infection or activation of VZV with severe and diffuse multifocal lesions in the CNS is generally considered to exclusively affect immunocompromised patients. There was also report showing that VZV could induce severe and diffuse multifocal lesions in the CNS in immunocompetent patient, but it was in elderly (85-year-old) patient17 and the age-related lack of immune defenses may facilitate the VZV reactivation. While in current case, severe and diffuse multifocal lesions of the spinal cord was in an adolescent. This atypical age and immune setting and the lagging behind cutaneous rash could justify the delay in proper diagnosis and in starting the antiviral treatment, thereby reducing the possibility of clinical recovery. Our patient’s disastrous outcome may, at least partially, be attributed to the delayed antiviral treatment, as early treatment with acyclovir and steroids usually lead to a significant recovery in immunocompetent patient.18 A significant lesson is that a correct diagnosis was delayed due to the atypical age, normal immune setting, and lagging rash. An immediate acyclovir and corticosteroids treatment, after the early detection of serum anti-VZV IgM, an indicator of active infection, may be helpful for the prognosis. In this VZV myelitis case report, we wanted to emphasize the hemorrhage, the extensiveness of inflammatory spinal cord changes in our immunocompetent patient. In the conclusion, we would like to highlight the importance that uncommon neurological complications of VZV reactivation should also be taken into account, regardless
Copyright
r
Hemorrhagic Myelitis Caused by Varicella-Zoster
of the apparent immune state of the patient, especially for those with herpes zoster lagging behind the onset of myelitis, as delayed treatment may lead to disastrous outcome. REFERENCES 1. Chang CC, McLean C, Vujovic O, et al. Fatal acute varicellazoster virus hemorrhagic meningomyelitis with necrotizing vasculitis in an HIV-infected patient. Clin Infect Dis. 2009;48:372–373. 2. Tascini C, Polidori M, Flammini S, et al. Acute varicella-zoster virus necrotizing meningoencephalomyelitis with sudden visual loss and paraparesis in an HIV-infected patient. New Microbiol. 2010;33:267–269. 3. Yylmaz S, Koseolu HK, Yucel E. Transverse myelitis caused by varicella zoster: case reports. Braz J Infect Dis. 2007;11:179–181. 4. Moudgil SS. Teaching NeuroImages: herpes zoster myelitis. Neurology. 2010;75:e55. 5. Celik Y, Tabak F, Mert A, et al. Transverse myelitis caused by Varicella. Clin Neurol Neurosurg. 2001;103:260–261. 6. Nagel MA, Forghani B, Mahalingam R, et al. The value of detecting anti-VZV IgG antibody in CSF to diagnose VZV vasculopathy. Neurology. 2007;68:1069–1073. 7. Hogan EL, Krigman MR. Herpes zoster myelitis. Evidence for viral invasion of spinal cord. Arch Neurol. 1973;29:309–313. 8. Nagel MA, Cohrs RJ, Mahalingam R, et al. The varicella zoster virus vasculopathies: clinical, CSF, imaging, and virologic features. Neurology. 2008;70:853–860. 9. Gilden D, Nagel MA, Mahalingam R, et al. Clinical and molecular aspects of varicella zoster virus infection. Future Neurol. 2009;4: 103–117. 10. Devinsky O, Cho ES, Petito CK, et al. Herpes zoster myelitis. Brain. 1991;114(pt 3):1181–1196. 11. Kleinschmidt-DeMasters BK, Amlie-Lefond C, Gilden DH. The patterns of varicella zoster virus encephalitis. Hum Pathol. 1996; 27:927–938. 12. Salvarani C, Brown RD Jr, Calamia KT, et al. Primary CNS vasculitis with spinal cord involvement. Neurology. 2008;70: 2394–2400. 13. Goertz C, Wegner C, Bruck W, et al. Primary angiitis of the CNS with pure spinal cord involvement: a case report. J Neurol. 2010;257:1762–1764. 14. Romero-Vargas S, Ruiz-Sandoval JL, Barges-Coll J, et al. Pathological demonstration of cervical spinal cord inflammatory vasculitis in a patient with spontaneous spinal epidural haematoma associated with systemic lupus erythematosus. Neuropathol Appl Neurobiol. 2010;36:568–570. 15. Fukae J, Noda K, Fujishima K, et al. Subacute longitudinal myelitis associated with Behcet’s disease. Intern Med (Tokyo, Japan). 2010;49:343–347. 16. Zhao B, He L, Lai XH. A case of neuro-Behcet’s disease presenting with lumbar spinal cord involvement. Spinal Cord. 2010;48:172–173. 17. Tavazzi E, Minoli L, Ferrante P, et al. Varicella zoster virus meningo-encephalo-myelitis in an immunocompetent patient. Neurol Sci. 2008;29:279–283. 18. Amlie-Lefond C, Jubelt B. Neurologic manifestations of varicella zoster virus infections. Curr Neurol Neurosci Rep. 2009;9: 430–434.
2015 Wolters Kluwer Health, Inc. All rights reserved.
www.theneurologist.org |
95
Acute Longitudinal and Hemorrhagic Myelitis Caused by Varicella-Zoster Virus in an Immunocompetent Adolescent Yu Wang, MD, PhD, Chuan-Yong Yu, MD, Lin Huang, MD, Yuan-Yuan Han, MD, Yi-Min Liu, MD, and Jin Zhu, MD
Objectives: In immunocompetent person, varicella-zoster virus (VZV)-induced myelitis is rare and the lesion usually comprise focal spinal cord segment. VZV-induced hemorrhagic myelitis with lesions comprising longitudinal spinal cord has never been reported. Methods: We report a 15-year-old male patient who developed acute flaccid quadriplegia, days after a common cold. One week after the quadriplegia developed, he presented a typical herpes zoster on the lateral side of his left waist. Results: IgG antibody for VZV was detected in cerebrospinal fluid and the spinal cord magnetic resonance imaging findings were consistent with that of small vessel vasculitis. Treatment with acyclovir and corticosteroids resulted in no significant clinical improvement. Conclusions: In this report, we wanted to emphasize the hemorrhage, the extensiveness of inflammatory changes induced by VZV in spinal cord. It is suggested that VZV should be considered as a possible cause of a severe hemorrhagic myelitis even in immunocompetent adolescent. Key Words: varicella-zoster virus, myelitis, hemorrhage, immunocompetence, pathogenesis
(The Neurologist 2015;19:93–95)
T
he presentations of neurological complications of varicellazoster virus (VZV) infection or reactivation depend on the immune setting of the patients. Severe and diffuse multifocal lesions in the central nervous system (CNS) are usually observed in immunocompromised patients,1,2 in contrast, focal lesion, for example, transverse myelitis, usually in immunocompetent patients.3,4 Myelitis is rarely associated with VZV infection in immunocompetent patients and VZV-induced myelitic lesions comprising extensive spinal cord with hemorrhage has never been reported in immunocompetent patients. Treatment with high doses of steroids and acyclovir usually has significant recovery, though fatal cases have been reported.5 To the best of our knowledge, we describe for the first time a case of hemorrhagic myelitis with lesions comprising extensive spinal cord and this severe myelitis occurred before the appearance of cutaneous zoster rash. Combined From the Department of Neurology, The First Hospital of Anhui Medical University, Hefei, China. Supported in part by Natural Science grants from the National Natural Science Foundation of China (grant number 30970997) and from Natural Science Foundation of Anhui Province (grant number 09020103008). The authors declare no conflict of interest. Reprints: Yu Wang, MD, PhD, Department of Neurology, The First Hospital of Anhui Medical University, Jixi Road 218, Hefei 230022, China. E-mail: [email protected]. Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 1074-7931/15/1904-0093 DOI: 10.1097/NRL.0000000000000016
The Neurologist
Volume 19, Number 4, April 2015
treatment with high doses of steroids and acyclovir failed to achieve significant clinical improvement.
CLINICAL REPORT A 15-year-old, previously healthy boy was presented to our department with the complaints of acute and progressive paresthesia and weakness in both legs with the starting symptom of bilateral radiating pain band at T10 level for 1 day. Three days before the onset of this neurological symptom he had a common cold and fever. In a few days he became paraplegic with T4 sensory level and urinary incontinence. On admission, the neurological examination showed intact consciousness, cooperation, orientation, as well as normal cranial nerve and cerebellar function. Motor examination revealed severely reduced tone of lower limbs and mildly reduced tone of upper limbs. Power was grade 4/5 in upper limbs, and grade 0/5 in lower limbs. Deep tendon jerks and bilateral plantar responses were absent. Algesthesia examination showed diminished sensation to pain and light touch below the T2 dermatome. Other neurological examinations revealed no positive sign. Physical examination also revealed that the patient was eupneic, with heart rate at 82 bpm and blood pressure at 110/75 mm Hg. Abdomen observations showed distended with hydroaerial sounds. Lung examinations were uneventful. Cardiac auscultation revealed normal valve click. Laboratory findings for blood cell count, liver enzymes, BUN, Creactive protein, erythrocyte sedimentation rate, and urinalysis were normal. Serological tests were negative for hepatitis B and C, cytomegalovirus, herpes simplex virus, Epstein-Barr virus, PPD, and human immunodeficiency virus but positive in IgM for VZV. Lumbar puncture revealed crystal CSF with pressure of 110 mm H2O. CSF protein was 3.9 g/L (normal range, 0.15 to 0.45 g/L), glucose level 3.0 mmoL/L (normal range, 2.5 to 4.5 mmoL/L), and RBC count 150 cells/mL with 15 polymorphs and 2 lymphocytes. CSF viral antibody test revealed negative for all the familiar virus, including hepatitis B and C, cytomegalovirus, and Epstein-Barr virus, but positive in IgG for VZV. The serum/CSF ratio for anti-VZV IgG was reduced compared with that for total IgG as well as for albumin. An immediate magnetic resonance imaging (MRI) study showed multifocal low-signal intensity lesions throughout the long segments of the spinal cord (T1-T12) on T2-weighted (Fig. 1) and T1-weighted images. Apart from the multifocal low-signal intensity lesions, the spinal cord was swelling with mildly high-signal intensity on T2weighted images (Fig. 1) and mildly low-signal intensity on T1weighted images. Cranial MRI examination was normal. The patient was initially diagnosed with spinal cord hemorrhage from possible vascular malformation and treatment was started with 20% mannitol and hemostatic. At the seventh day after he was afflicted with paraplegia, he presented a typical herpes zoster on the lateral side of his left waist, which reached peak on the ninth day (Fig. 2). The diagnosis was then adjusted to acute longitudinal and hemorrhagic myelitis caused by VZV. Further inquiry of the past history indicated that he had varicella 10 years ago and had never accepted vaccination against varicella. He was treated with intravenous (IV) acyclovir 10 mg/kg/d divided by every 8 hours for 2 weeks and IV methylprednisolone 1.0 g every day for 5 days and subsequent IV dexamethasone 20 mg/d for 2 weeks. Within 6 months, he had not recovered from paraplegia, sensory deficits, and voiding dysfunction.
www.theneurologist.org |
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The Neurologist
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Volume 19, Number 4, April 2015
FIGURE 1. T2-weighted MR images of the spinal cord. MRI shows extensive T2-hyperintense changes along the spinal cord from T1 level to T12. Multiple central cavitations (hemorrhage) indicated by the arrowheads can be observed along the longitudinal spinal cord. (C) is the axial image in section indicated by arrows in (A) and (B).
DISCUSSION We describe a longitudinal and hemorrhagic myelitis caused by VZV in an immunocompetent adolescent. Virological verification was provided by the detection of anti-VZV IgG antibody and intrathecal production of anti-VZV IgG antibody,6 which was indicated by the reduced serum/CSF ratios compared with normal serum/CSF ratios of total IgG and
FIGURE 2. Herpes zoster in the lumbar region.
94 | www.theneurologist.org
albumin in our patient. This report illustrates 3 notable features. First, it is the first case of myelitis with widespread multifocal hemorrhagic lesions due to VZV infection. Second, this disastrous myelitis was in an immunocompetent adolescent but not in an immunocompromised patient or in an elderly person. Third, the cutaneous herpes zoster appeared lagging behind the onset of myelitis in an immunocompetent patient. The pathogenesis of neurological complications associated with VZV infection is largely unclear. Direct viral invasion has been supposed in the pathogenesis of VZV myelitis, because VZV particles were found in glial cells. Hogan and Krigman7 presented evidence of direct invasion of VZV in a patient with herpes zoster transverse myelitis after a postmortem examination of the spinal cord. This supposed direct viral invasion pathogenesis is further supported by the clinical findings that VZV myelitis was usually monofocal lesions succeeding a zoster at the corresponding dermatome.3,8 In our case, the zoster dermatome was at T9-T10 levels of which the corresponding spinal cord segments were also most severely lesioned (biggest hemorrhagic lesion), and at the same levels the patient experienced the initial bilateral radiating pain band. Allergic mechanisms after VZV infection are also suggested.9 Pathologic examinations in a large series of patients with VZV myelitis showed that vasculitis, necrosis, and demyelination coexisted in the spinal cord.10 VZV infection can cause large vessel vasculitis as well as small vessel vasculitis. Large
Copyright
r
2015 Wolters Kluwer Health, Inc. All rights reserved.
The Neurologist
Volume 19, Number 4, April 2015
vessel vasculitis is characterized by acute onset of focal deficits with brain or spine MRI findings congruent of focal infarction, whereas small vessel vasculitis is characterized by multifocal lesions at the grey-white matter junction and in the deep white matter.11 The findings in our case supported the pathologic changes of probable VZV small vessel vasculitis with clinical evidence of acute onset and MRI evidence of diffused (longitudinal) and multifocal lesions. Another finding of the central cavitation of the lesion areas was also consistent with the characteristics of small vessel vasculitis.10 Detection of anti-VZV IgG antibody seemed also not against the speculative involvement of vasculopathy, as anti-VZV IgG antibody is the most valued sensitive indicator of VZV vasculopathy.6,8 Differential diagnosis of other causes of vasculitis should be taken into account. Spinal cord involvement was documented in 5% of patients with primary CNS vasculitis and the clinical characteristics were similar to those of the patients without spinal cord involvement.12 While primary angiitis of the CNS with pure spinal cord involvement is rare with only 1 case report in the literature.13 This is different from the pure involvement of spinal cord in our case. Other inflammatory vasculitis of the spinal cord were reported associated with immunologic disease including systemic lupus erythematosus14 and Behcet disease.15,16 This is different from the normal immunologic profile in our case. The convincing findings of no detectable HIV, no evidence of solid or hematological neoplasms, and normal immunologic profile led us to consider our patient as immunocompetent, though acute infection or activation of VZV with severe and diffuse multifocal lesions in the CNS is generally considered to exclusively affect immunocompromised patients. There was also report showing that VZV could induce severe and diffuse multifocal lesions in the CNS in immunocompetent patient, but it was in elderly (85-year-old) patient17 and the age-related lack of immune defenses may facilitate the VZV reactivation. While in current case, severe and diffuse multifocal lesions of the spinal cord was in an adolescent. This atypical age and immune setting and the lagging behind cutaneous rash could justify the delay in proper diagnosis and in starting the antiviral treatment, thereby reducing the possibility of clinical recovery. Our patient’s disastrous outcome may, at least partially, be attributed to the delayed antiviral treatment, as early treatment with acyclovir and steroids usually lead to a significant recovery in immunocompetent patient.18 A significant lesson is that a correct diagnosis was delayed due to the atypical age, normal immune setting, and lagging rash. An immediate acyclovir and corticosteroids treatment, after the early detection of serum anti-VZV IgM, an indicator of active infection, may be helpful for the prognosis. In this VZV myelitis case report, we wanted to emphasize the hemorrhage, the extensiveness of inflammatory spinal cord changes in our immunocompetent patient. In the conclusion, we would like to highlight the importance that uncommon neurological complications of VZV reactivation should also be taken into account, regardless
Copyright
r
Hemorrhagic Myelitis Caused by Varicella-Zoster
of the apparent immune state of the patient, especially for those with herpes zoster lagging behind the onset of myelitis, as delayed treatment may lead to disastrous outcome. REFERENCES 1. Chang CC, McLean C, Vujovic O, et al. Fatal acute varicellazoster virus hemorrhagic meningomyelitis with necrotizing vasculitis in an HIV-infected patient. Clin Infect Dis. 2009;48:372–373. 2. Tascini C, Polidori M, Flammini S, et al. Acute varicella-zoster virus necrotizing meningoencephalomyelitis with sudden visual loss and paraparesis in an HIV-infected patient. New Microbiol. 2010;33:267–269. 3. Yylmaz S, Koseolu HK, Yucel E. Transverse myelitis caused by varicella zoster: case reports. Braz J Infect Dis. 2007;11:179–181. 4. Moudgil SS. Teaching NeuroImages: herpes zoster myelitis. Neurology. 2010;75:e55. 5. Celik Y, Tabak F, Mert A, et al. Transverse myelitis caused by Varicella. Clin Neurol Neurosurg. 2001;103:260–261. 6. Nagel MA, Forghani B, Mahalingam R, et al. The value of detecting anti-VZV IgG antibody in CSF to diagnose VZV vasculopathy. Neurology. 2007;68:1069–1073. 7. Hogan EL, Krigman MR. Herpes zoster myelitis. Evidence for viral invasion of spinal cord. Arch Neurol. 1973;29:309–313. 8. Nagel MA, Cohrs RJ, Mahalingam R, et al. The varicella zoster virus vasculopathies: clinical, CSF, imaging, and virologic features. Neurology. 2008;70:853–860. 9. Gilden D, Nagel MA, Mahalingam R, et al. Clinical and molecular aspects of varicella zoster virus infection. Future Neurol. 2009;4: 103–117. 10. Devinsky O, Cho ES, Petito CK, et al. Herpes zoster myelitis. Brain. 1991;114(pt 3):1181–1196. 11. Kleinschmidt-DeMasters BK, Amlie-Lefond C, Gilden DH. The patterns of varicella zoster virus encephalitis. Hum Pathol. 1996; 27:927–938. 12. Salvarani C, Brown RD Jr, Calamia KT, et al. Primary CNS vasculitis with spinal cord involvement. Neurology. 2008;70: 2394–2400. 13. Goertz C, Wegner C, Bruck W, et al. Primary angiitis of the CNS with pure spinal cord involvement: a case report. J Neurol. 2010;257:1762–1764. 14. Romero-Vargas S, Ruiz-Sandoval JL, Barges-Coll J, et al. Pathological demonstration of cervical spinal cord inflammatory vasculitis in a patient with spontaneous spinal epidural haematoma associated with systemic lupus erythematosus. Neuropathol Appl Neurobiol. 2010;36:568–570. 15. Fukae J, Noda K, Fujishima K, et al. Subacute longitudinal myelitis associated with Behcet’s disease. Intern Med (Tokyo, Japan). 2010;49:343–347. 16. Zhao B, He L, Lai XH. A case of neuro-Behcet’s disease presenting with lumbar spinal cord involvement. Spinal Cord. 2010;48:172–173. 17. Tavazzi E, Minoli L, Ferrante P, et al. Varicella zoster virus meningo-encephalo-myelitis in an immunocompetent patient. Neurol Sci. 2008;29:279–283. 18. Amlie-Lefond C, Jubelt B. Neurologic manifestations of varicella zoster virus infections. Curr Neurol Neurosci Rep. 2009;9: 430–434.
2015 Wolters Kluwer Health, Inc. All rights reserved.
www.theneurologist.org |
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