Letters to Editor
Hematomyelia due to ruptured intramedullary aneurysm associated with arteriovenous malformation masquerading as demyelination
aneurysm become lax. Fine needle aspiration of fundus was done and aneurysm sac was emptied [Figure 5d]. Further dissection of aneurysm was not attempted fearing damage to normal surrounding cord substance. Postoperative MRI spine revealed no residual nidus [Figure 6a and 6b] and spinal angiography confirmed total excision of the AVM and complete obliteration of the aneurysm [Figure 7a and 7b]. At 6 weeks follow‑up, she was ambulant with support and
sir, Spinal aneurysms (SA) are very rare lesions.[1,2] They can be isolated (Type 1) or associated with arteriovenous malformations (AVM) (Type 2).[3‑5] We report a spinal aneurysm that bled, causing hematomyelia. This was mistaken for a demyelinating lesion and treated as such. An 18‑year‑old female presented with sudden onset of weakness and parasthesiae of all 4 limbs. She also complained of non‑radiating neck pain. On evaluation (at another hospital), she was found to have flaccid paralysis of all 4 limbs with complete loss of sensation below C4. She had shallow respiration and was intubated and electively ventilated. Magnetic resonance imaging (MRI) cervical spine T2W images revealed diffuse hyperintensity of the cord extending from the pontomedullary junction till the lower dorsal spine [Figure 1a]. T 1W images revealed an ill‑defined focal hyperintensity at C4 with cord expansion [Figure 1b]. A diagnosis of acute demyelination was made and she was administered corticosteroids and subsequently IV immunoglobulins. She recovered gradually and power improved to 3/5 in the upper limbs and 2/5 in the lower limbs. Repeat MRI one month later revealed partial resolution of the T 2 signal changes and multiple flow voids at C4‑5 [Figure 2]. She was then referred to our institute. A spinal angiogram with cannulation of the left thyrocervical trunk revealed aperimedullary AVM at C4‑5 level with a large intranidal aneurysm [Figure 3a and 3b]. MRI confirmed the position of aneurysm to be intramedullary with surrounding hemosiderin deposition suggestive of old rupture [Figure 4a and 4b]. Since this was a Type 1Ain SA, she underwent surgery. Via C4‑6 laminectomy and medial 1/3 C4‑5 left facetectomy, the lesion was accessed. A leash of abnormal vessels was found on the pial surface [Figure 5a]. The arterial feeder was identified in the C4‑5 foramen, coagulated and divided [Figure 5b]. Subsequently, the nidus on the perimedullary region was circumferentially dissected and excised. The aneurysm was buried within the cord substance and the radiculo‑pial arterial feeder [Figure 5c] was coagulated following which the Neurology India | May-Jun 2014 | Vol 62 | Issue 3
a
b Figure 1: (a) MRI T2W image showing holocord edema extending till pontomedullary junction (b) MRI T1W image showing focal cord hyperintensity at C4-5 level
Figure 2: MRI T2W image after 6 weeks showing flow voids at C4-5 level
a
b
Figure 3: (a) Spinal DSA revealing intranidal aneurysm with Arteriovenous malformation (b) Spinal DSA showing venous drainage of the AVM
311
Letters to Editor
a
a
b
c
d
b
Figure 4: (a) MRI T2W axial image showing the intramedullaryy location of the aneurysm (b) MRI T2W axial image showing multiple flow voids in the left foraminal region
Figure 5: (a) Intraop picture showing the nidus (b) Main arterial feeder from the radicular artery (c) Showing the lateral surface of the aneurysm wall (main fundus buried within the cord substance) (d) Aneurysm fundus being punctured to empty the sac
a
b Figure 6: (a) - A Postoperative T2W Sagittal MRI showing complete excision of nidus (b) Postoperative T2W Axial MRI showing complete excision of nidus
her sphincter functions were normal. In an extensive review of literature by Madhugir et al., most SA occurred in the cervical spine and were intraduralextra‑medullaryin location. [5] Most of these patients presented with spinal (and/or cranial) subarachnoid hemorrhage.[2‑4] Only 12 cases of intramedullary aneurysms are reported till date.[5] Hematomyelia due to SA is rare (14.7% of SA) and is associated with poor outcome.[5] In the present patient, hematomyelia in the initial MRI scan was masked by the extensive cord edema and the focal hyperintense signal at C4 level in T1W image (suggestive of bleed) was overlooked resulting in the erroneous diagnosis of demyelination. The diagnostic error could also be due to the extreme rarity of this lesion. Both microsurgical and endovascular obliteration are described to manage SA. However, microsurgical excision is the preferred option, if the SA is associated with an AVM (Type 1).[3,5] Microsurgically, aneurysms can either be excised or parent vessel can be clipped. Endovascular coiling would add to increased mass within cord substance resulting itself in compressive myelopathy. In the present case, the aneurysm could be successfully obliterated by coagulating the radiculo‑pial feeder vessels, thereby reliving the mass effect. It is probably not necessary to excise the aneurysm, if it is 312
a
b Figure 7: Postoperative Spinal DSA showing complete obliteartion of AVM & aneurysm and (a) right side and (b) left side injection
completely buried within the cord to prevent further cord damage during circumferential dissection of the aneurysm.
V. R. Roopesh Kumar, Venkatesh S. Madhugiri, A. S. Ramesh, A. Sathia Prabhu Department of Neuroaurgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India E‑mail: [email protected]
References 1. Biondi A, Merland JJ, Hodes JE, Pruvo JP, Reizine D. Aneurysms of spinal arteries associated with intramedullary arteriovenous malformations. I. Angiographic and clinical aspects. AJNR Am J Neuroradiol 1992;13:913‑22. 2. Cavuşoğlu H, Ozdilmaç A, Sahin Y, Aydin Y. Isolated posterior spinal artery aneurysm causing intracranial acute subarachnoidal hemorrhage. Acta Neurochir (Wien) 2010;152:721‑4. 3. Gonzalez LF, Zabramski JM, Tabrizi P, Wallace RC, Massand MG, Spetzler RF. Spontaneous spinal subarachnoid hemorrhage secondary Neurology India | May-Jun 2014 | Vol 62 | Issue 3
Letters to Editor
to spinal aneurysms: Diagnosis and treatment paradigm. Neurosurg 2005;57:1127‑31. 4. Kurita M, Endo M, Kitahara T, Fujii K. Subarachnoid haemorrhage due to a lateral spinal artery aneurysm misdiagnosed as a posterior inferior cerebellar artery aneurysm: Case report and literature review. Acta Neurochir (Wien) 2009;151:165‑9. 5. Madhugiri VS, Ambekar S, Roopesh Kumar VR, Sasidharan GM, Nanda A. Spinal aneurysms: Clinicoradiological features and management paradigms. J Neurosurg Spine 2013;19:34‑48.
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.137003
Received: 23-07-2014 Review completed: 01-06-2014 Accepted: 01-06-2014
Cervical spine brucellosis presenting as fever with neck stiffness and cervical compressive myelopathy: A case report Sir, Osteoarticular brucellosis is the most common presentation of systemic brucellosis and most commonly affects lumbar spine, but isolated involvement of cervical region is very rare, 1.2–2.1%.[1] Cervical spine involvement can pose diagnostic difficulties due to its similarities with other conditions such as tuberculous spondylitis, metastases, and myeloma,[2] causing delay in diagnosis and treatment. A 60‑year‑old male farmer presented with moderate‑grade intermittent fever and restricted neck movements of one month and 9 days duration, respectively. There was no history of chronic cough, hemoptysis, altered sensorium, neck trauma, weight loss, or any medical illness. General examination was normal except for pallor. Neck stiffness and neck muscle spasm was present. Neurological examination was normal. Blood investigations revealed normocytic normochromic anemia, elevated erythrocyte sedimentation rate of 70 mm at 1 hour, and hyperglobulinemia (globulins ‑ 7.2 gm%). Cerebrospinal fluid examination was normal. Sputum for acid fast bacilli and Mantoux test was negative. Neurology India | May-Jun 2014 | Vol 62 | Issue 3
Magnetic resonance imaging (MRI) of cervical spine showed altered marrow signal intensities in cervical vertebrae C3-C5 indicating diffuse marrow infiltrative disorder [Figure 1a]. Bone marrow biopsy revealed normocellular marrow with plasmacytosis (plasma cells 1.1). For definitive
a
c
b
d
Figure 1: (a) MRI on 1st visit showing altered marrow signal intensities in cervical vertebrae C3-C5 s/o diffuse marrow infiltrative disorder, diffuse disc bulges and osteophytes (b) MRI cervical spine on subsequent admission showing progression of lesion with altered marrow signal C1, C2, C3, C6, C7, D1 vertebral body, discs, tip of clivus with partial collapse (c) Bone scan showing increased radionucleotide uptake in cervical region (d) Vertebral biopsy showing chronic non specific inflammation with plasma cells and russel bodies
313
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Hematomyelia due to ruptured intramedullary aneurysm associated with arteriovenous malformation masquerading as demyelination
aneurysm become lax. Fine needle aspiration of fundus was done and aneurysm sac was emptied [Figure 5d]. Further dissection of aneurysm was not attempted fearing damage to normal surrounding cord substance. Postoperative MRI spine revealed no residual nidus [Figure 6a and 6b] and spinal angiography confirmed total excision of the AVM and complete obliteration of the aneurysm [Figure 7a and 7b]. At 6 weeks follow‑up, she was ambulant with support and
sir, Spinal aneurysms (SA) are very rare lesions.[1,2] They can be isolated (Type 1) or associated with arteriovenous malformations (AVM) (Type 2).[3‑5] We report a spinal aneurysm that bled, causing hematomyelia. This was mistaken for a demyelinating lesion and treated as such. An 18‑year‑old female presented with sudden onset of weakness and parasthesiae of all 4 limbs. She also complained of non‑radiating neck pain. On evaluation (at another hospital), she was found to have flaccid paralysis of all 4 limbs with complete loss of sensation below C4. She had shallow respiration and was intubated and electively ventilated. Magnetic resonance imaging (MRI) cervical spine T2W images revealed diffuse hyperintensity of the cord extending from the pontomedullary junction till the lower dorsal spine [Figure 1a]. T 1W images revealed an ill‑defined focal hyperintensity at C4 with cord expansion [Figure 1b]. A diagnosis of acute demyelination was made and she was administered corticosteroids and subsequently IV immunoglobulins. She recovered gradually and power improved to 3/5 in the upper limbs and 2/5 in the lower limbs. Repeat MRI one month later revealed partial resolution of the T 2 signal changes and multiple flow voids at C4‑5 [Figure 2]. She was then referred to our institute. A spinal angiogram with cannulation of the left thyrocervical trunk revealed aperimedullary AVM at C4‑5 level with a large intranidal aneurysm [Figure 3a and 3b]. MRI confirmed the position of aneurysm to be intramedullary with surrounding hemosiderin deposition suggestive of old rupture [Figure 4a and 4b]. Since this was a Type 1Ain SA, she underwent surgery. Via C4‑6 laminectomy and medial 1/3 C4‑5 left facetectomy, the lesion was accessed. A leash of abnormal vessels was found on the pial surface [Figure 5a]. The arterial feeder was identified in the C4‑5 foramen, coagulated and divided [Figure 5b]. Subsequently, the nidus on the perimedullary region was circumferentially dissected and excised. The aneurysm was buried within the cord substance and the radiculo‑pial arterial feeder [Figure 5c] was coagulated following which the Neurology India | May-Jun 2014 | Vol 62 | Issue 3
a
b Figure 1: (a) MRI T2W image showing holocord edema extending till pontomedullary junction (b) MRI T1W image showing focal cord hyperintensity at C4-5 level
Figure 2: MRI T2W image after 6 weeks showing flow voids at C4-5 level
a
b
Figure 3: (a) Spinal DSA revealing intranidal aneurysm with Arteriovenous malformation (b) Spinal DSA showing venous drainage of the AVM
311
Letters to Editor
a
a
b
c
d
b
Figure 4: (a) MRI T2W axial image showing the intramedullaryy location of the aneurysm (b) MRI T2W axial image showing multiple flow voids in the left foraminal region
Figure 5: (a) Intraop picture showing the nidus (b) Main arterial feeder from the radicular artery (c) Showing the lateral surface of the aneurysm wall (main fundus buried within the cord substance) (d) Aneurysm fundus being punctured to empty the sac
a
b Figure 6: (a) - A Postoperative T2W Sagittal MRI showing complete excision of nidus (b) Postoperative T2W Axial MRI showing complete excision of nidus
her sphincter functions were normal. In an extensive review of literature by Madhugir et al., most SA occurred in the cervical spine and were intraduralextra‑medullaryin location. [5] Most of these patients presented with spinal (and/or cranial) subarachnoid hemorrhage.[2‑4] Only 12 cases of intramedullary aneurysms are reported till date.[5] Hematomyelia due to SA is rare (14.7% of SA) and is associated with poor outcome.[5] In the present patient, hematomyelia in the initial MRI scan was masked by the extensive cord edema and the focal hyperintense signal at C4 level in T1W image (suggestive of bleed) was overlooked resulting in the erroneous diagnosis of demyelination. The diagnostic error could also be due to the extreme rarity of this lesion. Both microsurgical and endovascular obliteration are described to manage SA. However, microsurgical excision is the preferred option, if the SA is associated with an AVM (Type 1).[3,5] Microsurgically, aneurysms can either be excised or parent vessel can be clipped. Endovascular coiling would add to increased mass within cord substance resulting itself in compressive myelopathy. In the present case, the aneurysm could be successfully obliterated by coagulating the radiculo‑pial feeder vessels, thereby reliving the mass effect. It is probably not necessary to excise the aneurysm, if it is 312
a
b Figure 7: Postoperative Spinal DSA showing complete obliteartion of AVM & aneurysm and (a) right side and (b) left side injection
completely buried within the cord to prevent further cord damage during circumferential dissection of the aneurysm.
V. R. Roopesh Kumar, Venkatesh S. Madhugiri, A. S. Ramesh, A. Sathia Prabhu Department of Neuroaurgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India E‑mail: [email protected]
References 1. Biondi A, Merland JJ, Hodes JE, Pruvo JP, Reizine D. Aneurysms of spinal arteries associated with intramedullary arteriovenous malformations. I. Angiographic and clinical aspects. AJNR Am J Neuroradiol 1992;13:913‑22. 2. Cavuşoğlu H, Ozdilmaç A, Sahin Y, Aydin Y. Isolated posterior spinal artery aneurysm causing intracranial acute subarachnoidal hemorrhage. Acta Neurochir (Wien) 2010;152:721‑4. 3. Gonzalez LF, Zabramski JM, Tabrizi P, Wallace RC, Massand MG, Spetzler RF. Spontaneous spinal subarachnoid hemorrhage secondary Neurology India | May-Jun 2014 | Vol 62 | Issue 3
Letters to Editor
to spinal aneurysms: Diagnosis and treatment paradigm. Neurosurg 2005;57:1127‑31. 4. Kurita M, Endo M, Kitahara T, Fujii K. Subarachnoid haemorrhage due to a lateral spinal artery aneurysm misdiagnosed as a posterior inferior cerebellar artery aneurysm: Case report and literature review. Acta Neurochir (Wien) 2009;151:165‑9. 5. Madhugiri VS, Ambekar S, Roopesh Kumar VR, Sasidharan GM, Nanda A. Spinal aneurysms: Clinicoradiological features and management paradigms. J Neurosurg Spine 2013;19:34‑48.
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.137003
Received: 23-07-2014 Review completed: 01-06-2014 Accepted: 01-06-2014
Cervical spine brucellosis presenting as fever with neck stiffness and cervical compressive myelopathy: A case report Sir, Osteoarticular brucellosis is the most common presentation of systemic brucellosis and most commonly affects lumbar spine, but isolated involvement of cervical region is very rare, 1.2–2.1%.[1] Cervical spine involvement can pose diagnostic difficulties due to its similarities with other conditions such as tuberculous spondylitis, metastases, and myeloma,[2] causing delay in diagnosis and treatment. A 60‑year‑old male farmer presented with moderate‑grade intermittent fever and restricted neck movements of one month and 9 days duration, respectively. There was no history of chronic cough, hemoptysis, altered sensorium, neck trauma, weight loss, or any medical illness. General examination was normal except for pallor. Neck stiffness and neck muscle spasm was present. Neurological examination was normal. Blood investigations revealed normocytic normochromic anemia, elevated erythrocyte sedimentation rate of 70 mm at 1 hour, and hyperglobulinemia (globulins ‑ 7.2 gm%). Cerebrospinal fluid examination was normal. Sputum for acid fast bacilli and Mantoux test was negative. Neurology India | May-Jun 2014 | Vol 62 | Issue 3
Magnetic resonance imaging (MRI) of cervical spine showed altered marrow signal intensities in cervical vertebrae C3-C5 indicating diffuse marrow infiltrative disorder [Figure 1a]. Bone marrow biopsy revealed normocellular marrow with plasmacytosis (plasma cells 1.1). For definitive
a
c
b
d
Figure 1: (a) MRI on 1st visit showing altered marrow signal intensities in cervical vertebrae C3-C5 s/o diffuse marrow infiltrative disorder, diffuse disc bulges and osteophytes (b) MRI cervical spine on subsequent admission showing progression of lesion with altered marrow signal C1, C2, C3, C6, C7, D1 vertebral body, discs, tip of clivus with partial collapse (c) Bone scan showing increased radionucleotide uptake in cervical region (d) Vertebral biopsy showing chronic non specific inflammation with plasma cells and russel bodies
313
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
