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LETTERS TO THE EDITOR
Year : 2022  |  Volume : 25  |  Issue : 6  |  Page : 1241-1243
 

An unusual cause for cauda equina and spastic paraparesis: Two cases of brucellar arachnoiditis without spondylodiscitis or constitutional symptoms


1 Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
2 Department of Neuromicrobiology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
3 Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

Date of Submission02-Aug-2022
Date of Decision13-Sep-2022
Date of Acceptance15-Sep-2022
Date of Web Publication3-Dec-2022

Correspondence Address:
Deepak Menon
Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru-560 029, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aian.aian_667_22

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How to cite this article:
Menon D, Varghese N, Nagarathna S, Saini J, Netravathi M. An unusual cause for cauda equina and spastic paraparesis: Two cases of brucellar arachnoiditis without spondylodiscitis or constitutional symptoms. Ann Indian Acad Neurol 2022;25:1241-3

How to cite this URL:
Menon D, Varghese N, Nagarathna S, Saini J, Netravathi M. An unusual cause for cauda equina and spastic paraparesis: Two cases of brucellar arachnoiditis without spondylodiscitis or constitutional symptoms. Ann Indian Acad Neurol [serial online] 2022 [cited 2023 Jan 29];25:1241-3. Available from: https://www.annalsofian.org/text.asp?2022/25/6/1241/362583




Sir,

Brucellosis is a bacterial zoonosis caused by gram-negative coccobacilli of brucella species and is notorious to cause chronic multisystem involvement with protean manifestations. Neurological involvement is reported between 3 and 12% in most large series of brucellosis.[1],[2] The neurological manifestations are heterogenous ranging between central nervous system affection such as meningoencephalitis, cerebellitis, and myelitis and peripheral nervous system involvement including polyradiculopathies and cranial neuropathies.[1],[3],[4] Accompanying constitutional symptoms, such as fever, headache, and signs of meningeal irritation, are present in majority of the patients, in the absence of which a high index of suspicion is required for diagnosis. Presentation of neurobrucellosis, with chronic lumbosacral polyradiculopathy and chronic gradually progressive spastic paraparesis, without constitutional symptoms or evidence of spondylodiscitis is rare.

A 57-year-old man presented with a one-year history of progressive severe lower backache. Six months later, he noted buckling and twisting of right knee and ankle and in another three months had similar complaints in the left leg along with bladder symptoms with difficulty in initiation. At presentation, he was using wheelchair and on diapers due to continuous dribbling. He had no constitutional symptoms or history to suggest immunosuppression.

His examination revealed lower limb hypotonia, right more than left lower limb weakness distal more than proximal, absent lower limb deep tendon reflexes, bulbocavernosus, and anal reflexes. Sensory loss was present over legs but could not be well demarcated due to his troubling radicular pains. A non-contrast magnetic resonance imaging (MRI) done three months prior was normal. Evaluation for inflammatory, neoplastic, and infective causes includes anti-neutrophil cytoplasmic antibodies (ANCA), IgG4 levels, computerized tomography (CT) thorax, and abdomen; tuberculosis and human immunodeficiency virus (HIV) evaluation were negative. MRI contrast sequences at our center revealed cauda enhancement and exudates covering the conus–epiconus region [Figure 1]a, [Figure 1]b, [Figure 1]c. Cerebrospinal fluid (CSF) showed 25 lymphocytes with protein of 368 mg/dl and glucose 55 mg/dl. His serum and CSF brucella-standard agglutination test and serum and CSF IgG for brucella returned strongly positive. With ceftriaxone, rifampicin, and doxycycline along with dexamethasone, the patient had resolution of pain and was ambulant with minimal support at one month of follow-up.
Figure 1: (a) T1 post-contrast sagittal section of the whole spine (case 1) showing contrast enhancement around the cauda equina. (b and c), respectively, showing the lumbar T2 and T1 post-contrast sequences. (d) T1 post-contrast sagittal section (case 2) showing contrast enhancement around cauda, conus, epiconus, and over cervical region (arrow). (e and f), respectively, showing lumbar T2 and T1 post-contrast sequences. * Bladder distension due to LMN bladder

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A 27-year-old man presented with gradually progressive difficulty in walking of nine months duration with dragging of right leg followed by left with tripping and buckling. Around six months into the disease, he noted bladder urgency and urge incontinence and reported tingling paresthesia and numbness which ascended from feet to upper abdomen. His mobility progressively worsened, developed urinary retention requiring bladder catheterization. At the time of presentation, he was dependent on two-person support for ambulation. He had no constitutional symptoms.

On examination, he had grade 3 spasticity of both lower limbs and weakness in pyramidal distribution and sensory loss till D10 vertebral level. Plantar and abdominal reflexes were absent bilaterally. On leading question, he mentioned feeling mild decrease in hearing in the past six months, and his audiometry showed bilateral sensorineural hearing loss. His non-contrast MRI done elsewhere was normal. Repeat MRI spine from our center showed diffuse enhancement of nerve roots; thoracolumbar and cervical spinal cord showed meningeal enhancement [Figure 1d–f]. CSF was xanthochromic, with five lymphocytes, protein of 749 mg/dl, and normal glucose. Serum and CSF brucella IgG were strongly positive. Biopsy of the lumbar leptomeninges revealed perivascular chronic inflammation, epithelioid histiocytes with occasional giant cells, ill-formed granulomas without any necrosis, and negative AFB and fungal stains. On ceftriaxone, doxycycline, rifampicin, streptomycin, and dexamethasone, at four weeks the patient was ambulant without support though had spasticity and bladder urgency. The follow-up MRI showed significant resolution.

Presentation as polyradiculopathy and myelopathy is described with varying frequency in neurobrucellosis. In a large series of 187 patients from Turkey, peripheral neuropathy/radiculopathy was seen only in 7% and paraplegia in 4%.[4] The pathomechanism is not well elucidated and data are not backed up with imaging findings or a detailed neurological perspective.[2],[3],[4],[5] Spinal brucellosis due to osteoarticular involvement, spondylodiscitis, and epidural abscess are mechanism that can lead to cauda equina and compressive myelopathy.[6],[7] In our patients, the vertebral bodies, disk, and soft tissues were completely normal. It was the contrast sequences that revealed arachnoiditis, which was more restricted to the cauda conus region in the first case and more extensive, encasing the spinal cord up to cervical level in the other. Meningeal biopsy confirmed a chronic granulomatous meningitis. Similar findings have been noted in a biopsy series in brucellosis, but meningeal biopsy was not performed and has not been reported in neurobrucellosis.[8] The clinical manifestation depends on the neuraxis involved and on the initial site of breach of blood brain barrier. In the absence of any contiguous structural involvement, a hematogenous spread from gastrointestinal source may be postulated with invasion of blood meningeal barrier through infected monocytes rather than direct bacterial infiltration.[9] The ensuing cytokine reaction drives chronic reactive inflammatory cascade which leads to arachnoiditis. There have been two similar reports of short-duration spastic paraparesis in brucellosis where the spine MRI including contrast sequences were normal.[10]

The presence of systemic symptoms is the hallmark of neurobrucellosis that includes fever, myalgia, sweating, headache, and confusion, seen in 70 to 80% of patients.[3],[5] Their absence could lead to delay in diagnosis with evaluation focusing on alternate causes. The low virulence of infecting brucella species and the relative low infection load could result in less severe and more contained disease. An important clue to diagnosis is the presence of sensorineural hearing loss due to vestibulocochlear involvement but like patients may be oblivious to minor deficit and may require audiometry. As in tuberculous meningitis, use of steroids may ameliorate inflammation in brucellar meningitis, may reduce neural edema, and can aid penetration of antibiotics. Both our patients had remarkable response within one month of treatment.

Cauda equina syndrome and spastic paraparesis can be manifestations of neurobrucellosis. The absence of systemic symptoms need not be a deterrent to diagnosis, and a high index of suspicion should be maintained in endemic areas. Contrast sequences should be requested and can be the only pointer to diagnosis. Steroids could be an adjuvant to antibiotic treatment but needs further exploration.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Yetkin MA, Bulut C, Erdinc FS, Oral B, Tulek N. Evaluation of the clinical presentations in neurobrucellosis. Int J Infect Dis 2006;10:446–52.  Back to cited text no. 1
    
2.
Dreshaj S, Shala N, Dreshaj G, Ramadani N, Ponosheci A. Clinical manifestations in 82 neurobrucellosis patients from Kosovo. Mater Sociomed 2016;28:408–11.  Back to cited text no. 2
    
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Guven T, Ugurlu K, Ergonul O, Celikbas AK, Gok SE, Comoglu S, et al. Neurobrucellosis: Clinical and diagnostic features. Clin Infect Dis 2013;56:1407–12.  Back to cited text no. 3
    
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Gul HC, Erdem H, Bek S. Overview of neurobrucellosis: A pooled analysis of 187 cases. Int J Infect Dis 2009;13:e339–43.  Back to cited text no. 4
    
5.
Buzgan T, Karahocagil MK, Irmak H, Baran AI, Karsen H, Evirgen O, et al. Clinical manifestations and complications in 1028 cases of brucellosis: A retrospective evaluation and review of the literature. Int J Infect Dis 2010;14:e469–78.  Back to cited text no. 5
    
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Lifeso RM, Harder E, McCorkell SJ. Spinal brucellosis. J Bone Joint Surg Br 1985;67:345–51.  Back to cited text no. 6
    
7.
Etemadi A, Moniri R, Banaee S. Cauda equnia syndrome due to Brucella spondylodiscitis and epidural abscess formation: A case report. Interdiscip Neurosurg 2019;17:42–4.  Back to cited text no. 7
    
8.
Akhtar M, Ashraf AM. Pathology of brucellosis: A review of 88 biopsies. Ann Saudi Med 1989;9:247–53.  Back to cited text no. 8
    
9.
Rodríguez AM, Delpino MV, Miraglia MC, Giambartolomei GH. Immune mediators of pathology in neurobrucellosis: From blood to central nervous system. Neuroscience 2019;410:264–73.  Back to cited text no. 9
    
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Ahmed R, Patil BS. Neurobrucellosis: A rare cause for spastic paraparesis. Braz J Infect Dis 2009;13:245.  Back to cited text no. 10
    


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