Spinal adhesive arachnoiditis in an adult patient with spinal muscular atrophy type 3 treated with intrathecal therapy.

BACKGROUND: Spinal adhesive arachnoiditis is a chronic inflammatory process of the leptomeninges and intrathecal neural elements. The possible causes of arachnoiditis are: infections, injuries of spinal cord, surgical procedures and intrathecal administration of therapeutic substances or contrast. CASE PRESENTATION: We present a case of 56-old woman with spinal muscular atrophy type 3 who developed a severe back pain in the lumbosacral region after the fifth dose of nusinersen given intrathecally. Magnetic resonance of lumbosacral spine showed spinal adhesive arachnoiditis. She received high doses of methylprednisolone intravenously, and later non-steroidal anti-inflammatory drugs, alpha lipoic acid, vitamins and rehabilitation with slight improvement. CONCLUSIONS: The authors summarize that scheduled resonance imaging of the lumbosacral spine may be an important element of the algorithm in the monitoring of novel, intrathecal therapy in patients with spinal muscular atrophy.

Intracranial Arachnoiditis and Hydrocephalus.

A 62-year-old man presented a diffuse and predominantly cisternal acute nonaneurysmal subarachnoid hemorrhage associated with hydrocephalus. An external ventricular drain was placed, followed by clinical deterioration after its removal. At this point, a heavily T2-weighted high-resolution sequence of a brain magnetic resonance imaging showed acute hydrocephalus recrudescence and multiple arachnoid adhesions in the supravermian and interpeduncular cisterns, creating a loculated/cystic appearance. The diagnosis of intracranial arachnoiditis was made. Intracranial arachnoiditis results from meningeal inflammation. Fibrosis and adhesions at the subarachnoid spaces may follow, restricting cerebrospinal fluid circulation, particularly at the cranial base. Hydrocephalus probably resulted from the combination of subarachnoid hemorrhage and extensive scaring at the basal cisterns, precluding transdural and transvenous cerebrospinal fluid efflux. Heavily T2-weighted high-resolution magnetic resonance imaging sequences allow an exquisite depiction of arachnoiditis, displaying obstructive cisternal membranes, and contribute to better etiologic assessment and management of hydrocephalus.

Patterns of Intrathecal Ossification in Arachnoiditis Ossificans: A Retrospective Case Series.

Arachnoiditis ossificans is an uncommon end-stage appearance of chronic adhesive arachnoiditis. Imaging features of arachnoiditis ossificans are characteristic and should be diagnosed to avoid unnecessary intervention and guide prognosis and management. In this case series, we retrospectively analyzed CT and MR imaging of 41 patients to identify common patterns of intrathecal ossification and present the common etiologies. Thirty-two patients had a confirmed history of spinal instrumentation, 7 were discovered on imaging without prior surgical history, 1 had a history of ankylosing spondylitis, and 1 had trauma. The most frequent site of ossification was at the conus and cauda equina. Four patterns of ossification were identified, including central, nerve root encasing, weblike, and peripheral. Arachnoiditis ossificans is an important, likely under-recognized consideration in patients who present with back pain. Diagnosis can be made readily on CT; MR imaging diagnosis is also possible but may be challenging.

Giant intradural extramedullary spinal ependymoma, a rare arachnoiditis-mimicking condition: case report and literature review.

BACKGROUND AND IMPORTANCE: Ependymomas are tumours arising from the ependymal cells lining the ventricles and the central canal of the spinal cord. They represent the most common intramedullary spinal cord tumour in adults and are very rarely encountered in an extramedullary location. Only 40 cases of intradural extramedullary (IDEM) ependymomas have been reported, all of which were diagnosed pre-operatively as IDEM ependymomas on contrast-enhanced MRI. CLINICAL PRESENTATION: We report a 23-year old male presenting with rapidly worsening signs and symptoms of spinal cord disease. A spinal MRI demonstrated a posterior multi-cystic dilatation extended between T1 and T12. Post-contrast sequences showed peri-medullar leptomeningeal enhancement and the diagnosis of spinal arachnoiditis was made. The patient underwent surgery and the spinal cord appeared circumferentially wrapped by an irregular soft tissue. The tissue was sub-totally removed and the pathological diagnosis was ependymoma WHO grade II. The patient experienced an excellent neurological recovery and no further treatments were administered. A small residue is now stable at 2.5 years follow-up. CONCLUSIONS: Giant IDEM ependymomas are rare entities and pre-operative diagnosis can be challenging in some cases. Surgery represents the main treatment option being resolutive in most cases.

Thoracal arachnoiditis ossificans associated with multifocal motor neuropathy: a case report.

The association of arachnoiditis ossificans with acquired peripheral nerve disease is rare. We report a case who presented with progressive myelopathy due to arachnoiditis ossificans from prior trauma, complicated with multifocal motor neuropathy. Intradural bone was removed at surgery.

Reasons for re-hospitalization in patients with tuberculous meningitis, and its impact on outcome: a prospective observational study.

BACKGROUND: Patients with tuberculous meningitis may worsen despite being treated adequately with anti-tuberculosis drugs. This worsening may lead to re-hospitalization. The exact frequency and causes of re-hospitalization have not been studied previously. We aimed to study the causes of clinical worsening leading to re-hospitalization and its impact on prognosis. METHODS: This was a prospective observational study. Newly diagnosed patients with tuberculous meningitis (N = 150) were enrolled. Baseline clinical evaluation, neuroimaging, and cerebrospinal fluid examination were performed. Anti-tuberculosis drug regimen and corticosteroids were given as per WHO guidelines. Patients were followed for 6 months. Re-hospitalized patients were worked up and clinical evaluation, neuroimaging, and cerebrospinal fluid examination were performed again. Outcome assessment was done at the end of 6 months, and a modified Barthel index of ≤ 12 was considered a poor outcome. RESULTS: Twenty-three (15.3%) out of 150 patients needed re-hospitalization. The median time between discharge after the first hospitalization and re-hospitalization was 60 days. The common reasons for re-hospitalization were paradoxical neurological deterioration seen in 19 (82.6%) out of 23 patients, followed by drug toxicities (N = 2) and systemic involvement (N = 2). Paradoxically developed spinal arachnoiditis and opto-chiasmatic arachnoiditis were amongst the predominant reasons for re-hospitalization. At six months, re-hospitalization was an independent predictor of poor outcome (OR = 7.39, 95% CI 2.26-24.19). CONCLUSION: Approximately 15% of tuberculous meningitis patients needed re-hospitalization. Paradoxically developed spinal arachnoiditis and opto-chiasmatic arachnoiditis were predominant reasons for re-hospitalization. Re-hospitalization adversely affected the outcome.

Comparison of dexamethasone regimens in tubercular meningitis (TBM): a randomized open label clinical trial.

INTRODUCTION: Corticosteroids are used as adjunctive treatment in tuberculous meningitis (TBM). However, there is no universally accepted regimen, type, duration, or route of steroid administration. METHODOLOGY: In a randomized open labelled pilot study, TBM patients were divided into overlap oral dexamethasone (OOD) and direct oral dexamethasone (DOD) arms. The total duration of steroid administration was 8 weeks. The primary outcome was symptomatic resolution at 1 month post randomization. The secondary outcomes were mortality and modified Rankin scale (mRS) at 3 and 6 months after initiation of steroids. RESULTS: Symptomatic resolution after one month of randomization in 53 randomized patients was similar in OOD (71.4% (15/21)) versus DOD ((85.0% (17/20)) arm (p value:0.45). Median mRS was also similar in OOD versus DOD (OOD: 2.5 (IQR: 1.0; 6.0) versus DOD: 1.0 (IQR: (0.0; 4.0); p value: 0.31)) arm at 6 months. The mortality at 6 months was 31.8% (7/22) in the OOD versus 20.0% (4/20) in the DOD arm (p value: 0.49). CONCLUSIONS: In this open label pilot study, the outcomes were similar in OOD versus DOD arms in terms of symptomatic resolution at 1 month, and morbidity, and mortality at 3 and 6 months. Patients with stage I to III TBM may be given injectable steroids for 1 week after which they may be switched to oral steroid. This regime cannot be applied to stage IV TBM and patients with complications like optico-chiasmatic or spinal arachnoiditis or vasculitic infarcts.

The leptomeninges as a critical organ for normal CNS development and function: First patient and public involved systematic review of arachnoiditis (chronic meningitis).

BACKGROUND & IMPORTANCE: This patient and public-involved systematic review originally focused on arachnoiditis, a supposedly rare "iatrogenic chronic meningitis" causing permanent neurologic damage and intractable pain. We sought to prove disease existence, causation, symptoms, and inform future directions. After 63 terms for the same pathology were found, the study was renamed Diseases of the Leptomeninges (DLMs). We present results that nullify traditional clinical thinking about DLMs, answer study questions, and create a unified path forward. METHODS: The prospective PRISMA protocol is published at Arcsology.org. We used four platforms, 10 sources, extraction software, and critical review with ≥2 researchers at each phase. All human sources to 12/6/2020 were eligible for qualitative synthesis utilizing R. Weekly updates since cutoff strengthen conclusions. RESULTS: Included were 887/14286 sources containing 12721 DLMs patients. Pathology involves the subarachnoid space (SAS) and pia. DLMs occurred in all countries as a contributor to the top 10 causes of disability-adjusted life years lost, with communicable diseases (CDs) predominating. In the USA, the ratio of CDs to iatrogenic causes is 2.4:1, contradicting arachnoiditis literature. Spinal fusion surgery comprised 54.7% of the iatrogenic category, with rhBMP-2 resulting in 2.4x more DLMs than no use (p<0.0001). Spinal injections and neuraxial anesthesia procedures cause 1.1%, and 0.2% permanent DLMs, respectively. Syringomyelia, hydrocephalus, and arachnoid cysts are complications caused by blocked CSF flow. CNS neuron death occurs due to insufficient arterial supply from compromised vasculature and nerves traversing the SAS. Contrast MRI is currently the diagnostic test of choice. Lack of radiologist recognition is problematic. DISCUSSION & CONCLUSION: DLMs are common. The LM clinically functions as an organ with critical CNS-sustaining roles involving the SAS-pia structure, enclosed cells, lymphatics, and biologic pathways. Cases involve all specialties. Causes are numerous, symptoms predictable, and outcomes dependent on time to treatment and extent of residual SAS damage. An international disease classification and possible treatment trials are proposed.

"PTFE Sleeve Graft" Technique to Remove Neurovascular Conflict in Micro Vascular Decompression for Trigeminal Neuralgia.

Background: Microvascular decompression (MVD) of the trigeminal nerve is a well-accepted nondestructive procedure for trigeminal neuralgia. Usually, Teflon (PTFE) puff or felt graft techniques, which are most commonly used, are associated with arachnoiditis and recurrence among other complications. We use the "sleeve graft" technique using PTFE to separate the neurovascular conflict and here we describe our experience with the same in 376 cases. Objectives: To study the outcomes in 376 patients treated with sleeve graft technique for trigeminal neuralgia. Materials and Methods: For a period of 18 years, from 2002 to 2020, all cases of medically refractory trigeminal neuralgia were subjected to the "sleeve graft" technique for MVD. Pre- and post-operatively, pain score was given according to Barrow Neurological Institute pain intensity score. Cases were observed for any complications and pain relief in short and long-term follow-up. Results: In total, 376 cases of refractory primary trigeminal neuralgia cases, among which 198 patients underwent MVD with no prior intervention, 158 underwent MVD following percutaneous ablative procedure, 13 were "Revision MVD" previously done at other centers, and four were post gamma knife failure. There was no incidence of arachnoiditis or recurrence of symptoms. Further, 368 (97.8%) patients had complete recovery from symptoms while eight (2.2%) had partial recovery after 5 years of follow-up. Complications included hearing loss (n = 1), temporary hypoesthesia (n = 45), and permanent hypoesthesia (n = 7). Conclusion: "PTFE Sleeve Graft" technique to remove the neurovascular conflict in micro vascular decompression (MVD) for trigeminal neuralgia is a safe and effective technique that yields better results.

Magendie's foramen debridement and catheterisation for the treatment of syringomyelia due to diffuse craniocervical junction arachnoiditis. A case report and technical note.

A 36 year old woman was referred to our department for symptomatic lumbar spinal arachnoiditis following an epidural anaesthesia for childbirth. She did not had other known causative factor and she was free of any neurological symptoms before. She rapidly developed lower limbs impairment by compressing intradural lumbar collections and arachnoiditis requiring surgical decompression and subsequently internal cerebrospinal fluid shunting for acute hydrocephalus. Three years and the half later, she developed a severe tetraparesis due to a massive syrinx consecutive to the fourth ventricle outlets obstruction cause by the ongoing diffuse craniocervical junction arachnoiditis. Our aim was to treat all the problems in one step. An open fourth ventriculostomy of the Magendie's foramen with catheter insertion from the fourth ventricle down to the upper cervical subarachnoid space improve both the patient status and imagery.

Cyclophosphamide therapy as an adjunct in refractory post-tubercular arachnoiditis.

INTRODUCTION: There is no satisfactory treatment for post tubercular arachnoiditis (TB arachnoiditis). We did this study to investigate the efficacy and safety of cyclophosphamide as adjuvant therapy for post TB arachnoiditis refractory to corticosteroids and anti-tubercular therapy (ATT). METHODS: This was a retrospective case series of patients of refractory post TB arachnoiditis leading to paraparesis and vision loss who received cyclophosphamide as an adjuvant therapy along with standard ATT and corticosteroids. These patients were treated with intravenous cyclophosphamide (dose 500 mg/m2) once a month for 4 consecutive months after informed written consent and were assessed clinically and radiologically before and after cyclophosphamide therapy. RESULTS: We had 4 patients with refractory post TB arachnoiditis of whom three became independently ambulatory. There was significant clinical as well as radiological improvement in all the patients. CONCLUSIONS: Cyclophosphamide therapy could be an effective therapy for patients with refractory post TB arachnoiditis. Well-designed randomized controlled studies are essential to study the safety and efficacy of cyclophosphamide in this condition.

A Case Series on Neuropathogenic Nature of an Ancient Disease ----Tuberculosis.

Tuberculosis is one of the oldest diseases known to affect humans and the top cause of infectious death worldwide caused by M. tuberculosis complex. Tuberculosis may be pulmonary, extra-pulmonary or both. Nervous system tuberculosis is relatively rare and has protean nature of symptoms so poses diagnostic difficulty. Neurological manifestations of tuberculosis includes 1) intracranial 2) spinal 3) peripheral nerve tuberculosis. Central nervous system tuberculosis accounts about 5% of extra pulmonary cases and 1% all tuberculosis. MATERIAL: Here we are presenting the series of 10 cases which have wide variety of neuropathogenic nature of tuberculosis. OBSERVATION: Here we are presenting the series of 10 cases which have wide variety of neuropathogenic nature of tuberculosis. These includes 1) Tubercular cortical vein thrombosis -patient who is a known case of pulmonary tuberculosis presented with severe headache, seizure and altered behavior, MRI brain shows cortical vein thrombosis and normal coagulation profile (Review of literature shows only 4 cases). 2)Tubercular myelitis/ arachnoiditis-presented with low backache and bilateral lower limb weakness,CSF panel and MRI L S spine shows tubercular arachnoiditis/myelitis. 3)Tubercular peripheral neuropathy; patient who is a non- diabetic presented with pain abdomen and bilateral lower limb tingling and numbness with no past history of treatment with anti-tubercular drug, CECT abdomen shows ileocecal tuberculosis and NCS study shows sensory affection of lower limb nerve. 4)Tuberculoma-patient presented with severe headache, seizure and altered behavior, MRI brain shows tuberculoma. 5) and 6) are tubercular vascular infarct in 1 of these 2 cases patient was having multiple necrotic foci and few foci of cavitation in left hilar region which is extending into left inferior pulmonary vein and even reaching upto left atrium. 7) and 8) cases are pott's spine who presented with low backache. 9) and 10) are tubercular meningitis and tubercular meningitis with hydrocephalus respectively. These patients were treated according to their diagnosis and for focal neurological deficit physiotherapy was advised. Except a case of septic foci emboli from left atrium which shows moderate recovery rest all cases shows good recovery at discharge. CONCLUSION: There is paucity of literature on neuropathogenic nature of tuberculosis. In this case series we are presenting the series of 10 cases of tubercular nervous system manifestations so that it will helps to diagnose the disease as early as possible and allows us to initiate the prompt treatment so that we can mitigate the significant morbidity and mortality among survivors of nervous system tubercular disease.

CNS cryptococcosis presenting with cerebellar stroke and spinal arachnoiditis.

A 67-year-old man presented with a low-grade fever for 2 months, weakness of all four limbs for five days and altered sensorium for two days. He was recently diagnosed with AIDS and was treatment-naive. Investigations revealed a CD4 count of 27cells/mm3 MRI brain and spine exhibited bilateral cerebellar lesions with diffusion restriction, and severe arachnoiditis at the level of the lumbar spine. High suspicion of central nervous system tuberculosis in an endemic country like ours, led us to start antitubercular therapy and steroids. Repeated lumbar punctures resulted in a dry tap leading to a delay in diagnosis. Serum cryptococcal antigen detection came positive, following which antifungal treatment was initiated. Later a small amount of cerebrospinal fluid sample was obtained which confirmed the diagnosis of cryptococcosis. However, the patient worsened and succumbed to the illness. This case highlighted the rare presentation of cryptococcal cerebellar stroke and spinal arachnoiditis.