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.

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.

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.

A new surgical method for treating syringomyelia secondary to arachnoiditis following cervical spine surgery: the syringo-cisterna magna shunt.

PURPOSE: The 5-year postoperative failure rate of conventional shunt treatment for syringomyelia is 50%, with arachnoditis, shunt obstruction, and shunt malfunction being the most common causes. We report a new syringo-cisterna magna (SCM) shunt that allows syrinx cerebrospinal fluid (CSF) drainage normally into the cerebellomedullary cisterns through the subarachnoid space. METHODS: Between November 2012 and February 2017, six patients (mean age: 57.25 years; sex: four male and two female) received the SCM shunt. They had spinal cord injury, abscess formation after a spine operation, and cerebral meningitis-related syringomyelia (syrinx between C0 and T9), and presented sensory changes and motor weakness. Preoperatively and at 1 year postoperatively, the syrinx length and diameter were assessed using magnetic resonance imaging (MRI). Clinical outcomes were evaluated using the visual analog scale (VAS) and Japanese Orthopedic Association (JOA) scores. RESULTS: Motor weakness improved, pain subsided, and sensory disturbance resolved in all patients who returned to work within 6 weeks postoperatively. In all cases, the syrinx collapsed (length: 3.3 levels decreased; diameter: decreased from 7.90 to 4.64 mm, p = 0.046) on postoperative MRI. No patient experienced syrinx recurrence and shunt malfunction on MRI or showed spinal instability signs on plain radiography. The VAS (pre- vs post-shunt: 6.50 vs 3.83, p = 0.027) and JOA scores (pre- vs post-shunt: 10.00 vs 11.17, p = 0.167) were improved postoperatively. CONCLUSION: We developed a new shunting system allowing syrinx CSF drainage to the posterior fossa, with symptomatic improvement, minimal complications, and syrinx decrease on follow-up MRI. The SCM shunt is effective for treating syringomyelia.

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.

MRI Findings of Arachnoiditis, Revisited. Is Classification Possible?

BACKGROUND: Prior imaging studies characterizing lumbar arachnoiditis have been based on small sample numbers and have reported inconsistent results. PURPOSE: To review the different imaging patterns of lumbosacral arachnoiditis, their significance, and clinical implications. STUDY TYPE: Retrospective. POPULATION: A total of 96 patients (43 women; average age 61.3 years) with imaging findings of arachnoiditis (postsurgical: N = 49; degenerative: N = 29; vertebral fracture: N = 6; epidural and subdural hemorrhage: N = 3, infectious: N= 1; other: N = 8) from January 2009 to April 2018. FIELD STRENGTH/SEQUENCE: Sagittal and axial T2-weighted Turbo Spin Echo at 1.5 T and 3 T. ASSESSMENT: Chart review was performed to assess the cause of arachnoiditis, and imaging was reviewed by two musculoskeletal and three neurology radiologists, blinded to the clinical data and to each other's imaging interpretation. Previous classification included a three-group system based on the appearance of the nerve roots on T2-weighted images. A fourth group was added in our review as "nonspecified" and was proposed for indeterminate imaging findings that did not fall into the classical groups. The presence/absence of synechiae/fibrous bands that distort the nerve roots and of spinal canal stenosis was also assessed. STATISTICAL TESTS: The kappa score was used to assess agreement between readers for both classification type and presence/absence of synechiae. RESULTS: Postsurgical (51%) and degenerative changes (30%) were the most common etiologies. About 7%-55% of arachnoiditis were classified as group 4. There was very poor classification agreement between readers (kappa score 0.051). There was also poor interreader agreement for determining the presence of synechiae (kappa 0.18) with, however, strong interreader agreement for the presence of synechia obtained between the most experienced readers (kappa 0.89). DATA CONCLUSION: This study demonstrated the lack of consensus and clarity in the classification system of lumbar arachnoiditis. The presence of synechia has high interreader agreement only among most experienced readers and promises to be a useful tool in assessing arachnoiditis. EVIDENCE LEVEL: 3 TECHNICAL EFFICACY: Stage 2.

Arachnoiditis, a complication of epidural blood patch for the treatment of low-pressure headache: A case report and systematic review.

OBJECTIVE: To report a case of arachnoiditis as a complication of epidural blood patch procedures and to systematically review the diagnostic workup, clinical outcomes, and treatment modalities reported in the literature. BACKGROUND: Epidural blood patching is an effective treatment for low-pressure headache secondary to spontaneous cerebrospinal fluid leak or iatrogenic post-dural puncture. Spontaneous intracranial hypotension is believed to be a rare headache disorder, but recently has been diagnosed at higher frequencies, making it an important differential diagnosis for intractable headaches. Arachnoiditis has surfaced as a rare complication of epidural blood patching. Symptom presentation does not always correlate with evidence of meningeal enhancement on imaging. Optimal methods for treatment remain largely unknown. METHODS: Databases Embase and PubMed were searched for all published studies on arachnoiditis post-epidural blood patch using a combination of the following medical subject headings and keywords: arachnoiditis, arachnoid inflammation, adverse event, and epidural blood patch. All original English-language articles that described arachnoid and/or meningeal inflammation in conjunction with epidural blood patch procedures were included for analysis. Title and abstract screening, data extraction, and risk of bias assessment were conducted independently and in duplicate by two reviewers. RESULTS: Seven other cases of arachnoiditis post-blood patch placement have been documented, most of which were diagnosed via magnetic resonance imaging. Six of these were a result of a spinal-epidural anesthesia for labor and delivery. Common symptoms reported were headache, back and radicular pain, paresthesia, and motor weakness. There are currently no proven consensus-based treatment recommendations available. While intravenous methylprednisolone followed by oral prednisone taper was found to be effective in the case presented, the benefit of other multi-modal therapies was unclear. CONCLUSIONS: Headache specialists who treat postural headache should be aware of arachnoiditis as a potentially severe complication of epidural blood patch. The case presented is the first of its kind to report arachnoiditis as a complication of high-volume blood patch for the treatment of spontaneous intracranial hypotension. More studies are required to determine suitable treatment options for post-epidural blood patch arachnoiditis.

MRI characteristics of syringomyelia associated with foramen magnum arachnoiditis: differentiation from Chiari malformation.

BACKGROUND: It is important to distinguish foramen magnum arachnoiditis (FMA) from Chiari malformation (CM) before surgery because the operative strategies for these diseases differ. In the current study, we compared pretreatment magnetic resonance imaging (MRI) of FMA with CM and investigated the MRI findings useful to differentiate between these diseases. METHODS: We retrospectively reviewed patients with FMA or CM aged ≥ 18 years who underwent surgeries at our institution between 2007 and 2019. The morphologies of the syrinx, neural elements, and posterior cranial fossa were preoperatively evaluated with MRI. We used the receiver operating characteristic (ROC) curve for the fourth ventricle-to-syrinx distance (FVSD). RESULTS: Ten patients with FMAs and 179 with CMs were included. FVSD in the FMA group was significantly shorter than that in the CM group (7.5 mm [IQR, 2.8-10 mm] in FMA vs. 29.9 mm [IQR, 16.3-52.9 mm] in CM, p < 0.0001). The other MRI findings that showed the height, size, and length of the syrinx; size of the foramen magnum; degree of cerebellar tonsillar descent; shape of the cerebellar tonsil; and dorsal subarachnoid space at the foramen magnum differed significantly between the two groups. The ROC curve analysis showed that patients whose FVSD was less than 11 mm could be diagnosed with FMA with a specificity of 90% and sensitivity of 96%. CONCLUSIONS: A more cranial syrinx development (FVSD < 11 mm) appears to be the characteristic MRI finding in FMA.

[Arachnoiditis following spinal anesthesia-Case report and review of the literature]. / Arachnoiditis nach Spinalanästhesie ­ Fallbericht und Literaturübersicht.

A 61-year-old woman underwent a tension-free vaginal tape (TVT) operation due to stress incontinence. After technically difficult spinal anesthesia with two attempts the patient developed symptoms of nerve irritation, complained about neckache and headache and showed signs of agitation. The regimen was shifted to general anesthesia and surgery was performed. Because of postoperatively persistent headache and sensory disturbances an MRI scan of the lumbar spine was performed on the first postoperative day without pathological findings. The patient was able to leave the hospital after 1 week with significant relief of symptoms but 3 weeks later she developed neurocognitive impairment with memory deficits. A second MRI scan of the head now showed signs of disturbance of CSF circulation with hydrocephalus. Treatment was performed with drainage and ventriculoperitoneal shunt. Further evaluation showed a severe, multisegmental arachnoiditis and the patient developed a progressive paraparesis. The patient presented her case for assessment to a commission on medical malpractice 13 months after anesthesia. The commission detected no treatment errors. In connection to the case report a literature review of characteristics and etiologies of chronic adhesive arachnoiditis is given, which is a known but very rare complication of spinal anesthesia or similar procedures.

Subarachnoid neurocysticercosis: emerging concepts and treatment.

PURPOSE OF REVIEW: Subarachnoid neurocysticercosis (SUBNCC) is caused by a morphologically unique proliferative form of Taenia solium involving the subarachnoid spaces. Prolonged therapy based upon the pathophysiology of SUBNCC and long-term follow-up have shed light on the course of disease and led to highly improved outcomes. RECENT FINDINGS: SUBNCC has a prolonged incubation period of between 10 and 25 years characterized by cyst proliferation and growth and invasion of contiguous spaces leading to mass effect (Stage 1). With induction of the host-immune responses, cysts degenerate leading to a predominately inflammatory arachnoiditis (Stage 2) causing hydrocephalus, infarcts, and other inflammatory based neurological manifestations. Inactive disease (Stage 3) may occur naturally but mostly is a result of successful treatment, which generally requires prolonged intensive anthelminthic and antiinflammatory treatments. Cerebral spinal fluid cestode antigen or cestode DNA falling to nondetectable levels predicts effective treatment. Prolonged treatment with extended follow-up has resulted in moderate disability and no mortality. Repeated short intensive 8-14-day courses of treatment are also used, but long-term outcomes and safety using this strategy are not reported. SUMMARY: SUBNCC gives rise to a chronic arachnoiditis. Its unique ability to proliferate and induce inflammatory responses requires long-term anthelmintic and antiinflammatory medications.

A Novel Technique Using Magnetic Resonance Imaging in the Supine and Prone Positions for Diagnosing Lumbar Adhesive Arachnoiditis: A Preliminary Study.

BACKGROUND: Lumbar adhesive arachnoiditis is a debilitating neuropathic condition and is difficult to diagnose owing to lack of definitive diagnostic criteria. By focusing on the intrathecal mobility of nerve roots, we assessed whether useful diagnostic criteria could be established using MRI. METHODS: Seventeen patients with a high risk for lumbar adhesive arachnoiditis and 18 no-risk patients with chronic low back pain and/or leg pain participated in this study. The patients underwent MRI in both the supine and prone positions. Eleven axial T2-weighted images between the L2 and L5/S levels were obtained, and the proportion of the low-intensity area in the dorsal half to the total low-intensity area in the dural sac was calculated for each axial view. RESULTS: At some lumbar levels, the low-intensity area in the dorsal half of the dural sac was relatively larger in patients with a high risk for lumbar adhesive arachnoiditis than in the no-risk patients. In the no-risk group, the proportion of the low-intensity area in the dorsal half in the supine position was significantly higher than that in the prone position at all lumbar levels. However, in the high-risk group, at some levels, the proportions were not significantly different in the dorsal half of the dural sac between the supine and prone positions. CONCLUSION: In patients with a known risk for lumbar adhesive arachnoiditis, nerve roots lose their potential to migrate in the dural sac in the gravitational force direction on MRI.

Arachnoiditis as an outcome factor for microvascular decompression in classical trigeminal neuralgia.

BACKGROUND: Neurovascular conflict is considered a key element of classical trigeminal neuralgia (TN) and consequently, microvascular decompression (MVD) is an effective treatment. Nevertheless, failures of MVD are described by many authors. In some patients, the arachnoid membranes surrounding the trigeminal nerve and neighbouring vessels may be thickened and adhesive. Here we analyse the impact of such focal arachnoiditis on outcome after MVD for TN. METHODS: A cohort of prospectively followed patients after their MVD was reviewed for intraoperative, imaging and clinical data if findings of arachnoiditis during MVD were described. Long-term outcome assessment was the main endpoint. RESULTS: We reviewed data from 395 MVD procedures, performed for TN from 2001 to 2014. Intraoperative evidence of focal arachnoiditis, as described by the surgeon, has been noted in 51 patients (13%). In 35 (68.6%), neuralgia was typical and in the other 17 (31.4%) it was atypical. As expected by definition, neurovascular conflict was found in 49 interventions (96%); it was predominantly arterial in 27 (52.9%). Accompanying arachnoiditis was encountered: mild in 20 interventions (39.2%), severe in 31 (60.8%). A successful result (BNI I or II) was achieved in 29 patients (56.9%). The other 22 patients (43.1%) had persistence or recurrence of pain. Overall KM probability of being pain free at 15 years was 72%. CONCLUSIONS: Intraoperative finding of arachnoiditis during MVD for classical trigeminal neuralgia is associated with poorer outcome than that of classical trigeminal neuralgia in general. This is particularly true for low grades of conflict.

Arachnoiditis ossificans associated with syringomyelia: a case report.

The association of arachnoiditis ossificans with syringomyelia is a rare pathological entity. We present an unusual case who presented with progressive myelopathy caused by arachnoidits ossificans and syringomyelia. The pathophysiology and treatment strategy of this rare entity are still controversial.

Minimally invasive asterional approach for microvascular decompression in trigeminal neuralgia.

OBJECTIVE: Trigeminal neuralgia (TN) is a neuropathic disorder that can be treated surgically. This study aimed to present the surgical findings and the clinical outcomes of 26 patients with TN treated by minimally invasive asterional surgery. METHODS: Longitudinal descriptive study. Twenty-six patients with TN underwent minimally invasive asterional surgery. The medical history, surgical findings, therapeutic response, and complications were registered. They were followed for 36 months. RESULTS: Nineteen cases were associated with vascular compression; five were associated with arachnoiditis. The two remaining cases were associated with multiple sclerosis and post-herpetic neuralgia. The pain was substantially reduced in all patients in the immediate postoperative period. At 36 months, in 25 patients, total or acceptable pain control was achieved. In the long term, 22 patients evolved with no permanent complications. CONCLUSION: The microvascular decompression surgery by an asterional approach is an alternative with similar results to the classic retrosigmoid approach to treat TN, but that adds the benefits of the principles of minimally invasive surgery. Constant efforts need to be made to optimize minimally invasive surgical techniques for TN.

Corrosion of Harrington rod in idiopathic scoliosis: long-term effects.

PURPOSE: Metal implants have been used to treat adolescent idiopathic scoliosis since the 1960s. Only recently, however, it has the issue of metal-bone breakdown secondary to metal corrosion in situ come to light, raising concerns of possible long-term complications from the resulting metallosis and inflammation of spinal tissues. We present a case of a patient with neurological deficit, pain, and disability with Harrington rod in place for over 30 years, to bring attention to the issue of bio-corrosion of metal implants and its effect on human tissue. We call attention to the need for protocols to better diagnose and treat these patients. METHODS: We provide a complete review of the history and clinical manifestations as well as serum metal, X-ray, and CT/myelogram test results. RESULTS: A 52-year-old female with spinal fusion and Harrington rod presents with pain, lymphedema, disability, and neurological deficits including thoracic outlet syndrome, hyperreflexia, peripheral muscle weakness and atrophy, hypertonicity, Raynaud's phenomenon, and balance and gait abnormalities. Serum chromium levels were elevated (26.73 nmol). X-rays showed no evidence of rod breakdown. Serial X-rays can demonstrate subtle corrosive changes but were not available. Adhesive arachnoiditis was diagnosed via CT/myelogram. CONCLUSION: We hypothesize that bio-corrosion is present in this case and that it is associated with intraspinal metallosis. Trauma secondary to a motor vehicle accident, as well as arachnoiditis, and their possible effects on this case are outlined. Challenges in proper diagnosis and management are discussed.

[Treatment of patients with ossifying arachnoiditis and progressive syringomyelia (analysis of three cases and a literature review)]. / Lechenie patsientov s ossifitsiruiushchei arakhnopatiei i progressiruiushchei siringomieliei (analiz trekh sluchaev i obzor literatury).

To date, only 12 cases of ossifying arachnoiditis with concomitant syringomyelia have been reported in the world literature. In this article, we presented our experience of treating three patients with syringomyelia associated with ossifying arachnoiditis. OBJECTIVE: based on the literature data and our own experience, we tried to define the most optimal treatment approach for this disease. MATERIAL AND METHODS: We analyzed the data of 15 patients (12 cases reported in the literature and 3 our own cases). The diagnosis was established on the basis of complaints, medical history, patient examination, MRI and CT of the spinal cord, and intraoperative data. Progression of syringomyelia was evaluated by comparison of the neurological status and calculation of the Vaquero index before and after surgery; the Domenicucci classification was used to evaluate the severity of ossifying arachnoiditis. Surgical treatment included several stages: decompressive laminectomy, elimination of spinal cord fixation, excision of ossifying arachnoiditis, shunting of the syrinx, and repair of the dura mater. RESULTS: An analysis of the patient data reported in the literature (12 cases) and those in our series (3) revealed that in the early postoperative period, 7 (46.7%) of these 15 patients had improvements, 5 (33.3%) patients had no changes, and 3 (20%) patients experienced a worsening. At present, there is no clear algorithm for diagnosing and treating this disease. CONCLUSION: Further investigation of the problem may facilitate an earlier diagnosis of the disease and provide more effective medical care.

Spinal Arachnoiditis as a Complication of Cryptococcal Meningoencephalitis in Non-HIV Previously Healthy Adults.

BACKGROUND: Cryptococcus can cause meningoencephalitis (CM) among previously healthy non-HIV adults. Spinal arachnoiditis is under-recognized, since diagnosis is difficult with concomitant central nervous system (CNS) pathology. METHODS: We describe 6 cases of spinal arachnoiditis among 26 consecutively recruited CM patients with normal CD4 counts who achieved microbiologic control. We performed detailed neurological exams, cerebrospinal fluid (CSF) immunophenotyping and biomarker analysis before and after adjunctive immunomodulatory intervention with high dose pulse corticosteroids, affording causal inference into pathophysiology. RESULTS: All 6 exhibited severe lower motor neuron involvement in addition to cognitive changes and gait disturbances from meningoencephalitis. Spinal involvement was associated with asymmetric weakness and urinary retention. Diagnostic specificity was improved by MRI imaging which demonstrated lumbar spinal nerve root enhancement and clumping or lesions. Despite negative fungal cultures, CSF inflammatory biomarkers, sCD27 and sCD21, as well as the neuronal damage biomarker, neurofilament light chain (NFL), were elevated compared to healthy donor (HD) controls. Elevations in these biomarkers were associated with clinical symptoms and showed improvement with adjunctive high dose pulse corticosteroids. CONCLUSIONS: These data suggest that a post-infectious spinal arachnoiditis is an important complication of CM in previously healthy individuals, requiring heightened clinician awareness. Despite microbiological control, this syndrome causes significant pathology likely due to increased inflammation and may be amenable to suppressive therapeutics.