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.

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.

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.

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 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.

Spinal Syrinx Due to Lipiodol-Induced Arachnoiditis.

We present a case of a progressive symptomatic intramedullary cyst, diagnosed decades after Lipiodol injection. Lipiodol was originally administered intrathecally for the radiologic diagnosis of spinal masses. A link between the lesion and the history of Lipiodol injection was never suspected. Surgical exploration revealed a membrane above the lesion, separating the intradural space in a cranial and caudal compartment. On the level of the cyst, we identified glassy pearls containing a fatty liquid, compatible with Lipiodol deposits. We hypothesize that the syrinx is secondary to the impact of cerebrospinal fluid pulsations on the reactive membrane and that this membrane originated from an arachnoiditis caused by Lipiodol deposits. Lipiodol was indeed abandoned after it was found to cause arachnoiditis and neurologic sequelae. Despite the cessation of its usage, the causal role of Lipiodol in arachnoiditis and spinal cyst formation should still be considered, as symptoms may arise many years after Lipiodol administration.

Arachnoiditis Ossificans of the Lumbar Spine: A Rare Cause of Progressive Cauda Equina Syndrome.

Arachnoiditis ossificans of the spine is a rare entity defined as an ossification of the leptomeninges resulting in neurologic decline. We describe the case of a 42-year-old woman, without any obvious predisposing factor, who presented with a progressive cauda equina syndrome. The imaging findings on magnetic resonance imaging were confusing by showing an atypical intraspinal lesion extending from L1 to S1. The computed tomography scan was more specific by showing suggestive images of a huge arachnoiditis ossificans of the lumbar spine. The patient underwent a large lumbar laminectomy with an incomplete resection of the ossified arachnoid. The histologic study confirmed the bony nature of the lesion. This illustrative case highlights the importance of helical computed tomography scan with multiplanar reconstruction for the diagnosis of arachnoiditis ossificans.

Spinal adhesive arachnoiditis: three case reports and review of literature.

Spinal adhesive arachnoiditis is a rare pathology involving pia mater of the spinal cord and nerve roots. It can potentially lead to disability-many patients end up wheelchair-bound due to subsequent paraparesis. It is an infrequent but possible cause of lower extremities weakness in patients with a history of spinal surgery, epidural anaesthesia, myelography or spinal tumors. Three patients, one male and two females, admitted to our unit due to paraparesis presented at least one of the above mentioned risk factors. Each of them had a severe course of illness-progressive paresis of lower extremities. All above cases were diagnosed with spinal adhesive arachnoiditis confirmed with Magnetic Resonance Imaging (MRI) scan-the most sensitive and specific diagnostic tool. Despite conservative treatment and intensive rehabilitation none of the presented patients preserved the ability to mobilise independently. Considering spinal adhesive arachnoiditis in patients with paraparesis and history of typical risk factors should be included in clinical diagnostic procedure.

Hemifacial Spasm Secondary to Arachnoiditis Due to Neurocysticercosis: Clinical Image.

Subarachnoid neurocysticercosis (NCC) is a form of NCC with cysticerci located in the subarachnoid space. This form of NCC can cause general and focal neurologic symptoms, and sometimes requires surgical intervention as a treatment. In this report, we present a rare case of hemifacial spasm secondary to arachnoiditis because of an NCC cyst in the cerebellopontine angle. The cysticercus was removed and the facial nerve was liberated via a retrosigmoidal approach. At 8-month follow-up, the patient reported no recurrence of symptoms. To our knowledge, this is the first case reported to surgically manage hemifacial spasm secondary to NCC arachnoiditis.

Compressive myelopathy secondary to posthemorragic arachnoiditis: Case report and literature review.

BACKGROUND: Spinal arachnoiditis is an arachnoid inflammatory process frequently caused by infection or spinal surgery; there are different degrees of severity, including arachnoid thickening and severe adhesive lesions that can lead to the development of arachnoid cysts. Non-traumatic subarachnoid haemorrhage (ntSAH) is a relatively uncommon cause of arachnoiditis; further complication with spinal cord compression (SCC) is even more unusual. METHOD: we describe a 70-year-old female, with SCC caused by arachnoid cysts. Her medical past history was relevant for an episode of ntSAH after rupture of a posterior communicating artery aneurysm, eight months prior to the onset of symptoms. We also present a literature review of previous published cases. RESULTS: we selected 23 articles with 24 case reports. A noticeable female predominance (11:1) was observed. It is more common between the fourth and fifth decades. The majority of cases (58 %) were secondary to aneurysmal SAH due to rupture of a posterior circulation aneurysm. The most common location of the cyst is in the cervicothoracic spine. The average time between the initial bleeding and symptom development is 3-6 months. The most frequently described treatment is laminectomy and marsupialization of the cyst, but reports show a high recurrence rate. CONCLUSIONS: ntSAH is an uncommon aetiology of arachnoiditis and arachnoid cysts. SCC from arachnoid cysts secondary to ntSAH is exceptional. Treatment through laminectomy has a relatively high recurrence rate (33 %). We present different hypotheses to try to explain how the alteration of cerebrospinal fluid (CSF) dynamics after ntSAH can lead to arachnoid cyst development and SCC. Although the small number of cases included in the present series precludes us to draw definite conclusions, ventriculoperitoneal shunt (VPS) placement can be considered as an alternative treatment in the management of known ntSAH patients that present recurrent symptomatic arachnoid cysts.