Intravenous methadone causes acute toxic and delayed inflammatory encephalopathy with persistent neurocognitive impairments.

BACKGROUND: The mu-opioid agonist methadone is administered orally and used in opioid detoxification and in the treatment of moderate-to-severe pain. Acute oral methadone-use and -abuse have been associated with inflammatory and toxic central nervous system (CNS) damage in some cases and cognitive deficits can develop in long-term methadone users. In contrast, reports of intravenous methadone adverse effects are rare. CASE PRESENTATION: Here, we report a patient who developed acute bilateral hearing loss, ataxia and paraparesis subsequently to intravenous methadone-abuse. While the patient gradually recovered from these deficits, widespread magnetic resonance imaging changes progressed and delayed-onset encephalopathy with signs of cortical dysfunction persisted. This was associated with changes in the composition of monocyte and natural killer cell subsets in the cerebrospinal fluid. CONCLUSION: This case suggests a potential bi-phasic primary toxic and secondary inflammatory CNS damage induced by intravenous methadone.

Turbulent Cerebrospinal Fluid Flow in Enlarging Terminal Myelocystocele.

Terminal myelocystocele is a rare type of neural tube malformation, consisting of a skin-covered lumbosacral mass, highly associated with other complex abdominal malformations within the OIES complex (omphalocele, imperforate anus, exstrophy of the cloaca and spinal defects). We present a case of a premature female with an extensive lumbosacral mass at birth, as well as an omphalocele, cloacal exstrophy, renal abnormalities, and sacral agenesis. Lumbar magnetic resonance imaging revealed a meningocele sac herniating through the bone defects and containing a syringocele sac. Advanced imaging techniques showed turbulent cerebrospinal fluid flow. At control, 4 weeks later, the defect doubled in size. The myelocystocele sac was evacuated and closed, and the patient persisted with paraparesis. The role of cerebrospinal fluid flow analysis is well established in Chiari-type malformations, in which turbulent flow within the syrinx is related to a better outcome after surgery. It is possible that the same principle could be applied to other spinal malformations, as shown in this case of terminal myelocystocele.

An institutional review of 10 cases of spinal hemangiopericytoma/solitary fibrous tumor.

BACKGROUND: Spinal hemangiopericytoma is very rare tumors with only a few case reports and one case series. We have treated ten patients between 2004 and 2017 and, thus, present a retrospective review of our patients with a focus on clinical presentation, radiological features, management, pathology, and outcome. MATERIALS AND METHODS: Histopathological data were reviewed in all the cases and clinical and follow-up details were collected from data available in our department. RESULTS: There were five males and five females, including one pediatric patient. The mean age of the patients was 34.7 years (Range 12-52 years). Dorsal, cervical, and lumbar spine involvement were found in five, four, and one patient, respectively. Intradural extramedullary tumor was the most common tumor and all patients presented motor weaknesses. Gross total resection of the tumor was done in seven patients and six patients received postoperative radiotherapy. Histopathology showed anaplastic tumor in two cases with high MIB-1 labelling index. Most patients were positive for CD34, vimentin, mic-2, and bcl-2. While the seven patients who underwent gross total resection improved significantly and were self-ambulatory in the follow-up period, two patients who underwent subtotal resection expired due to tumor metastasis. CONCLUSION: Spinal hemangiopericytoma is a very rare tumor. We present a series of cases treated at our institute for the same. Gross total resection is the goal and radiotherapy should be given in case of residual tumor or high-grade tumors. Prognosis is good after gross total excision and functional recovery can be expected in most patients.

Primary Lumbar Paraganglioma: Clinical, Radiologic, Surgical, and Histopathologic Characteristics from a Case Series of 13 Patients.

BACKGROUND: Paragangliomas are uncommon neuroendocrine tumors, rarely occurring in the lumbar spine. Primary lumbar paragangliomas are prominently vascularized, can present variably, and pose both diagnostic and surgical challenges. We report on a large case series with long-term follow-up and intraoperative footage to characterize the natural history, diagnostic approach, and operative approach to this rare surgical disease. METHODS: This is a single-center, retrospective cohort study including all patients with histologically confirmed primary lumbar paraganglioma treated at our tertiary neurosurgical center between 1997 and 2018. Clinical, radiologic, surgical, and histologic data were collected from medical records. RESULTS: There were 13 cases of primary lumbar paraganglioma (8 men [61.5%], 5 women [38.5%]; mean age, 51.3 years; range, 33.2-68.9 years). Symptom duration correlated with tumor size (Spearman r = 0.735, P = 0.01). The main presenting symptoms were lower back pain and radiculopathy, often long-standing with recent deterioration. Seven patients (53.8%) were admitted as emergency cases, including 3 with cauda equina syndrome. Preoperative differential diagnoses included nerve sheath tumor, ependymoma, meningioma, and disk herniation. The mean Ki-67 mitotic index was 5.7% (range, 1%-10%). Surgical resection improved pain in 8 of 13 patients (61.5%) and weakness improved in 5 of 5 patients (100%). CONCLUSIONS: Primary lumbar paragangliomas are rare neoplasms of the cauda equina that typically progress slowly but may also present acutely. They are often related to the filum terminale, which should be resected prior to other attachments intraoperatively to prevent displacement of the tumor out of view. Total resection can be curative, and long-term follow-up in this series found no recurrence.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin (POEMS) changes syndrome presenting with a pseudosensory level: a case report.

INTRODUCTION: Polyneuropathy is a key feature of polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome, which is a paraneoplastic manifestation of an underlying lymphoproliferative neoplasm. We report the first case of polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome presenting with a pseudosensory level. CASE PRESENTATION: A 59-year-old Tamil woman with long-standing diabetes mellitus and hypertension developed painless, progressive inguinal lymphadenopathy. A contrast-enhanced computed tomography scan showed mild hepatomegaly and intra-abdominal lymphadenopathy. A histological examination of an enlarged inguinal lymph node showed features of a plasma cell-type Castleman disease. She was treated with rituximab. Six months later, she developed gradually ascending numbness and weakness of both lower limbs. On examination, she had flaccid paraparesis (power 3/5) with a sensory level to pinprick at thoracic level 9. Joint position sense was preserved. Her cranial nerves and upper limbs were neurologically normal. Nerve conduction studies confirmed peripheral neuropathy with conduction slowing and a magnetic resonance imaging of her spine did not show cord or root compression. Serum protein electrophoresis showed a monoclonal band. A bone marrow biopsy showed a hypercellular marrow with 30% plasma cells. A repeat contrast-enhanced computed tomography scan showed sclerotic bony lesions involving multiple vertebrae in addition to mild hepatomegaly and intra-abdominal lymphadenopathy. Polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome was diagnosed and she was treated with intravenously administered pulse therapy of dexamethasone and cyclophosphamide. After three cycles of treatment, she regained normal muscle power and sensation. CONCLUSIONS: Polyneuropathy in polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome can present as a pseudosensory level.

Transradial approach in the treatment of a sacral dural arteriovenous fistula: a technical note.

Sacral dural arteriovenous fistulas (SDAVFs) are rare, constituting no more than 10% of all spinal dural fistulas. They are most commonly fed by the lateral sacral artery (LSA), a branch of the internal iliac artery (IIA). Catheterization of this vessel requires either a crossover at the aortic bifurcation in cases of right femoral access or retrograde catheterization from the ipsilateral common femoral artery. We present the case of a 79-year-old man with tethered cord syndrome and a symptomatic SDAVF fed by two feeders from the left LSA. Spinal diagnostic angiography was made exceptionally challenging by an aorto-bi-iliac endograft, and selective catheterization of the left IIA was not possible. The patient could not undergo surgery due to multiple comorbidities, therefore embolization was considered the best approach. The procedure was carried out through a transradial access (TRA) with Onyx and n-butyl cyanoacrylate. The SDAVF was successfully treated and the patient made a full neurological recovery.

Acute Paraparesis Due to Protrusion of a Disc Following Lateral Interbody Fusion for Degenerative Kyphoscoliosis: A Case Report.

CASE: A 74-year-old woman presented with severe trunk deformity. Radiographs revealed severe sagittal and coronal imbalance with spinal canal stenosis at L4 to L5. Anterior cages were placed at L2 to L3, L3 to L4, and L4 to L5. Three days later, posterior correction surgery from T5 to the ilium with decompression at L4 to L5 was performed. At 30 minutes after surgery, leg muscle strength severely deteriorated. Emergency surgery revealed disc fragments protruding into the spinal canal at L2 to L3. CONCLUSION: Because posteriorly placed extreme lateral interbody fusion (XLIF) cages can be a risk factor for disc protrusion into the spinal canal, computed tomographic evaluation or prophylactic posterior decompression should be considered before the correction procedure.

Intervertebral disc herniation in two coatis (Nasua nasua) and postoperative laminectomy membrane formation.

Magnetic resonance imaging revealed spinal cord compression due to intervertebral disc herniation of Hansen type I and II in the thoracolumbar vertebral column in two middle-aged coatis (Nasua nasua) with chronic progressive paraparesis. Surgical treatment included hemilaminectomy and partial corpectomy in one and dorsal laminectomy in the other coati. Both coatis recovered well after surgery. One showed unremarkable gait 6 and 15 months post surgery, while the other one suffered from recurrence of paraparesis leading to euthanasia because of deterioration of neurological signs 20 months after the first surgery. Necropsy revealed formation of a laminectomy membrane compressing the spinal cord. Histopathological signs of spinal cord injury and findings of degenerative processes in the intervertebral disc were comparable to those described in dogs. In conclusion, this case report shows for the first time that surgical intervention seems to be a useful and safe treatment in chronic intervertebral disc herniation in coatis, but relapses are possible.

ANIMAL TRAINING AND ACUPUNCTURE IN A BENGAL TIGER ( PANTHERA TIGRIS TIGRIS) WITH HIND LIMB PARAPARESIS.

An 18-yr-old, captive-born male Bengal tiger ( Panthera tigris tigris) presented a 1-yr history of chronic and progressive paraparesis in both hind limbs. Lateral and hind limb radiographs were revealed normal except for severe spondylosis deformans, forming a bony bridge between the last lumbar and the first sacral vertebra and disc mineralization between the second and third lumbar vertebra. Medical therapies were instituted, including corticosteroids, hydroacupuncture and electroacupuncture. Animal training allowed veterinarians to perform acupuncture safely without having to anesthetize the animal. Animal training made intensive treatment possible. Neither corticosteroids nor hydroacupuncture alone provided much clinical improvement. The tiger reacted positively after electroacupuncture was performed. The tiger began showing clinical improvement after three electroacupuncture treatments and could eventually walk on all four limbs at the end of the treatment.

Visual feedback during pedaling allows individuals poststroke to alter inappropriately prolonged paretic vastus medialis activity.

Individuals who have experienced a stroke often demonstrate inappropriate muscle activity phasing in the paretic leg during locomotion. Past research has demonstrated that inappropriate paretic phasing varies between behavioral contexts and is reduced during unilateral pedaling with the nonparetic leg inactive. We investigated whether individuals could voluntarily alter activity in a target muscle of the paretic limb in a consistent behavioral context and whether this voluntary change differed between bilateral and unilateral pedaling. During a fixed-speed motorized pedaling task, participants were asked to use visual feedback to deactivate the vastus medialis (VM) before a 90° target region of the pedaling cycle, as measured by surface electromyography and by change in fraction of total cycle amplitude in the target region. We based the start of this target region on the earliest observed deactivation for this muscle (found in fast pedaling), which allowed us to challenge both the paretic and nonparetic VM. During visual feedback, participants significantly reduced the fraction of activity found in the target region, with no significant difference in degree of reduction between paretic and nonparetic legs or between bilateral and unilateral pedaling. Surprisingly, in bilateral pedaling, individuals with greater clinical impairment demonstrated greater paretic limb response to feedback. Our results demonstrated that during this tightly constrained task, the paretic VM showed a surprisingly similar flexibility of muscle activity to the nonparetic VM. Our findings show that participants were able to use provided visual feedback to modulate the degree of an observed poststroke muscle-phasing impairment. NEW & NOTEWORTHY This study demonstrates that by using visual feedback during a constrained task with minimized kinematic control requirements, participants with poststroke hemiplegia can voluntarily change muscle activity phase in the vastus medialis. Surprisingly, we did not observe a significant difference in ability to alter phasing between paretic and nonparetic legs or between bilateral and unilateral pedaling. In this visual feedback task, participants appear to modify muscle activity well in both the paretic and nonparetic legs.

Peak oxygen uptake in Paralympic sitting sports: A systematic literature review, meta- and pooled-data analysis.

BACKGROUND: Peak oxygen uptake (VO2peak) in Paralympic sitting sports athletes represents their maximal ability to deliver energy aerobically in an upper-body mode, with values being influenced by sex, disability-related physiological limitations, sport-specific demands, training status and how they are tested. OBJECTIVES: To identify VO2peak values in Paralympic sitting sports, examine between-sports differences and within-sports variations in VO2peak and determine the influence of sex, age, body-mass, disability and test-mode on VO2peak. DESIGN: Systematic literature review and meta-analysis. DATA SOURCES: PubMed, CINAHL, SPORTDiscusTM and EMBASE were systematically searched in October 2016 using relevant medical subject headings, keywords and a Boolean. ELIGIBILITY CRITERIA: Studies that assessed VO2peak values in sitting sports athletes with a disability in a laboratory setting were included. DATA SYNTHESIS: Data was extracted and pooled in the different sports disciplines, weighted by the Dersimonian and Laird random effects approach. Quality of the included studies was assessed with a modified version of the Downs and Black checklist by two independent reviewers. Meta-regression and pooled-data multiple regression analyses were performed to assess the influence of sex, age, body-mass, disability, test mode and study quality on VO2peak. RESULTS: Of 6542 retrieved articles, 57 studies reporting VO2peak values in 14 different sitting sports were included in this review. VO2peak values from 771 athletes were used in the data analysis, of which 30% participated in wheelchair basketball, 27% in wheelchair racing, 15% in wheelchair rugby and the remaining 28% in the 11 other disciplines. Fifty-six percent of the athletes had a spinal cord injury and 87% were men. Sports-discipline-averaged VO2peak values ranged from 2.9 L∙min-1 and 45.6 mL∙kg-1∙min-1 in Nordic sit skiing to 1.4 L∙min-1 and 17.3 mL∙kg-1∙min-1 in shooting and 1.3 L∙min-1 and 18.9 mL∙kg-1∙min-1 in wheelchair rugby. Large within-sports variation was found in sports with few included studies and corresponding low sample sizes. The meta-regression and pooled-data multiple regression analyses showed that being a man, having an amputation, not being tetraplegic, testing in a wheelchair ergometer and treadmill mode, were found to be favorable for high absolute and body-mass normalized VO2peak values. Furthermore, high body mass was favourable for high absolute VO2peak values and low body mass for high body-mass normalized VO2peak values. CONCLUSION: The highest VO2peak values were found in Nordic sit skiing, an endurance sport with continuously high physical efforts, and the lowest values in shooting, a sport with low levels of displacement, and in wheelchair rugby where mainly athletes with tetraplegia compete. However, VO2peak values need to be interpreted carefully in sports-disciplines with few included studies and large within-sports variation. Future studies should include detailed information on training status, sex, age, test mode, as well as the type and extent of disability in order to more precisely evaluate the effect of these factors on VO2peak.

Spinal cord abscess secondary to infected dorsal dermal sinus in an infant: uncommon presentation of a known entity.

Infection along the congenital dermal sinus tract is well known. However, congenital dorsal dermal sinus presenting with intramedullary abscess is quite rare. The sinus tract usually presents in the midline and acts as a portal of entry for infection that may manifest as meningitis, extradural or subdural abscess and may further involve the cord. Surgical drainage of pus and complete excision of the sinus tract is the standard treatment. Here we describe an infant with an infected congenital dorsal dermal sinus with atypical presentation as large paracentral abscess in the upper back. We further highlight the importance of recognising and treating these skin dimples even when clinically silent to avoid catastrophic complications.

Improved strength on 5-hydroxytryptophan and carbidopa in spinal cord atrophy.

There is ample evidence of an important role of descending serotonergic projections in modulating spinal motor neuron activation and firing, and experimental studies suggest that 5-HT receptor stimulation can improve motor function after spinal cord injury; however, relevant clinical data is sorely lacking. We describe two sisters with hemiplegic migraine, low CSF and platelet serotonin levels, and progressive spastic paraparesis associated with profound spinal cord atrophy whose lower extremity strength and ambulation responded to a precursor replacement strategy (5-hydroxytryptophan and carbidopa administration), an approach that may have broader applicability in myelopathies of diverse etiology where descending serotonergic projections are compromised.

The effect of intercostal artery reimplantation on spinal cord injury in thoracoabdominal aortic aneurysm surgery.

OBJECTIVE: Intercostal artery (ICA) reimplantation (ICAR) is thought to decrease spinal cord injury (SCI) in thoracic aortic aneurysm and thoracoabdominal aortic aneurysm (TAAA) surgery. Patients treated from 1989 to 2005 without ICAR were compared with those treated from 2005 to 2013 with ICAR to determine whether ICAR reduced SCI. We hypothesized that ICAR would reduce SCI, especially in the highest-risk patients. METHODS: This was a retrospective analysis using a prospectively maintained Investigational Review Board-approved database from a university tertiary referral center. The analysis included all patients (n = 805) undergoing thoracic aortic aneurysm and TAAA surgery from 1989 to 2013. The main outcome measure was any transient or permanent paraplegia or paraparesis (SCI). From 1989 to 2004, ICAR was not performed in patients, and open ICAs were ligated; from 2005 to 2013, open ICAs at T7 to L2 were reimplanted in patients with Crawford type I, II, and III TAAAs. Surgical technique was cross clamp without assisted circulation. Anesthetic management was the same from 1989 to 2013. Demographic, intraoperative, and outcome variables were assessed by univariate and multivariate analysis. Observed/expected ratios for paralysis were calculated. RESULTS: A total of 540 patients had surgery before 2005, and 265 had surgery after 2005, when ICAR was begun. There were 275 type I, II, and III TAAAs before 2005 and 164 after 2005. Aneurysm extent, acuity, SCI, mortality, renal failure, and pulmonary failure were the same in patients treated before and after 2005. Multivariate modeling of all patients showed type II TAAA (P = .0001), dissection (P = .00015), and age as a continuous variable (P = .0085) were significant for SCI. Comparing only type I, II, and III TAAAs, there was no difference in SCI between those with ICAR after 2005 and those without ICAR before 2005 (5.1% vs 8.8%; P = .152). In a subanalysis of the highest-risk patients (type II, dissection, acute), ICAR was not significant (P = .27). Observed/expected ratios ratios were 0.23 before 2005 and 0.16 after 2005 (χ2 = .796; P = .37). CONCLUSIONS: Although there was a small decrease in SCI with ICAR, reattaching ICAs did not produce a statistically significant reduction in SCI, even in the highest-risk patients.

IgG4-related disease presenting with an epidural inflammatory pseudotumor: a case report.

BACKGROUND: Inflammatory pseudotumor is a rare clinical condition that can be related to immunoglobulin G4 disease. Only a few cases of spinal inflammatory pseudotumors have been reported in the literature and an association with immunoglobulin G4 disease was not conclusive in any of them. We describe what we believe to be the first biopsy-proven case of an epidural inflammatory pseudotumor related to immunoglobulin G4 disease. CASE PRESENTATION: A 57-year-old Caucasian woman presented to our hospital with severe paraparesis, gait disturbance, and sensory loss secondary to a relapsing epidural mass. Examination of a biopsy specimen revealed a lymphoplasmacytic infiltration with fibrosis and an immunoglobulin G4-positive plasma cell ratio of over 50 %, which are compatible with a diagnosis of immunoglobulin G4-related inflammatory pseudotumor. Our patient was successfully treated with systemic and epidural administration of glucocorticoids. CONCLUSION: Immunoglobulin G4-related disease is an emerging clinical condition in which central nervous system involvement is still uncommon. We describe the case of a patient with an epidural mass with medullar compression, which was proved to be an immunoglobulin G4-related epidural inflammatory pseudotumor. Our findings suggest a new manifestation of immunoglobulin G4-related disease. This disorder should be considered in the differential diagnosis of spinal tumors as a potentially treatable condition with glucocorticoids.