Adjunctive hyperbaric oxygen therapy for spinal cord ischemia after complex aortic repair.

OBJECTIVE: Spinal cord ischemia (SCI) with paraplegia or paraparesis is a devastating complication of complex aortic repair (CAR). Treatment includes cerebrospinal fluid drainage, maintenance of hemoglobin concentration (>10 g/L), and elevating mean arterial blood pressure. Animal and human case series have reported improvements in SCI outcomes with hyperbaric oxygen therapy (HBOT). We reviewed our center's experience with HBOT as a rescue treatment for spinal cord ischemia post-CAR in addition to standard treatment. METHODS: A retrospective review of the University Health Network's Hyperbaric Medicine Unit treatment database identified HBOT sessions for patients with SCI post-CAR between January 2013 and June 2021. Mean estimates of overall motor function scores were determined for postoperative, pre-HBOT, post-HBOT (within 4 hours of the final HBOT session), and at the final assessment (last available in-hospital evaluation) using a linear mixed model. A subgroup analysis compared the mean estimates of overall motor function scores between improvement and non-improvement groups at given timepoints. Improvement of motor function was defined as either a ≥2 point increase in overall muscle function score in patients with paraparesis or an upward change in motor deficit categorization (para/monoplegia, paraparesis, and no deficit). Subgroup analysis was performed by stratifying by improvement or non-improvement of motor function from pre-HBOT to final evaluation. RESULTS: Thirty patients were treated for SCI. Pre-HBOT, the motor deficit categorization was 10 paraplegia, three monoplegia, 16 paraparesis, and one unable to assess. At the final assessment, 14 patients demonstrated variable degrees of motor function improvement; eight patients demonstrated full motor function recovery. Seven of the 10 patients with paraplegia remained paraplegic despite HBOT. The estimated mean of overall muscle function score for pre-HBOT was 16.6 ± 2.9 (95% confidence interval [CI], 10.9-22.3) and for final assessment was 23.4 ± 2.9 (95% CI, 17.7-29.1). The estimated mean difference between pre-HBOT and final assessment overall muscle function score was 6.7 ± 3.1 (95% CI, 0.6-16.1). The estimated mean difference of the overall muscle function score between pre-HBOT and final assessment for the improved group was 16.6 ± 3.5 (95% CI, 7.5-25.7) vs -4.9 ± 4.2 (95% CI, -16.0 to 6.2) for the non-improved group. CONCLUSIONS: HBOT, in addition to standard treatment, may potentially improve recovery in spinal cord function following SCI post-CAR. However, the potential benefits of HBOT are not equally distributed among subgroups.

Descending thoracic aortic mural ulceration is associated with postoperative spinal cord ischemia after branched endovascular aortic aneurysm repair.

OBJECTIVE: Paraplegia is one of the most feared complications after thoracoabdominal aortic aneurysm repair. The purpose of this study is to determine whether aortic thrombus characteristics are associated with spinal cord ischemia (SCI) after branched endovascular aneurysm repair (BEVAR). METHODS: From April 2011 to April 2020, 62 patients underwent elective BEVAR for thoracoabdominal aortic aneurysm and pararenal aortic aneurysms using a low-profile device and had a complete preoperative computed tomography angiography of the aorta from the sinotubular junction to the aortic bifurcation. Aortic thrombus was evaluated for thrombus thickness ≥5 mm, thrombus >2/3 of aortic circumference, and the presence of an ulcer-like thrombus. One point was assigned at each 5 mm axial image if all 3 criteria were met, resulting in a total "shaggy score" for the entire aorta. Data on demographics, procedural details, and outcomes were collected prospectively. All patients underwent a standard spinal cord protection protocol, including routine cerebrospinal fluid drainage. In July 2016, an insulin infusion protocol (IIP) was initiated to maintain postoperative blood glucose levels <120 mg/dL for 48 hours. The primary clinical end point was postoperative SCI. RESULTS: 10 (16%) patients developed postoperative SCI: 6 with transient paraparesis, 2 with persistent paraparesis, and 2 with persistent paraplegia. Patients with SCI were older, had higher shaggy scores, and were less likely to have been on an IIP. There were no significant differences in demographics, aneurysm type, or operative parameters. In a logistic multivariate regression model for SCI, age (odds ratio [OR]: 1.2 [1.1-1.4], P = .02) and shaggy score (OR: 1.2 [1.1-1.4], P = .02) were independently associated with increased risk of SCI, whereas treatment with the IIP was associated with lower risk of SCI (OR: 0.04 [0.006-0.50], P = .05). Of the individual components of the shaggy score, higher descending thoracic aortic ulcer scores were the most strongly associated with postoperative SCI (P = .009). CONCLUSIONS: Preoperative characterization of aortic wall thrombus is an important adjunctive tool for individualized clinical decision-making and patient counseling about the risk of SCI after BEVAR.

Open Versus Zone 0/1 Endovascular Aortic Repair for Arch Aneurysm: A Propensity Score-Matched Study from the National Clinical Database in Japan.

BACKGROUND: Although open surgical repair (OSR) is the gold standard for treating arch aneurysms, thoracic endovascular aortic repair (TEVAR) may be a less invasive alternative. However, it remains unclear which of the 2 methods yields better outcomes. In this study, we compared the perioperative outcomes of both procedures for arch aneurysms using a nationwide surgical database. METHODS: Data of patients who underwent elective aortic repair for true arch aneurysms were extracted from the National Clinical Database of Japan. Patients who underwent OSR and Zone 0/1 TEVAR were matched in a 1:1 ratio using propensity scores and their mortality and morbidity rates were compared. RESULTS: A total of 2,815 and 1,125 patients underwent OSR and Zone 0/1 TEVAR, respectively. After propensity score matching, 1,058 patients were included in both groups. Compared with OSR, Zone 0/1 TEVAR was associated with a significantly higher incidence of stroke (5.8 vs. 10.0%, P < 0.001) and paraplegia/paraparesis (1.6 vs. 4.4%, P < 0.001). However, there were no significant differences in the 30-day and operative mortality rates between the 2 groups (2.2 vs. 2.7% and 4.5 vs. 5.4%, respectively). In the Zone 0/1 TEVAR group, postoperative computed tomography was performed in 92.4% of patients, and types I and III endoleaks were identified in 6.4% and 1.1% of patients, respectively. CONCLUSIONS: Zone 0/1 TEVAR has higher incidences of stroke and paraplegia/paraparesis than OSR, with a risk of postoperative endoleaks. Resolving these problems is the key for expanding the application of Zone 0/1 TEVAR and in the meantime OSR remains the gold standard for surgically fit patients.

Syringomyelia secondary to thoracic spinal dural arteriovenous fistula presenting as paraparesis.

Spinal dural arteriovenous fistula (SDAVF) is also known as a type 1 spinal arteriovenous malformation, representing the most frequent vascular malformation of the spine. A high suspicion index is often required for the initial diagnosis of SDAVF because of subtle magnetic resonance imaging signs. We present the case of a patient with SDAVF associated with syringomyelia of the thoracic spinal cord and hypothesize that a fistula might induce intramedullary fluid accumulation due to venous hypertension, which leads to syrinx formation.

Natural history and surgical outcomes of idiopathic spinal cord herniation.

STUDY DESIGN: Retrospective multicenter study. OBJECTIVES: Although surgery is frequently selected for the treatment of idiopathic spinal cord herniation (ISCH), its impact on functional outcomes has yet to be fully understood given the limited number of patients in previous studies. This study aims to evaluate the symptomatic history and surgical outcomes of ISCH. SETTING: Three institutions in Japan. METHODS: A total of 34 subjects with ISCH were retrospectively enrolled and followed up for at least 2 years. Demographic information, imaging findings, and clinical outcomes were collected. Functional status was assessed using the JOA score. RESULTS: The types of neurologic deficit were monoparesis, Brown-Sequard, and paraparesis in 5, 17, and 12 cases, with their mean disease duration being 1.2, 4.2, and 5.8 years, respectively. Significant differences in disease duration were observed between the monoparesis and Brown-Sequard groups (p < 0.01) and between the monoparesis and paraparesis groups (p = 0.04). Surgery promoted significantly better recovery rates from baseline. Correlations were observed between age at surgery and recovery rate (p < 0.01) and between disease duration and recovery rate (p = 0.04). The mean recovery rates were 82.6%, 51.6%, and 29.1% in the monoparesis, Brown-Sequard, and paraparesis groups, respectively. The monoparesis group had a significantly higher recovery rate than did the Brown-Sequard (p = 0.045) and paraparesis groups (p < 0.01). CONCLUSIONS: Longer disease duration was correlated with the progression of neurologic deficit. Older age, and worse preoperative neurologic status hindered postoperative functional recovery. These results highlight the need to consider surgical timing before neurologic symptoms deteriorate.

An extradural cyst in a French Bulldog.

CASE HISTORY: A 7-year-old, male neutered French Bulldog was referred to a specialist veterinary hospital for evaluation of progressive paraparesis of 6-months' duration. The owners reported both faecal and urinary incontinence at home. CLINICAL FINDINGS: The dog presented with ambulatory paraparesis and pelvic limb ataxia that was more pronounced in the right pelvic limb. The pelvic limb withdrawal response and sciatic myotatic response were reduced bilaterally. Postural reaction responses were delayed in both pelvic limbs, and this was more obvious in the right pelvic limb. The anal tone and perineal sensation were normal at the time of examination.An L4-S3 myelopathy was suspected. CT of the spine revealed a compressive, bilobed, extramedullary, cyst-like structure within the vertebral canal, between L7 and S3. Surgical removal of the cyst via a L7-S1 dorsal laminectomy was performed. Histopathological examination and additional immunohistochemistry of the excised structure indicated a probable ependymal cyst with a ciliated lining. The dog recovered well post-operatively, and at follow-up 3 weeks later had some improvement of his neurological signs. The paraparesis and pelvic limb ataxia had improved; however, the remaining neurological examination was similar to the pre-surgical examination. DIAGNOSIS: Extradural cyst. CLINICAL RELEVANCE: Spinal cysts can contribute to clinical signs that resemble other common chronic spinal cord diseases, such as intervertebral disc disease. Therefore, this disease should be considered as a differential when dealing with cases of progressive paraparesis and pelvic limb ataxia. This case report may potentially provide opportunities in the future for further understanding of the pathogenesis, behaviour, outcomes and subclassification of spinal cysts in dogs.

Multifocal spinal inflammatory myofibroblastic tumors in a juvenile paraparetic dog.

A 1-year-old, female English Bulldog presented with a 10-day history of progressive paraparesis. Neuroanatomical localization was consistent with T3-L3 segment myelopathy. Magnetic resonance imaging (MRI) revealed a severely compressive, mildly contrast enhancing, extradural, dorsal, broad-based mass at the level of L3-4. Similar, non-compressive, smaller nodules were present along the extradural space and dura mater of the caudal lumbar spine. Owners elected euthanasia based on these imaging findings and progressive clinical signs. Necropsy, histopathology and immunohistochemistry revealed a mesenchymal mass and nodules, admixed with numerous inflammatory cells. The diagnosis of an extradural inflammatory myofibroblastic tumor (IMT) with a multifocal presentation was made.

[Efficacy of Distal Perfusion During Frozen Elephant Trunk Procedure:Continuous Perfusion Versus Intermittent Perfusion].

PURPOSE: To compare the effectiveness of continuous distal perfusion( CDP) with that of intermittent distal perfusion( IDP) during frozen elephant trunk( FET) procedures. METHODS: There were 54 patients in the CDP group and 15 patients in the IDP group. There were no significant differences in preoperative characteristics between the two groups, but dissection was more common in the IDP group than in the CDP group, and the maximum aneurysmal diameter was larger in the CDP group than in the IDP group. RESULTS: Emergency surgery was significantly more common in the IDP group than in the CDP group. Operating time, cardiopulmonary bypass time, lower body arrest time, and aortic cross-clamp time were significantly longer in the IDP group. Postoperative paraparesis occurred in one case in each group, and temporary paraparesis occurred in two cases in the CDP group, with no significant differences, including in mechanical ventilation time. There were two in-hospital deaths, one due to respiratory failure and one due to ischemic colitis, in the CDP group and one due to multiple organ failure in the IDP group. Postoperative liver and renal functions did not differ significantly between the two groups. CONCLUSION: IDP has proven to be almost as effective as CDP during FET for preventing spinal cord ischemia and maintaining respiratory, liver, and renal functions.

PROGRESSIVE PARAPARESIS IN A 66-YEAR-OLD MAN - A CASE STUDY.

The paper presents a case of progressive paraparesis in a 66-year-old man with no history of tick bite, who was finally diagnosed with neuroborreliosis on the basis of the performed tests. Proper diagnosis and introduction of causal treatment resulted in rapid improvement of the patient's condition.

Disseminated blastomycosis in a 1-year-old, male golden retriever-poodle crossbred dog.

A 1-year-old male golden retriever-poodle crossbred dog was brought to a veterinary clinic with a 1-month travel history to Muskoka, Ontario and a 1-week history of left eye irritation and ocular discharge. Ophthalmic examination and blood analysis revealed bilateral uveitis with a normal complete blood (cell) count and biochemistry panel. Symptomatic treatment was administered with no improvement observed and the dog returned 2 weeks later for assessment of a draining swelling of the left hind 4th digit. Clinical examination of thoracic radiographs and abdominal ultrasound showed evidence of disseminated blastomycosis characterized by pulmonary lesions, and multifocal lymphadenopathy. Biopsy of the draining lesion and cytological examination of an enlarged lymph node established diagnosis. The dog was started on a standard antifungal treatment protocol (itraconazole) but returned after 11 d of treatment with neurological signs including ataxia, paraparesis, left head tilt, and compulsive turning. Humane euthanasia was chosen and the diagnosis was confirmed at postmortem examination.

Blastomycose disséminée chez un chien croisé golden retriever-caniche mâle de 1 an. Un chien croisé golden retriever-caniche mâle âgé d'un an a été amené à une clinique vétérinaire avec un historique de voyage d'un mois à Muskoka, en Ontario et une histoire d'irritation de l'oeil gauche et d'écoulement oculaire d'une semaine. L'examen ophtalmologique et l'analyse sanguine ont révélé une uvéite bilatérale avec une numération globulaire complète et un bilan biochimique normaux. Un traitement symptomatique a été administré sans amélioration observée et le chien est revenu 2 semaines plus tard pour l'évaluation d'une enflure drainante du 4e doigt postérieur gauche. L'examen clinique des radiographies thoraciques et de l'échographie abdominale a montré des signes de blastomycose disséminée caractérisée par des lésions pulmonaires et une lymphadénopathie multifocale. Une biopsie de la lésion drainante et un examen cytologique d'un noeud lymphatique augmenté de volume ont permis d'établir le diagnostic. Le chien a débuté un protocole de traitement antifongique standard (itraconazole) mais est revenu après 11 jours de traitement avec des signes neurologiques, notamment ataxie, paraparésie, inclinaison de la tête à gauche et tournis compulsif. L'euthanasie sans cruauté a été choisie et le diagnostic a été confirmé lors de l'autopsie.(Traduit par Dr Serge Messier).

[RAPIDLY PROGRESSING PARAPARESIS AND LOSS OF SENSATION BELOW T10 DURING NEOADJUVANT CHEMOTHERAPY FOR BREAST CANCER].

INTRODUCTION: A 35 years old woman was diagnosed with clinical stage 2, grade 3, hormone receptor positive, human epidermal growth factor receptor 2 (HER2) negative invasive ductal carcinoma, with ki-67 of 60%. She was treated with neoadjuvant chemotherapy with dose dense adriamycin and cyclophosphamide followed by paclitaxel. Six days following the third cycle of paclitaxel the patient presented with rapidly progressive weakness, proximal paresthesia and decreased sensation in both legs. Physical examination revealed hypoesthesia below level, proximal and distal weakness in both lower limbs and absence of reflexes. MRI of the spine demonstrated diffuse leptomeningeal enhancement from T11 to S1 including the cauda equina roots. The rapidly progressive neurological symptoms and the MRI findings were initially interpreted as leptomeningeal spread. High dose dexamethasone was promptly initiated and the patient was urgently planned for radiotherapy and received the first fraction of 3 Gy to level T11-S1. Further workup included lumbar puncture which showed elevated protein level (350 mg/dL), negative cytology for malignancy and EMG which demonstrated demyelinating injury compatible with Guillain-Barre syndrome (GBS). A diagnosis of GBS was made and treatment with intravenous immunoglobulins (IVIG) was initiated, followed by a gradual clinical improvement. Two months after the initial diagnosis, she had a near complete resolution of her neurological deficits. This case illustrates both the tendency to ascribe new symptoms and clinical findings in cancer patients to progressive disease, and the importance of keeping a wide differential diagnosis for non-cancer etiologies when treating our patients.

Effect of thoracoabdominal aortic aneurysm extent on outcomes in patients undergoing fenestrated/branched endovascular aneurysm repair.

OBJECTIVE: The outcomes after open repair of thoracoabdominal aneurysms (TAAAs) have been definitively demonstrated to worsen as the TAAA extent increases. However, the effect of TAAA extent on fenestrated/branched endovascular aneurysm repair (F/BEVAR) outcomes is unclear. We investigated the differences in outcomes of F/BEVAR according to the TAAA extent. METHODS: We reviewed a single-institution, prospectively maintained database of all F/BEVAR procedures performed in an institutional review board-approved registry and/or physician-sponsored Food and Drug Administration investigational device exemption trial (trial no. G130210). The patients were stratified into two groups: group 1, extensive (extent 1-3) TAAAs; and group 2, nonextensive (juxtarenal, pararenal, and extent 4-5) TAAAs. The perioperative outcomes were compared using the χ2 test. Kaplan-Meier analysis of 3-year survival, target artery patency, reintervention, type I or III endoleak, and branch instability (type Ic or III endoleak, loss of branch patency, target vessel stenosis >50%) was performed. Cox proportional hazards modeling was used to assess the independent effect of extensive TAAA on 1-year mortality. RESULTS: During the study period, 299 F/BEVAR procedures were performed for 87 extensive TAAAs (29%) and 212 nonextensive TAAAs (71%). Most repairs had used company-manufactured, custom-made devices (n = 241; 81%). Between the two groups, no perioperative differences were observed in myocardial infarction, stroke, acute kidney injury, dialysis, target artery occlusion, access site complication, or type I or III endoleak (P > .05 for all). The incidence of perioperative paraparesis was greater in the extensive TAAA group (8.1% vs 0.5%; P = .001). However, the incidence of long-term paralysis was equivalent (2.3% vs 0.5%; P = .20), with nearly all patients with paraparesis regaining ambulatory function. On Kaplan-Meier analysis, no differences in survival, target artery patency, or freedom from reintervention were observed at 3 years (P > .05 for all). Freedom from type I or III endoleak (P < .01) and freedom from branch instability (P < .01) were significantly worse in the extensive TAAA group. Cox proportional hazards modeling demonstrated that F/BEVAR for extensive TAAA was not associated with 1-year mortality (hazard ratio, 1.71; 95% confidence interval, 0.91-3.52; P = .13). CONCLUSIONS: Unlike open TAAA repair, the F/BEVAR outcomes were similar for extensive and nonextensive TAAAs. The differences in perioperative paraparesis, branch instability, and type I or III endoleak likely resulted from the increasing length of aortic coverage and number of target arteries involved. These findings suggest that high-volume centers performing F/BEVAR should expect comparable outcomes for extensive and nonextensive TAAA repair.

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.

Isolated primary spinal epidural hydatid cyst in a child with progressive paraparesis.

BACKGROUND: Primary spinal hydatid cyst is a rare but serious condition which occurs in about 1% of patients with hydatid disease. This disease may result in severe spinal cord compression presenting with various neurological deficits. CASE REPORT: A 3-year-old boy was referred to our center with progressive weakness of lower limbs, frequency, and urinary incontinence. His parents did not report back pain during child's illness. Lumbar magnetic resonance imaging (MRI) revealed an extradural well-defined thin-walled cystic lesion at L4 to S3 vertebral levels without enhancement. The cyst had compressed the thecal sac associated with bone scalloping of vertebral bodies and posterior elements. Osteoplastic laminectomy of L4-L5 and laminectomy of S1-S3 was done. Intraoperative and histopathological findings indicated an epidural hydatid cyst. CONCLUSION: Although spinal hydatid cysts are rare but might be considered in the differential diagnosis of any patient with signs and symptoms of spinal cord compression. The pathophysiology, clinical manifestations, epidemiology, diagnosis, and surgical treatment of extradural hydatid cyst are discussed.

Early surgical intervention for active thoracic spinal tuberculosis patients with paraparesis and paraplegia.

BACKGROUND: To explore the therapeutic effect of early surgical intervention for active thoracic spinal tuberculosis (TB) patients with paraparesis and paraplegia. METHODS: Data on 118 active thoracic spinal TB patients with paraparesis and paraplegia who had undergone surgery at an early stage (within three weeks of paraparesis and paraplegia) from January 2008 to December 2014 were retrospectively analyzed. The operation duration, blood loss, perioperative complication rate, VAS score, ASIA grade and NASCIS score of neurological status rating, Erythrocyte Sedimentation Rate (ESR), C-reactive protein (CRP), kyphotic Cobb's angle, and duration of bone graft fusion were analyzed to evaluate the therapeutic effects of surgery. RESULTS: The mean operating time was 194.2 minutes, and the mean blood loss was 871.2 ml. The perioperative complication rate was 5.9 %. The mean preoperative VAS score was 5.3, which significantly decreased to 3.2 after the operation and continued decreasing to 1.1 at follow up (P<0.05). All cases achieved an increase of at least one ASIA grade after operation. The rate of full neurological recovery for paraplegia (ASIA grade A and B) was 18.0 % and was significantly lower than the rate (100 %) for paraparesis (ASIA grade C and D) (P<0.05). On the NASCIS scale, the difference in the neurological improvement rate between paraplegia (22.2 % ± 14.1 % in sensation and 52.2 % ± 25.8 % in movement) and paraparesis (26.7 % ± 7.5 % in sensation and 59.4 % ± 7.3 % in movement) was remarkable (P<0.05). Mean preoperative ESR and CRP were 73.1 mm /h and 82.4 mg/L, respectively, which showed a significant increase after operation (P>0.05), then gradually decreased to 11.5 ± 1.8 mm/h and 2.6 ± 0.82 mg/L, respectively, at final follow up (P<0.05). The mean preoperative kyphotic Cobb's angle was 21.9º, which significantly decreased to 6.5º after operation (P<0.05) while kyphotic correction was not lost during follow up (P>0.05). The mean duration of bone graft fusion was 8.6 ± 1.3 months. CONCLUSIONS: Early surgical intervention may be beneficial for active thoracic spinal TB patients with paraparesis and paraplegia, with surgical intervention being more beneficial for recovery from paraparesis than paraplegia.

Acute paraparesis in HIV-infected patient after initiation of highly active antiretroviral therapy.

Neurological syndromes occur in around 40-70% of HIV-infected people. Direct central nervous system involvement by the virus usually manifests as HIV encephalitis, HIV leucoencephalopathy, vacuolar leucoencephalopathy or vacuolar myelopathy. Indirect involvement is usually associated with neurotropic opportunistic infections which include tuberculosis, toxoplasmosis, cryptococcosis and viral encephalitis such as herpes simplex, varicella-zoster, cytomegalovirus and Human polyomavirus 2. We report a case of transverse myelitis in a recently diagnosed HIV patient who was otherwise asymptomatic initially and developed paraparesis after 1 month of initiation of antiretroviral therapy. After ruling out opportunistic infections and other causes of compressive and non-compressive myelopathy, development of transverse myelitis was attributed to immune reconstitution inflammatory syndrome in view of baseline low CD4 count and their improvement after HAART initiation. Prompt treatment with corticosteroids successfully reversed the symptoms.

Occult tethered cord syndrome: a reversible cause of paraparesis not to be missed.

A 15-year-old female former gymnast with a history of pectus excavatum was reviewed due to unexplained paraparesis and urinary incontinence since age 10. Symptoms were commenced with intolerable upper back pain and development of a soft mass at the sacrum that remitted spontaneously. Brain and whole spine MRI imaging and blood and CSF testing were normal. The combination of skeletal, neurological, and bladder symptoms with normal lumbar MRI and abnormal urodynamic and neurophysiological studies led to the clinical suspicion of occult tethered cord syndrome (oTCS). Surgical cord "untethering" was performed leading to remarkable postoperative clinical improvement. oTCS is a recently defined functional disorder of the spinal cord due to fixation (tethering) of the conus medullaris by inelastic elements that may lead to severe neurological impairment. High clinical suspicion is required as oTCS is a treatable spinal cord disorder.

A comparison of the energy costs with use of different types of manual wheelchair in disabled persons.

SUMMARY: The energy cost of physical activity is a well-documented field of research both in non-disabled subjects and in subjects with physical disabilities, in particular spinal cord lesions. The aim of this study was, therefore, to investigate the energy cost and subjective fatigue in disabled persons who make daily use of a manual wheelchair by comparing three different types of wheelchair (standard, lightweight and ultra-light) in order to obtain indices useful for prescribing the most effective and appropriate wheelchairaid for the individual patient. The study was carried out on 18 patients affected by paraplegia or paraparesis due to spinal cord injury at different levels. Result revealed a significant difference across the three types of wheelchair, with the energy expenditure to cover 100 m increasing from the ultra-light (lowest expenditure) to the lightweight to the standard type (highest expenditure). The differences observed in the average energy consumed to cover a distance of 100 meters with the three types of wheelchair confirm the hypothesis that it is the weight of the wheelchair chosen by the rehabilitation team together with the patient that constitutes the fundamental criterion in making such a prescription. Obviously, in making the final choice, other factors as well need to be taken into account, such as the person's age and anthropometric characteristics, the nature of the disability and prognosis, the achievable degree of autonomy, functional capacities, personal preferences, the type of use (domestic or external), accessibility, reliability and durability, esthetic features, eventual accessories available, etc.

Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge.

Background and aims - Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports - Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion - Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.

Nonfocal Transient Neurological Attacks Are Associated With Cerebral Small Vessel Disease.

Background and Purpose- Nonfocal transient neurological attacks (TNAs), such as unsteadiness, bilateral weakness, or confusion, are associated with an increased risk of stroke and dementia. Cerebral ischemia plays a role in their pathogenesis, but the precise mechanisms are unknown. We hypothesized that cerebral small vessel disease is involved in the pathogenesis of TNAs and assessed the relation between TNAs and manifestations of cerebral small vessel disease on magnetic resonance imaging. Methods- We included participants from the HBC (Heart-Brain Connection) study. In this study, hemodynamic and cardiovascular contributions to cognitive impairment have been studied in patients with heart failure, carotid artery occlusion, or possible vascular cognitive impairment, as well as in a reference group. We excluded participants with a history of stroke or transient ischemic attacks. The occurrence of the following 8 TNAs was assessed with a standardized interview: unconsciousness, confusion, amnesia, unsteadiness, bilateral leg weakness, blurred vision, nonrotatory dizziness, and paresthesias. The occurrence of TNAs was related to the presence of lacunes or white matter hyperintensities (Fazekas score, ≥2; early confluent or confluent lesions) in logistic regression analysis, adjusted for age, sex, and hypertension. Results- Of 304 participants (60% men; mean age, 67±9 years), 63 participants (21%) experienced ≥1 TNAs. Lacunes and early confluent or confluent white matter hyperintensities were more common in participants with TNAs than in participants without TNAs (35% versus 20%; adjusted odds ratio, 2.32 [95% CI, 1.22-4.40] and 48% versus 27%; adjusted odds ratio, 2.65 [95% CI, 1.44-4.90], respectively). Conclusions- In our study, TNAs are associated with the presence of lacunes and early confluent or confluent white matter hyperintensities of presumed vascular origin, which indicates that cerebral small vessel disease might play a role in the pathogenesis of TNAs.