Pregnancy following Brown-Séquard syndrome: A rare case report

Brown-Séquard syndrome is an incomplete spinal cord lesion characterized by hemisection injury of the cord. We present a case of pregnancy, delivery and postpartum course following this rare neurological condition. A 42-year-old woman presented with past history of idiopathic hemicord myelitis leading to right sided hemiplegia with decreased contralateral sensation of pain and temperature, consistent with Brown-Séquard syndrome, which was treated with steroids and Therapeutic Plasma Exchange. Thereafter, she had near-complete motor recovery and complete sensory recovery over the next 3months. Three years later, she presented to us at 37+2 weeks of gestation with residual hemiparesis with motor power grade of 4/5 in right upper and lower limbs. She underwent Caesarean section for breech presentation, which was done under general anaesthesia in view of prior spinal cord lesion. She was discharged for follow-up in Neurology outpatient clinic and physical rehabilitation. At follow up after 12 months of delivery, she had complete motor and sensory recovery. Management of spinal cord lesions in pregnancy and delivery requires specialist multidisciplinary care due to risk of medical and obstetric complications. This case demonstrates a rare scenario of a primigravida at term gestation with residual deficits of a past spinal cord lesion.

Weakness With Neck Flexion

A 51-year-old woman sought care for difficulty “picking up” her right foot after walking for 30 minutes; with rest, the symptoms would subside. A few months later, she reported a “zinging” sensation in her right 4th and 5th fingers and down her right side with neck flexion. Brain magnetic resonance imaging showed tiny nonspecific lesions not suggestive of demyelinating disease and a single T2 hyperintensity at the cervicomedullary junction that did not enhance with gadolinium administration. Cerebrospinal fluid analysis showed 10 oligoclonal bands on isoelectric focusing electrophoresis and a mildly increased immunoglobulin G index of 0.73. The patient was diagnosed with solitary sclerosis, suspected to be a form of central nervous system demyelinating disease strongly related to multiple sclerosis. Treatment was based on a diagnosis of “primary progressive multiple sclerosis,” although the patient did not satisfy the criterion of dissemination in space. The patient was being treated with glatiramer acetate 40 mg 3 times weekly for a presumptive diagnosis of multiple sclerosis before evaluation at Mayo Clinic. After our evaluation, she was advised that no treatments at that time were efficacious to prevent deterioration in patients with primary progressive multiple sclerosis. The mainstay of treatment is physical medicine modalities, including principles of energy conservation and, when necessary, mobility aids, such as braces and canes. The presentation of disease in this patient suggested a chronic myelopathy. Her symptoms did not develop acutely and manifested only after she walked a distance. Symptoms involving the upper and lower extremities and precipitation of Lhermitte sign with neck flexion also suggested a spinal cord lesion, typically a demyelinating lesion. However, a single spinal cord lesion present at the cervicomedullary junction. Oligoclonal bands are detected in patients with central nervous system infections or paraneoplastic disorders that might be associated with myelopathy.

The Need for a Broad Differential: Intramedullary Neurosarcoidosis Case Report

BackgroundSarcoidosis, an idiopathic multisystem inflammatory disorder, involves the nervous system in as few as 5-15% of cases. We aim to detail how a rare case of intramedullary neurosarcoidosis spinal-cord lesion, present in less than 1% of sarcoidosis cases, presented with features mimicking a neoplasm. MethodsRetrospective chart review was performed to obtain pertinent details regarding history and examination, pathological findings, and treatment course.Case PresentationWe report a case of intramedullary sarcoidosis involving the cervical and thoracic spinal cord with syringomyelia, which presented as subacute neck pain, intermittent leg paresthesias, and difficult micturition. Historically, a spinal syrinx with concern for neoplasm has led surgeons to decompress the spinal cord for certain enhancing intramedullary lesions, which is unnecessary for neurosarcoidosis. Immunosuppressant treatment resulted in symptomatic resolution without the need for spinal cord biopsy or syrinx decompression in this case.Conclusions Expansile contrast-enhancing intramedullary lesions, most commonly neoplastic, may instead be non-neoplastic etiologies mimicking neoplastic features; therefore, it is the responsibility of any surgeon to maintain a broad differential diagnosis in the absence of a confirmed pathology.

Gait Analysis Using Animal Models of Peripheral Nerve and Spinal Cord Injuries

The present review discusses recent data regarding rodent models of spinal cord and peripheral nerve injuries in terms of gait analysis using the CatWalk system (CW), an automated and exceptionally reliable system for assessing gait abnormalities and motor coordination. CW is a good tool for both studying improvements in the walking of animals after suffering a peripheral nerve and spinal cord lesion and to select the best therapies and procedures after tissue destruction, given that it provides objective and quantifiable data. Most studies using CW for gait analysis that were published in recent years focus on injuries inflicted in the peripheral nerve, spinal cord, and brain. CW has been used in the assessment of rodent motor function through high-resolution videos, whereby specialized software was used to measure several aspects of the animal’s gait, and the main characteristics of the automated system are presented here. CW was developed to assess footfall and gait changes, and it can calculate many parameters based on footprints and time. However, given the multitude of parameters, it is necessary to evaluate which are the most important under the employed experimental circumstances. By selecting appropriate animal models and evaluating peripheral nerve and spinal cord lesion regeneration using standardized methods, suggestions for new therapies can be provided, which represents the translation of this methodology into clinical application.

Indolent nonendemic central nervous system histoplasmosis presenting as an isolated intramedullary enhancing spinal cord lesion

Background: Histoplasma capsulatum infection is largely seen in endemic regions; it results in symptomatic disease in <5% of those infected and is most often a self-limiting respiratory disease. Disseminated histoplasmosis is considered rare in the immunocompetent host. Central nervous system (CNS) dissemination can result in meningitis, encephalitis, and focal lesions in the brain and spinal cord, stroke, and hydrocephalus. An intramedullary spinal cord lesion as the only manifestation of CNS histoplasmosis has been rarely described. Case Description: We present an atypical case of a 44-year-old man from a nonendemic region, on adalimumab therapy for ulcerative colitis who developed an isolated intramedullary spinal cord lesion in the setting of disseminated histoplasmosis. His course was initially indolent with vague systemic symptoms that led to consideration of several other diagnoses including sarcoidosis and lymphoma. Biopsies of several positron emission tomography positive lymph nodes revealed granulomatous inflammation, but no firm diagnosis was achieved. He was ultimately diagnosed with histoplasmosis after an acute respiratory infection in the setting of anti-tumor necrosis factor therapy. With appropriate antifungal therapy, the spinal cord lesion regressed. The previous systemic biopsies were re-reviewed, and rare fungal elements consistent with H. capsulatum were identified. A presumptive diagnosis of CNS histoplasmosis was made in the absence of direct laboratory confirmation in the setting of rapid and complete resolution on antifungal therapy. Conclusion: Disseminated histoplasmosis should be considered in granulomatous disease, even if the patient resides in a nonendemic region. Furthermore, clinicians should be mindful that CNS histoplasmosis may present in an atypical fashion.

The functional properties of synapses made by regenerated axons across spinal cord lesion sites

While the anatomical properties of regenerated axons across spinal cord lesion sites have been studied extensively, little is known of how the functional properties of regenerated synapses compare to those in unlesioned animals. This comparison has been performed here in the lamprey, a model system for spinal injury research, in which functional locomotor recovery after spinal cord lesions is associated with axonal regeneration across the lesion site. Regenerated synapses below the lesion site did not differ to synapses from unlesioned axons with respect to the amplitude and duration of single excitatory postsynaptic potentials (EPSPs). They also showed the same activity-dependent depression over spike trains. However, regenerated synapses did differ to unlesioned synapses as the estimated number of synaptic vesicles was greater and there was evidence for an increased postsynaptic quantal amplitude. For axons above the lesion site, the amplitude and duration of single synaptic inputs also did not differ significantly to unlesioned animals. However, in this case there was evidence of a reduction in release probability and inputs facilitated rather that depressed over spike trains. Synaptic inputs from single regenerated axons below the lesion site thus do not increase in amplitude to compensate for the reduced number of descending axons after functional recovery. However, the postsynaptic input is maintained at the unlesioned level using different synaptic properties. Conversely, the facilitation from the same initial amplitude above the lesion site will make the synaptic input over spike trains functionally stronger. This may help to increase propriospinal activity across the lesion site to compensate for the lesion-induced reduction in supraspinal inputs.