Bilateral Lower Extremity Weakness Research Articles

Teaching NeuroImages: Cervical synovial cyst leading to spinal cord compression

Cervical synovial cysts are an uncommon degenerative finding that can lead to spinal canal stenosis, myelopathy, and radiculopathy.1 Here we present the radiographic findings of a synovial cyst in a patient presenting with progressively worsening bilateral upper and lower extremity weakness. It is seen to cause prominent uplifting of the posterior longitudinal ligament and furthermore significant central canal stenosis (figure). CT-guided needle aspiration may provide symptomatic relief though frequently resulting in reoccurrence due to preservation of an intact synovium. Definitive treatment is surgical and may consist of laminoplasty, laminectomy, and in severe instances, spinal fusion.2

SUN-001 A NEURO-RENAL SYNDROME WITH NEUROFASCIN ANTIBODIES

We present a case of focal segmental glomerulosclerosis (FSGS) and acute-onset chronic inflammatory demyelinating polyneuropathy (CIDP) associated with rare neurofascin autoantibodies, supporting a newly defined neuro-renal syndrome. A 48-year-old African-American male presented to the hospital with lower extremity paresthesias that progressed rapidly to bilateral upper and lower extremity weakness with areflexia. He underwent an electrodiagnostic study that demonstrated sural sparing, absent F waves, conduction block and markedly reduced recruitment of normal appearing motor unit potentials, consistent with acute inflammatory demyelinating polyneuropathy (AIDP) and received 2 gm/kg of intravenous immunoglobulin. He also developed new-onset proteinuria with 10 gm on spot urine albumin:creatinine ratio (UACR). Urine microscopy revealed 10-20 RBCs/HPF with few dysmorphic RBCs and a fine granular cast. A limited kidney biopsy with 3 glomeruli without tubulointerstitial damage on light microscopy (LM) and severe podocyte foot process effacement on electron microscopy (EM) was interpreted as minimal change disease (MCD). He was started on 1mg/kg prednisone daily; however, subsequently developed respiratory failure due to neuromuscular weakness requiring mechanical ventilation and was treated with plasmapheresis for 7 sessions. The patient was later extubated and discharged to acute rehabilitation. He was readmitted 2 weeks later for a suspected AIDP relapse with respiratory failure, again prompting intubation and plasmapheresis for 5 sessions. Proteinuria worsened to 24 gm despite continued treatment with prednisone. A repeat kidney biopsy showed 1/12 glomeruli with segmental sclerosis and acute tubular damage on LM, no deposits on immunofluorescence and severe podocyte foot processes effacement on EM, resulting in a new diagnosis of primary FSGS. In light of waxing and waning symptoms and protracted course he was diagnosed with acute-onset CIDP. A detailed work up returned positive for NF antibodies, NF140 and NF155, that have been reported with IVIG-refractory acute-onset CIDP. He was treated with pulse dose IV methylprednisolone and tacrolimus resulting in marked improvement of both proteinuria of 100 mg and polyneuropathy over 6 months of follow up. FSGS and CIDP may share immunopathogenic mechanisms. NF is a protein encoded by NFASC gene and belongs to the L1 family of immunoglobulin cell adhesion molecules that are involved in neuronal growth and development. NF is a paranodal protein, critical to neuronal transmission. Interestingly, NF is also a part of podocyte cytoskeleton. The presence of NF antibodies and concurrence of severe proteinuria and polyneuropathy suggests a strong neuro-renal interface. NF antibodies are typically of the IgG4 subclass. Physicians should have a high clinical suspicion for sampling errors on kidney biopsy if the disease in question does not seem to respond to adequate treatment. Our patient was first diagnosed with MCD and AIDP; however, despite adequate treatment suffered a relapse and was later diagnosed with FSGS and a rare subtype of CIDP. The pathophysiological role NF 140 and NF 155 antibodies in FSGS and their prognostic significance needs validation in further studies.

Microsurgical Management of a Spinal Dural Arteriovenous Fistula With Shared Blood Supply to the Artery of Adamkiewicz: 3-Dimensional Operative Video.

The presented case is of a 65-yr-old gentleman referred for thoracic myelopathy. He developed bilateral, nondermatomal foot dysesthesia 14 mo prior to presentation, which progressed to numbness below the L3 level. He reported progressive gait instability, bilateral lower extremity weakness, and required a cane for ambulation. He subsequently developed urinary incontinence, while bowel function was spared. The neurological examination upon presentation revealed lower extremity strength was reduced to 4/5 in all major muscle groups bilaterally, while sensation and proprioception were reduced below the L3 level. Patellar and Achilles reflexes were not elicited and clonus was absent. Gait was unsteady and slow. The patient was not able to perform heel, toe and tandem gait. MRI revealed abnormal T2 hyper-intense signal and spinal cord expansion from T6 through the conus medullaris. Angiography revealed a dural arteriovenous fistula (dAVF) originating from the left T9 radicomedullary pedicle, which also supplied the Artery of Adamkiewicz (AoA). The patient underwent T8-10 laminectomies. Prior to disconnection of the fistula, an aneurysm clip was applied to the fistulous point and an indocyanine green video angiogram was completed to show that the fistula no longer opacified, but that the AoA remained patent, which was later confirmed with angiography. As of discharge, the patient's motor exam had improved substantially, though his sensory deficits persisted. This case demonstrates that surgical disconnection is requisite in the treatment of spinal dAVF that have a shared blood supply with the AoA, as embolization risks spinal cord infarction. The patient consented to presentation of this case in a de-identified fashion.

Lower limb purpura in setting of acute paraplegia: cutaneous clue to a catastrophic cardiac event

A middle-aged shopkeeper, with type II diabetes mellitus presented with purple-black skin discolouration on both lower limbs, extending from toes till upper thighs (figure 1A,B, C). He complained of sudden...

SUN-LB098 Mimic Presentation of Neuromuscular Symptoms in Thyrotoxic Periodic Paralysis in Hispanic Male

Introduction: Thyrotoxic periodic paralysis (TPP) is a rare condition characterized by sudden paralysis with profound hypokalemia. Clinical Case: A 34 year-old obese Hispanic male presented to ED with sudden onset of generalized weakness and numbness of bilateral upper & lower extremities for one day. The patient was not able to get up when he woke up in the morning. He described having blurry vision, more severe on the right side, which was associated with right retroocular pain. He reported over twenty pounds of weight loss within past two months, accompanied with intermittent episodes of watery diarrhea, palpitations, restlessness, mild headache, and dizziness. He denied loss of consciousness, urinary or fecal incontinence, fever, cough or recent respiratory infection. Past medical history included gastritis. He reported no recent trauma, injury or hospitalization. No smoking, alcohol abuse or illicit drug use. On physical examination, the patient was having mild respiratory distress. Right eye exophthalmos and normal cardiopulmonary examination were noted. Neck examination showed no thyroid gland enlargement. Neurological findings revealed cranial nerve and sensory examination intact, but all four limbs were flaccid and motor strength of all extremities was 1/5. Deep tendon reflex was absent. Initial blood test on admission showed Potassium 1.9 mmol/l, Phosphorus 2 mg/dl, Magnesium 1.9 mg/dl, Lactic acid 4.6 mmol/l, TSH 0.005 mIU/l, Free T3: 8.80 pg/ml, Free T4: 2.3 ng/ml, and TBG 14.1 mcg/dl. Thyroid peroxidase was positive. EKG revealed sinus tachycardia. ABG indicated primary respiratory acidosis. Lumbar puncture was normal and ruled out Guillain-Barré syndrome and, viral or bacterial meningitis. Thyroid sonogram showed heterogeneous thyroid with no nodules. CT head without contrast showed no evidence of intracranial hemorrhage. MRI brain revealed no intracranial mass. MRI lumbar & thoracic spine showed a normal finding. After the aggressive replacement of potassium chloride, the muscle weakness improved significantly, and methimazole was initiated. The patient was admitted to ICU for close monitoring and electrolyte management. Conclusion: Thyrotoxicosis periodic paralysis (TPP) is a rare condition that manifests through acute paralysis, and hypokalemia in the presence of hyperthyroidism (1). It is usually found in relatively young Asian population, but also the occurrence has increased in the Hispanic population recently. Early recognition of TPP in a patient with unexplained hypokalemia and painless generalized weakness, is imperative to demand thyroid studies to rule out this condition. Close electrolyte management is important rebound hyperkalemia and life-threatening arrhythmias. Reference: (1) Meseeha M et al. Thyrotoxic periodic paralysis: a case study and review of the literature. J Community Hosp Intern Med Perspect. 2017;7(2):103-106. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. s presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.

SAT-602 Thyrotoxic Periodic Paralysis Precipitated by Glucocorticoid Injection

Background: Thyrotoxic periodic paralysis (TPP) is a rare manifestation of hypokalemia that is driven by thyrotoxicosis. TPP is associated with any etiology of thyrotoxicosis, though Graves’ disease is the most common. Some of the provoking factors for these episodes are a high carbohydrate meal, rest after heavy exercise, fasting, stress, alcohol intake, and pulse steroid therapy. Clinical Case: A 48-year-old man presented with acute-onset lower extremity paralysis. He had been in his usual state of health until two days prior to admission when he developed a streptococcal throat infection. He was given IM injections of 80mg methylprednisolone and 1g ceftriaxone in an urgent care clinic and discharged. Soon after discharge, he developed progressively worsening bilateral lower extremity soreness and weakness resulting in a fall. He was taken to the hospital by EMS, where he was found to have bilateral lower extremity paresis. He was anxious, mildly tachycardic, and had a non-tender, 25-gram diffuse goiter. No thyroid nodules were palpated nor did the patient exhibit cervical lymphadenopathy. Lid lag, a fine tremor, and onycholysis were also detected. Basic metabolic panel revealed a serum potassium of 2.3 mmol/L (ref: 3.5-5.1 mmol/L). An ECG was normal. He was given 60 mEq of oral potassium chloride and started on propranolol 10mg TID and reported symptomatic improvement within several hours. During hospitalization, the patient remained asymptomatic and his serum potassium remained normal. Thyroid function testing revealed an undetectable TSH, a Free T4 of 4.41 ng/dL (ref: 0.61-1.18 ng/dL), and a Free T3 of 10.29 pg/mL (ref: 2.5-3.9 pg/mL). Radionucleotide uptake scan of the thyroid showed diffusely increased uptake (81%) with no nodules, consistent with Graves’ disease. Thyrotropin receptor antibody and Thyroid stimulating immunoglobulin levels were elevated. He underwent successful radioactive iodine ablation. Serial laboratory testing was done and within six weeks the patient became hypothyroid. He is now treated with levothyroxine for post-ablative hypothyroidism. A literature search reveals case reports implicating steroids in the precipitation of TPP. Corticosteroids, along with thyroid hormone, increase the activity of sodium-potassium ATPase channels in skeletal muscle. Steroids also lead to hyperglycemia and subsequent increased insulin release that causes an intracellular shift of potassium. Conclusion: Though rare, TPP is important to recognize, as failure to treat underlying hyperthyroidism can lead to repeated episodes of hypokalemia and paralysis. This case highlights TPP precipitated by pulse glucocorticoid injection, described rarely in the literature. The potential of glucocorticoids to precipitate paralysis should be recognized when considering steroid treatment for other medical conditions in hyperthyroid patients.

MON-502 It's Not Always Osteoporosis: FGF-23 Induced Osteomalacia

It’s not always osteoporosis: FGF-23 induced osteomalacia. Introduction Osteomalacia is a bone disorder that is caused by many conditions which could be inherited or acquired, associated with frequent fractures and pain. Tumor induced osteomalacia (TIO) is caused by excess FGF-23 most commonly by mesenchymal tumors, which leads to phosphaturia and inactivation of 1,25 (OH)2D3, in turn causing chronic pain and/or severe muscle weakness. The diagnoses for these patients is typically delayed by years and many times labelled as osteoporosis. Case Presentation This is a 83 yo African-American male with history of prostate cancer with prostatectomy in 2002 and osteoporosis diagnosed in August 2016 at which time he was started on alendronate, cholecalciferol, and calcium. Since 2013 he had an elevated alkaline phosphatase, and given this oncology was consulted in November 2016 due to history of prostate cancer and metastatic work up. A bone scan performed in November 2016 showed increase uptake in the ribs concerning for metastatic disease, which were not seen in CT of the chest, and PET scan was negative for malignancy. In January 2018, on follow up clinic visit, he was reporting severe bilateral lower extremity weakness and pain causing difficulty walking, progressively getting worse to the point of requiring a wheelchair for about 6-12 months, due to this he was admitted to the hospital for evaluation in February 2018. His phosphorus level was 0.7, and 1,25 (OH)2D3 was also low, suggestive of tumor induced osteomalacia. An FGF-23 level was sent, which was elevated at 456 RU/ml (44-215). On clinic follow up, full physical exam revealed a left chest mass that was 3-4 cm, ovoid, non-tender, mobile and indurated. Patient then stated it had been present for 2-3 years. He underwent excision of the mass in March 2018 which was consistent with osteoclast-like giant cell. Follow up FGF 23 level was 44 (44-215). After the surgery the Patient’s pain resolved, he regained his muscle strength and was able to ambulate without assistance. Conclusion TIO is a rare paraneoplastic syndrome which requires a good level of suspicion. Many patient are treated as osteoporosis erroneously and the actual cause of the disease goes unnoticed for many years. Should be suspected in patients with phosphaturia, low 1,25 (OH)2D3, and clinical symptoms of muscle weakness and pain. Many times finding this tumors can be challenging and detailed physical exam is important to make the diagnosis.

A case of methylenetetrahydrofolate reductase deficiency enzyme deficiency carrying a novel mutation

AbstractMethylenetetrahydrofolate reductase (MTHFR) enzyme deficiency is the most common genetic cause of elevated levels of homocysteine in the plasma (hyperhomocysteinemia). We describe a 22‐year‐old male, who presented to the clinic with history of MTHFR deficiency and homocystinuria. At baseline, the patient was nonverbal, wheelchair‐bound with seizures since young age. Physical examination showed increased spasticity and weakness in bilateral lower extremities. Elevated levels of homocysteine were seen in the serum. Genetic testing revealed a single homozygous pathogenic mutation in c.1699C>T p. R587x. not previously reported for homocystinuria. Our patient carries a unique single homozygous pathogenic mutation c.1699C>T p. R587x leading to homocystinuria that hasn't been described before. More research is required to identify the pathophysiology of this mutation and role in other diseases.

Neoadjuvant durvalumab +/- tremelimumab affects the expression of immune checkpoint (IC) molecules on myeloid derived suppressor cells (MDSC) in patients (pts) with locally advanced renal cell carcinoma (RCC).

587 Background: In a single arm, open label phase 1b clinical trial the safety of neoadjuvant durvalumab +/- tremelimumab was studied in pts with (w) locally advanced RCC. Expression of IC molecules on immunomodulatory cells in peripheral blood (PB) and tumor (T) and the association w treatment (tx) was investigated. Methods: Pts with ≥ T2bN0-1M0 RCC received either durvalumab or combination durvalumab + tremelimumab prior to surgery. Blood samples were drawn prior to neoadjuvant tx, prior to surgery, and approximately 30 days after surgery before adjuvant tx. The percentage of MDSC (CD33+/HLADR-) and subtypes in PB and T and expression of PD1, PD-L1, and V-domain Ig suppressor of T cell activation (VISTA) were measured. MDSC subtypes included polymorphonuclear (PMN; CD15+/CD14-), monocytic (M; CD15-/CD14+) and uncommitted (UC; CD15-/CD14-). Linear mixed model was used for each MDSC subtype to estimate and compare cohorts over time. Results: Eighteen pts were enrolled: 4 women and 14 men, median age 62, 17 pts had T3-4 and 4 pts had N1 disease. Six pts received 1 dose of durvalumab and 12 pts received 1 dose of durvalumab + tremelimumab before surgery. Tx-related grade 3 adverse events (per CTCAE, v5.0) included thrombocytopenia, bilateral lower extremity weakness, hyperglycemia, chest pain, and diabetic ketoacidosis.One pt had grade 4 elevated lipase. One pt had sudden death from a non-drug related cardiac event 9 days after receiving combination therapy prior to surgery. PB and T samples from 17 pts were available. Expression of VISTA on M-MDSC and UC-MDSC were positively correlated in PB and T (Spearman’s rho = 0.61; P=0.03 for both). VISTA expression on UC-MDSC in PB was significantly higher in pts who received durvalumab monotherapy compared to those treated w durvalumab + tremelimumab (P=0.04). Frequencies of PD-L1 expression on M-MDSC and UC-MDSC in PB decreased significantly from pre- to post-neoadjuvant tx (P < 0.01). Conclusions: Neoadjuvant durvalumab + tremelimumab in pts w locally advanced RCC is feasible and affects the expression of IC molecules (PD-L1 and VISTA) on M-MDSC and UC-MDSC. Clinical trial information: NCT02762006.

Variant Guillain-Barré syndrome in a patient with Hodgkin lymphoma: AMSAN

Lymphomas are solid tumors characterized by the malignant proliferation of lymphoid cells. Neurologic signs encountered in patients with Hodgkin's lymphoma can be due to the direct spread of tumor to the nervous system, secondary to chemotherapy or radiation, secondary to tumor mass compression, infectious causes and paraneoplastic syndromes. Paraneoplastic neurologic syndromes are rarely encountered in patients with Hodgkin's lymphoma and non-Hodgkin's lymphoma. Except for paraneoplastic cerebellar degeneration in Hodgkin's lymphoma and dermato/polymyositis in both Hodgkin's lymphoma and non-Hodgkin's lymphoma, other paraneoplastic syndromes are uncommon and have only been reported as isolated case reports or short series. Here, we present a patient with Hodgkin's lymphoma with symptoms of bilateral lower extremity weakness and loss of sensation before the start of therapy, who was eventually diagnosed as having motor and sensory axonal neuropathy.

Demyelinating Neuropathy in a Patient Treated With Revusiran for Transthyretin (Thr60Ala) Amyloidosis

Transthyretin amyloidosis patients develop length-dependent peripheral neuropathy, autonomic dysfunction, and restrictive cardiomyopathy associated with deposition of amyloid fibrils in these tissues. Despite advances in management over the past decade, this disorder causes profound debilitation and ultimately proves fatal. In this report, we describe a man with late-onset cardiac amyloidosis due to a transthyretin Thr60Ala mutation who was treated with an investigational RNAi therapeutic, revusiran, which targets hepatic transthyretin production. Sixteen months into treatment, he developed bilateral lower-extremity weakness and numbness, worsening balance, difficulty manipulating objects with his hands, and finger numbness. Nerve conduction studies were consistent with multifocal demyelinating neuropathy. Intravenous immunoglobulin therapy improved sensation in his hands and feet, and improved hand dexterity. A sural nerve biopsy demonstrated demyelination with substantial axonal loss in the absence of histologically detectable endoneurial amyloid deposition. This case expands the clinicopathologic spectrum of transthyretin amyloidosis and may represent complex disease and treatment effects.

Rapidly progressive neurologic decline and morbilliform rash presenting in a patient with lymphoma.

A 67-year-old male with past medical history of mantle cell lymphoma and atrial fibrillation presented with a truncal rash, bilateral lower extremity weakness, and confusion. Within three days of presentation, his condition rapidly deteriorated with the onset of diffuse flaccid paralysis, aphasia, and severe alteration in mental status. Initial results from serum studies, lumbar puncture, magnetic resonance imaging, and electroencephalogram were not diagnostic. However, on the ninth day after initial presentation, the West Nile Virus (WNV) immunoglobulin M antibody returned positive from the cerebrospinal fluid. West Nile Virus encephalitis is endemic worldwide, and is the most common viral encephalitis in the United States. WNV presents in a variety of ways, and the recognition by physicians is crucial due to the estimated 2-12% mortality rate and significant longterm morbidity of neuroinvasive disease. The initial management and long term prognosis are points of ongoing research. This case represents a particularly profound example of neuroinvasive WNV. Our patient made a significant recovery after his initial presentation with aggressive supportive care, however still suffers from bilateral lower extremity weakness more than a year later.

Development of Gray Zone Lymphoma during Remission of Classical Hodgkin Lymphoma

Although Hodgkin lymphoma (HL) is largely curable with frontline treatment, many patients will not have a complete response or will later relapse. Recurrence of HL often occurs within the first five years in remission. However, recurrence rates vary widely and are largely dependent on lymphoma type, stage, patient age, and other variables [Devita, 2015]. In this case, we discuss an individual with HL in remission for over a decade, who was found to have Gray Zone Lymphoma (GZL). GZL is a rare lymphoma with intermediate features between classical Hodgkin lymphoma (cHL) and Diffuse Large B-cell Lymphoma (DLBCL), but cannot be classified as one or the other [Evans, 2015]. We report this case to discuss the recurrence of HL that progressed and transformed into GZL.A 75-year-old male with a significant history of HL in remission, presented with sudden onset of bilateral lower extremity weakness. The patient had associated back pain, 3 weeks of fatigue, decreased appetite, and decreased urine output for the last two days. He denied any nausea, vomiting, fever, chest pain, cough, and bowel or bladder incontinence.Vital signs were significant for blood pressure of 97/56 mmHg and fever of 102.5°F. Pertinent physical examination findings included diffuse cervical, axillary, and inguinal lymphadenopathies, and hepatomegaly. Pedal edema was also noted on both lower extremities, but more pronounced on the right lower extremity. Initial pertinent labs were a leukocytosis (37,600 u/L), lactic acid of 6.4 (0-1.99 mmol/L), hyponatremia (133 u/L), and bicarbonate of 18 (22-30 mEq/L) with a normal anion gap.The patient was admitted for sepsis of unknown origin and was started on vancomycin and meropenem. Ultra sound of the right leg, CT chest, and abdominal CT angiogram with and without contrast revealed right deep venous thrombosis, pulmonary embolism in the segmental branch of the left lung, and lymphadenopathy in the chest, abdomen and numerous retroperitoneal soft tissue masses that were suspicious for enlarged lymph nodes respectively. The patient was started on therapeutic enoxaparin.The bone marrow aspirate was found to be inconclusive. Cervical lymph node biopsy showed CD30+ large cell hematolymphoid neoplasm with features intermediate between DLBCL and HL - otherwise classified as GZL. There is no standard treatment for GZL, therefore the patient was given cHL chemotherapy regimen of ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine). The patient received 1 dose of ABVD in the hospital and was discharged to follow as outpatient. Although the patient was initially admitted for sepsis, pan cultures were unremarkable, suggesting that his symptoms were attributable to malignancy.We discuss this case to describe the recurrence and progression of Hodgkin lymphoma to Grey Zone lymphoma. Very few cases of GZL have been described in literature. To date, a treatment regimen for GZL remains to be elucidated due to the sparsity of this phenomenon. Because GZL shows intermediate characteristics between cHL and DLBCL, it is currently treated as either cHL and DLBCL [Pilichowska, 2017].[Pilichowska, 2017]. DisclosuresNo relevant conflicts of interest to declare.

A mini-open transspinous approach for resection of intramedullary spinal cavernous malformations

Background and importanceAdvances in minimally invasive (MIS) and mini-open surgical approaches have led to reductions in perioperative morbidity without compromising rates of resection of non-degenerative intradural spinal pathologies. Whether these approaches may be adapted for the surgical resection for intramedullary vascular malformations – such as cavernous malformations (CMs) – has yet to be reported. The authors describe a mini-open transspinous approach to resect a ruptured intramedullary CM of the conus medullaris. Clinical PresentationA 28-year-old man presented with sudden onset of bilateral lower extremity weakness, urinary retention and saddle anesthesia. Magnetic resonance imaging demonstrated a ruptured CM within the conus medullaris with pronounced extralesional hemorrhage. A mini-open transspinous approach with an expandable tubular retractor was successfully applied to facilitate microsurgical resection of the CM and evacuation of the associated hematoma. The patient made a good neurologic recovery, and postoperative imaging confirmed a gross total resection of the CM. ConclusionA mini-open transspinous approach utilizing an expandable tubular retractor offers feasible less invasive alternative to provide dorsal midline access for the microsurgical resection of intramedullary spinal CMs. Larger case series or future randomized prospective trials are warranted to fully explore suitability of MIS techniques for the surgical management of intradural spinal cord vascular pathologies – such as CMs.

48-Year-Old Woman With Bilateral Lower Extremity Paresthesias and Weakness

48-Year-Old Woman With Bilateral Lower Extremity Paresthesias and Weakness

Man with Bilateral Lower Extremity Weakness

Man with Bilateral Lower Extremity Weakness

Vogt–Koyanagi–Harada case accompanied by polyneuropathy

AbstractVogt–Koyanagi–Harada disease (VKHD) is a systemic autoimmune disease that targets melanocyte‐rich tissues such as eye, central nervous system, inner ear, and skin. Here, we report a case of VKHD accompanied by polyneuropathy. After VKHD was diagnosed, depending on the involvement of bilateral ocular, auditory, and central nervous systems, he expressed muscle weakness, pain, and numbness in bilateral lower extremities. Nerve conduction studies suggested sensorimotor polyneuropathy, while a skin, muscle, and nerve biopsy suggested vasculitis. Steroid therapy improved his symptoms. The present case might suggest a relationship between VKHD and polyneuropathy.

Myelopathy caused by Ossification of Thoracic Ligamentum Flavum

Hypertrophy of the posterior spinal elements leading to compromise of the spinal canal and its neural elements is a well-recognized pathological entity affecting the lumbar or cervical spine. Such stenosis of the thoracic spine in the absence of a generalized rheumatological,metabolic, or orthopedic disorder, or a history of trauma is generally considered to be rare. Spinal ligaments, such as the ligamentum flavum (LF), are prone to degeneration and can lead to back pain and nerve dysfunction. Ossification of ligamentum flavum (OLF) is a pathological condition that cause neurological symptoms and usually occurs in the thoracic spine and less frequently in the cervical spine. However the disease is now being increasingly recognized as acause of thoracic myelopathy. We report a rare case of thoracic myelopathy caused by OLF. A 48-year-old male presented with a chief complaint of weakness of bilateral lower extremities. Neurological examination revealed sensory deficit at Th 11 level below. Magnetic resonance imaging and computed tomography demonstrated OLF at the right T9–11 level. Thoracicmyelopathy caused by OLF was consider and surgical intervention was performed. Posterior decompression and laminoplasty has been performed for this patient. Keywords: ossification of ligamentum flavum, thoracic myelopathy, laminoplasty

Temporal evolution of a patient with a spinal dural arteriovenous fistula on serial MRI.

A spinal dural arteriovenous fistula is a rare type of vascular malformation. If left untreated, these fistulas can result in significant neurological deficits secondary to spinal cord infarct or hemorrhage. A 70-year-old female with a longstanding history of episodic progressive bilateral lower extremity weakness and sensory disturbances was previously misdiagnosed with multiple sclerosis. Imaging revealed a T2 signal change from T7 to the conus with associated signal change and she subsequently underwent a T10-L1 laminectomy for clip ligation of a spinal dural arteriovenous fistula. Here we present the clinical and radiographic progression of one patient with a spinal dural arteriovenous fistula. Spinal dural arteriovenous fistulas are a rare but treatable cause of myelopathy, so it is important to understand its natural progression and radiologic findings as it is frequently misdiagnosed.

Reversible thoracic myelopathy after surgical decompression and removal of paddle neurostimulator lead and encasing fibrosis: Technical video case report

Abstract Spinal cord stimulation is an established and safe technique for the treatment of medically refractory and chronic pain. Electrodes or paddle leads can be used for stimulation. Most complications reported are related to hardware insertion, and the devices are otherwise usually well tolerated. There have been several reports of epidural fibrosis formation around the leads with varying degrees of spinal cord compression, but no descriptions of the technical treatment of these lesions, or of patient outcome after surgery. We describe the interesting case of an 85-year-old woman with a thoracic spinal cord stimulator implanted 8 years prior to presenting with a 5-month history of bilateral lower extremity weakness, and myelopathic signs on exam, as well as intractable thoracic pain. Her CT Myelogram revealed significant epidural fibrosis around her thoracic paddle lead with compression of her spinal cord. After surgical removal of the lead and the surrounding fibrosis, she recovered her lower extremity function and her thoracic pain improved. This is an uncommon complication of spinal cord stimulator lead or paddle placement, but can carry a clinically significant impact. Unusual fibrotic scar formation around paddle or percutaneous leads should be suspected in patients experiencing worsening axial pain with decreasing relief from their spinal cord stimulator, especially in the context of progressive myelopathic symptoms.