Lower Extremity Weakness Research Articles

Inflammatory Polyneuropathy Associated with Myelodysplastic Syndrome

Inflammatory polyneuropathy may be associated with various tumorous diseases, including hematological malignancy. A 50-year-old man visited neurologic department complaining of bilateral lower extremities weakness, tingling sensation and weight loss. Initially he was diagnosed as demyelinating sensorimotor polyneuropathy after performing the nerve conduction study, furthermore myelodysplastic syndrome was revealed by the hematologic tests and bone marrow examination. Conclusively, high-dose steroid therapy, intravenous immunoglobulin and chemotherapy improved neurological symptoms. This case suggests myelodysplastic syndrome could be uncommon cause of the inflammatory polyneuropathy.

PMON285 Hypokalemic Periodic Paralysis: A Rare Presentation of Primary Hyperaldosteronism

Abstract Background Primary hyperaldosteronism (PHA) is caused by excessive production of aldosterone from the adrenal glands. Aldosterone levels are usually independent of renin, angiotensin II or sodium status, and often result in hypertension (HTN), hypokalemia and metabolic alkalosis. The most common presentation of this condition is with asymptomatic mild to severe or refractory HTN. This case highlights a patient presenting with hypokalemic periodic paralysis as the first sign of PHA. Clinical Case A 35 year old previously healthy Chinese male, presented with sudden onset bilateral lower extremity weakness for 1 day. This was associated with mild transient lower back pain and fatigue. He had no history of trauma, recent travel, exposure to animal or tick bites. He reported no recent illness or vaccination. His family history was negative for hypertension and various endocrinopathies. The patient was not on any medications and did not consume licorice. His exam was significant for a blood pressure (BP) of 217/136 mmHg, with lower limb evaluation revealing power of 3/5, normal reflexes and a positive heel to shin test. He demonstrated an unsteady gait but Romberg's sign was negative. Upper limb exam was normal. Laboratory findings were remarkable for potassium 1.9 mmol/L, bicarbonate (CO2) 37 mmol/L, normal magnesium (Mg), normal TSH and blood gas showing metabolic alkalosis. Subsequent testing showed renin 0.35 ng/ml/hr, aldosterone 23 ng/dl and aldosterone/pra ratio 66. Saline infusion test confirmed the diagnosis of PHA. Other endocrinologic tests were normal. Electrocardiogram showed prolonged QT interval and U waves. Renal ultrasound was negative for renal artery stenosis and CT abdomen/pelvis showed a 2.4 cm left adrenal adenoma. The patient was admitted to the intensive care unit for 24 hours, where he received intravenous potassium replacement and was started on spironolactone and other antihypertensive medications, which he was discharged on, along with oral potassium. His symptoms resolved after potassium levels normalized and he subsequently had a left adrenalectomy. Conclusion With PHA being the most common endocrinological cause of HTN, clinicians should recognize indicators of underlying PHA even in rare presentations such as this. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Open aortic reconstruction for middle aortic syndrome associated with right renal artery poststenotic aneurysm.

A 24-year-old woman was diagnosed with high blood pressure when she was 14 years old. During the last 4 years, she has experienced progressive symptoms of lower extremity weakness and fatigue and cannot do any heavy exercise. On physical examination, she presented with absent bilateral femoral pulses. Ultrasound ankle-brachial index (ABI) with Doppler of the lower extremities showed diffuse monophasic waveforms and diminished ABI bilaterally (right ABI = 0.68, left ABI = 0.65). A computed tomography angiography confirmed a severe narrowing of the aorta just distal to the origin of the left renal artery consistent with the diagnosis of middle aortic syndrome.

Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin abnormalities (POEMS) Syndrome in a Filipino Nurse Returning to Work Following Bortezomib Treatment: A Case Report

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) also known as Crow-Fukase syndrome or Takatsuki syndrome is a rare and disabling paraneoplastic syndrome that frequently occurs in the fifth or sixth decade of life without a known standard first-line therapy. A 34-year-old Filipino male nurse, who presented with gradually progressive distal lower extremity weakness and sharp shooting pain in bilateral legs accompanied by mild joint effusion on the left knee, hypertrichosis, bilateral conjunctival injection, and gynecomastia underwent extensive workup and was diagnosed with POEMS syndrome. Complete blood count revealed erythrocytosis and thrombocytosis with elevated serum VEGF (vascular endothelial growth factor) and elevated monoclonal serum free lambda light chains. The electrophysiologic studies revealed chronic demyelinating sensorimotor polyneuropathy while bone marrow core biopsy and bone marrow aspirate smear immunohistochemical staining showed it to be positive for lambda and CD138. He had an initial unsuccessful treatment course with melphalan and prednisone. Hence, bortezomib and dexamethasone were given which gave significant improvement in symptoms from the overall neuropathy limitation score of 5 to 1.

Radiographic presentation of a multi-compartmental atypical giant lipoma of the lower extremity

A 61-year-old female with no significant past medical history presented with painless progressive left lower extremity weakness and foot drop resulting in altered gait over the course of two years prior to seeking care. Examination revealed a hypertrophied left lower extremity, steppage gait, and 1 out of 5 strength with left ankle dorsiflexion. Initial x-ray of the affected ankle was unremarkable.

Progressive lower extremity weakness as the initial presentation of isolated large thoracic plasmacytoma

Plasmacytoma is a tumor of monoclonal plasma cells of bone or soft tissue that can occur anywhere in the body without evidence of systemic multiple myeloma. It may present as solitary or multiple masses and is classified as osseous if arising from bone or extraosseous if arising from soft tissue. Osseous plasmacytoma is the most common form of plasmacytoma with predominance in active hematopoietic bones, including vertebrae, femur, pelvis, and ribs. The diagnosis is made with a tissue biopsy. Different imaging modalities allow for tumor localization, and magnetic resonance imaging (MRI) is the gold standard to detect spinal cord compression. Corticosteroids provide analgesia, reduce vasogenic edema, and have anti-myeloma activity which may result in better neurological outcomes in cases of acute spinal cord compression. Corticosteroids should be started promptly once cord compression is suspected. We report a case of progressive lower extremity weakness as the initial presentation of a thoracic plasmacytoma. The patient was started on a high-dose corticosteroid after acute cord compression was suspected. Magnetic resonance imaging confirmed cord compression. T1-T2 corpectomy with C5-T5 posterior spinal fusion for decompression and stabilization was done. He was successfully discharged to an inpatient rehabilitation facility with plans for definitive radiotherapy. Worsening back pain and lower extremity weakness in elderly patients should raise concerns for acute cord compression. Early intervention to relieve compression is crucial to preserve neurological functions.
 
 Keywords: plasmacytoma, weakness, steroids, multiple myeloma

BILATERAL PULMONARY EMBOLISM MASQUERADING AS MEDIASTINAL LYMPHADENOPATHY DIAGNOSED WITH ENDOBRONCHIAL ULTRASOUND

BILATERAL PULMONARY EMBOLISM MASQUERADING AS MEDIASTINAL LYMPHADENOPATHY DIAGNOSED WITH ENDOBRONCHIAL ULTRASOUND

MYASTHENIA GRAVIS PRECIPITATED BY ASYMPTOMATIC COVID-19 INFECTION, VACCINE, OR BOTH? HOW GENETIC PREDISPOSITION AND IMMUNE RESPONSES PLAY A ROLE IN THE NEUROMUSCULAR JUNCTION

MYASTHENIA GRAVIS PRECIPITATED BY ASYMPTOMATIC COVID-19 INFECTION, VACCINE, OR BOTH? HOW GENETIC PREDISPOSITION AND IMMUNE RESPONSES PLAY A ROLE IN THE NEUROMUSCULAR JUNCTION

LEFT ATRIAL SEPTAL POUCH IN A PATIENT WITH RECURRENT STROKE

LEFT ATRIAL SEPTAL POUCH IN A PATIENT WITH RECURRENT STROKE

DELAYED CEREBRAL VENOUS SINUS THROMBOSIS (CSVT) AFTER AN INVASIVE MENINGIOMA RESECTION: AN UNCOMMON PRESENTATION OF A COMMON COMPLICATION

DELAYED CEREBRAL VENOUS SINUS THROMBOSIS (CSVT) AFTER AN INVASIVE MENINGIOMA RESECTION: AN UNCOMMON PRESENTATION OF A COMMON COMPLICATION

S2025 Eosinophilic Enterocolitis Improved After Breast Reduction

Introduction: Hypereosinophilic Syndrome (HES) is a group of disorders characterized by an accumulation of eosinophils within peripheral tissues or blood, with manifestations dependent on organ involvement. Eosinophilic gastrointestinal disorders (EGIDs) is one manifestation. In our case, it is believed that antigenic leakage from ruptured silicone breast implants was the proximate cause, as the patient had complete resolution of symptoms and normalization of labs after implant removal. Case Description/Methods: Our patient is a 59-year-old female with a history of asthma, who presents with new-onset sharp epigastric pain worsened with oral intake and associated with diarrhea. Recent evaluation at another hospital for leg weakness revealed peripheral eosinophilia and electromyography showed chronic inflammatory demyelinating neuropathy. After a full diagnostic evaluation for HES, she was diagnosed with idiopathic HES and treated with low dose steroids, IVIG and plasmapheresis without clinical improvement. At our hospital, labs revealed a white count of 35,000/μL, eosinophil count of 21,960/mm3 and IgE of 1299 IU/mL (Table). A CT scan revealed descending colon wall thickening, and intracapsular rupture of the right breast implant. Enteroscopy demonstrated erosions and ulcerations of the duodenum and jejunum. Colonoscopy showed areas of focal ulcerations with surrounding inflammation separated by normal mucosa and histology showed eosinophilic infiltration throughout. Video capsule endoscopy demonstrated diffuse inflammation and ulceration of the entire small bowel. She was empirically treated for strongyloides before starting high dose prednisone therapy. A month later, her breast implants were removed elsewhere. After a short interval, she was successfully tapered off steroids and achieved resolution of both her GI symptoms and lower extremity weakness, as well as normalization of lab work (Figure). Discussion: EGIDs are believed to be multifactorial with an emphasis on immunologic factors. Eosinophilic infiltration seen on biopsies and exclusion of secondary causes is required. Treatment is aimed at symptomatic improvement and removal of offending agent, as seen with our patient who experienced complete clinical resolution after removal of her silicone breast implants. Our case highlights a rare case of eosinophilic enterocolitis associated with a ruptured breast implant in an otherwise healthy patient and emphasizes the importance of remaining vigilant of all external factors that may be contributing to the disease process.Figure 1.: A, B: Small intestine duodenum & Jejunum. C, D: Colonic areas of inflammation. Table 1. - Initial labs versus labs post implant removal Units Reference Labs Initial Labs Labs post implant removal White Blood Count μL 4.0 - 10.5 35 x 103 7.1 Hemoglobin g/dL 11.2 - 15.7 9.6 11.2 Platelets μL 150-400 508 x 103 369 Eosinophil # μL 0.04 - 0.36 21,960 2.5 Eosinophils % 0.7-5.8 61 35 IgE IU/mL < 100 1299

S3016 Hepatocellular Carcinoma as an Uncommon Cause of PTHrP-Mediated Hypercalcemia

Introduction: Humoral hypercalcemia of malignancy driven by PTHrP is a syndrome frequently associated with squamous cell carcinoma. We present a case of humoral hypercalcemia as a rare manifestation of hepatocellular carcinoma and describe the impact of early diagnosis on patient care. Case Description/Methods: A 67-year-old male with HCC presented to the emergency department with subacute confusion, constipation, and lower extremity weakness. Physical exam revealed tangential thinking and no asterixis. Serum calcium was 14.8 (corrected to 15.3), creatinine 1.36 (baseline 0.8), AST 141, ALT 32, and AFP 135 (all previously normal). Imaging revealed new liver lesions in multiple segments, enlarged lymph nodes in the porta hepatis, widespread lytic lesions in the spine, calvarium, and facial bones with a 3.4 cm x 2.4 cm left orbital wall mass consistent with metastatic disease. Serum calcium levels normalized with IV fluids, calcitonin, and zolendronic acid. The PTHrP was elevated at 79. Vitamin D levels, SPEP and UPEP were normal. He returned one week later with recurrent severe hypercalcemia. No further cancer treatment options were available so the patient elected for hospice services and died at home. Discussion: Malignancy is the most common cause of hypercalcemia in the inpatient setting. Malignancy-associated hypercalcemia occurs through three distinct mechanisms – humoral (PTHrP mediated), lytic bone lesions, and increased absorption due to excess 1,25 vitamin D production by the cancer. Most cases of HCC-associated hypercalcemia are due to metastatic lytic bone lesions. Less than 10% of cases are attributable to PTHrP, which is severe and often refractory. The mechanism for this is unknown but may be related to an underlying paraneoplastic syndrome and downstream metabolic derangements. Humoral hypercalcemia in patients with HCC is associated with a more advanced TNM stage and higher tumor burden. In a study of 534 patients with HCC, 6.3% had humoral hypercalcemia and had worse prognoses by Child-Pugh scores. Another study of 165 patients with HCC and PTHrP-mediated hypercalcemia found a median survival time of 15 days. Early recognition of humoral hypercalcemia in patients with HCC is essential given its association with higher mortality. Doing so can help providers expedite locoregional therapy or guide goals of care discussions with their patients.

S3299 Acute Liver Injury as Initial Presentation of Adult Onset Still’s Disease

Introduction: We present a case of acute liver injury as a first presentation of Adult-Onset Still’s Disease (AOSD). Case Description/Methods: A 20-year-old man presented with fever and nausea. On admission liver function tests (LFTs) were notable for ALT 81 IU/L, AST 42 IU/L, ALP 58 IU/L and total bilirubin 6.7 mg/dL. Infectious work up was notable for positive EBV IgM that was treated appropriately. He presented again with worsening headache, fever, dark urine and petechial rash on both lower extremities. LFTs were still elevated. Patient was symptomatically managed and the elevation in LFTs was attributed to infectious etiology. Patient was discharged home with close follow up. He presented again, nine days after initial symptoms onset with worsening symptoms, jaundice and new left hip pain with lower extremity weakness. Patient had taken one pill of Metronidazole from India but denied any other drugs. LFTs were ALT 214 IU/L, AST 215 IU/L, ALP 215 IU/L and total bilirubin 6.6 mg/dL. Patient’s liver enzymes continued to rise despite discontinuing hepatotoxic medications, with ALT 186 IU/L, AST 146 IU/L, ALP 219 IU/L, total bilirubin 10.7 mg/dL and direct bilirubin 6.4 mg/dL. Therefore, a liver biopsy was ultimately performed. Histology showed a portal inflammatory process, with acute eosinophil spill over to the liver, without hepatic necrosis. Rheumatology team was consulted due to arthralgia. The patient met the Yamaguchi criteria for AOSD. He was started on prednisone 40 mg daily, with subjective and objective improvement with down trending LFTs. Outpatient follow-up with both rheumatology and hepatology continued to show significant improvement and down-trending LFTs. He continues to be on a prolonged steroid taper over 12 weeks. Discussion: In our case the patient met the Yamaguchi criteria which involves major and minor criteria with transaminitis included. In this case, the main challenge was to find a suitable diagnosis that fits all the symptoms. A previous case report suggested that Drug Induced Liver Injury (DILI) might be the inciting factor in AOSD activation, which might be proposed in our case as well. It was reported that liver involvement in AOSD can be attributed to its association with Macrophage Activation Syndrome (MAS). Treatment mainly focuses on treating AOSD itself with systemic steroid therapy. In conclusion, acute liver injury is being increasingly reported in literature and this case supports acute liver injury in association with AOSD which can be aggravated by DILI or MAS.

A Rare Case of Epithelioid Hemangioma Presenting as an Isolated Sacral Mass

Epithelioid hemangioma (EH) is an uncommon benign vascular tumor of mesenchymal origin. It mainly presents as expanding nodules around the ear, the forehead, and long bones. Only a handful of cases have been found in cervical, thoracic, lumbar, and sacral vertebrae as lytic lesions with pain and neurological impairment. We present the case of a 36-year-old female with an incidental finding of a sacral mass along with inguinal lymphadenopathy on imaging. Initially, there were no symptoms. The mass gradually progressed and later showed an extraosseous extension with involvement of sacral neural foramina and nerve roots causing severe low back pain and weakness of the left lower extremity. Differential diagnoses initially included secondary metastases and chordoma. However, the biopsy of the mass revealed findings consistent with an EH. To our knowledge, this is the first case of EH presenting as an isolated mass in the sacrum and the third case of EH involving the sacrum in continuation with other vertebrae.EH should be in our differential diagnoses when there is a sacral mass.

S3275 A Peculiar Case of Limb-Girdle Muscular Dystrophy Masquerading as Elevated Transaminases

Introduction: A disproportional elevation in aspartate transaminase (AST) and alanine transaminase (ALT) is considered a marker of hepatocellular injury. Guidance on the work up of chronically elevated liver chemistry suggests an algorithmic approach to testing of common causes of liver injury based on the degree of elevation and even mentions consideration of nonhepatic etiologies such as skeletal muscle damage. We present a unique case of asymptomatic elevation in liver chemistries as the herald finding in a patient with limb-girdle muscular dystrophy (LGMD). Case Description/Methods: Our patient is a 19-year-old adopted male who was referred to outpatient gastroenterology clinic for asymptomatic elevation in transaminases. AST was 133 IU/L and ALT was 223 IU/L. Total bilirubin and alkaline phosphatase were normal. He denied alcohol use, prescribed medications, herbal supplements, or weightlifting supplements. BMI was normal. Workup for viral hepatitis, hemochromatosis, autoimmune hepatitis, Wilson’s disease, alpha-1 antitrypsin, celiac disease, and thyroid disease were normal. Liver biopsy revealed reticulin collapse with complete regeneration without inflammation. Subsequent follow-up over years demonstrated persistently elevated but stable elevations. At the age of 24, he began to endorse proximal lower extremity muscle pain and weakness. Creatine kinase (CK) was elevated at 11,937 U/L; aldolase was elevated at 31.4 U/L; myositis workup was normal. Right thigh biopsy revealed a myopathic process. Genetic testing confirmed mutation in the CAPN3 gene consistent with calpainopathy, a subtype of LGMD. Discussion: LGMD is a rare, genetic disorder resulting in loss of ambulation within 20 years after disease onset. Concurrent liver disease is rare in LGMD, but elevations in transaminases can be found because of severe elevations in CK. Our patient was found to have calpainopathy, a subtype associated with a recessive mutation in the CAPN3 gene. Our case demonstrates that beyond the recommended algorithmic evaluation of abnormal liver chemistries, inconclusive evaluation of abnormal liver chemistries in children and young adults should include a CK level to further investigate nonhepatic causes, specifically a myopathic process. Although there is no cure for LGMD, interventions focused on muscle conditioning are more beneficial with early detection to mitigate the devastating effects of this disorder.

RARE CASE OF FULMINANT OF INTERSTITIAL LUNG DISEASE SECONDARY TO IDIOPATHIC INFLAMMATORY MYOSITIS

RARE CASE OF FULMINANT OF INTERSTITIAL LUNG DISEASE SECONDARY TO IDIOPATHIC INFLAMMATORY MYOSITIS

Guillain-Barré Syndrome: A Case Report of Post Covid-19 Vaccination in the Philippines

Guillain-Barré Syndrome (GBS) is a complex autoimmune disorder where a person’s own immune system damages the nerve and is usually characterized by ascending symmetrical weakness of the upper and lower extremities (Wijdicks &amp; Klein, 2017). It is a rare condition and the worldwide frequency of GBS is only 2 in 100,000 adult individuals (McGrogan et al., 2009). Studies have shown its association with different vaccines but in these pandemic times, there is a lack of literature on post-COVID-19 vaccination-associated GBS. We report a case of a 68-year-old male from Caloocan City with an initial complaint of febrile illness followed by distal lower extremity weakness which started seven (7) days after the patient received his first Sinovac-CoronaVac vaccine. We report a case of GBS that is related to the Sinovac-CoronaVac vaccine which adds to the body of literature that is currently available and may reflect a possible link.

Inadvertent Spinal Subdural (SSH) and Subarachnoid hematoma (SAH) from Epidural Blood Patch (EBP)

Inadvertent injection of autologous blood in both subdural and intrathecal spaces following EBP is extremely rare. Case reports describe SSH or SAH after attempted EBPs. This illustration depicts combined subdural and subarachnoid collection after EBP for post dural puncture headache (PDPH)(from a lumbar spinal drain), 6 days after 40 ml of autologous blood was injected in the “epidural space” at the same spinal level as the preceding spinal drain. The T1-weighted sagittal (Panel A) and axial (Panel B) MR image of the spine demonstrates a subdural collection of blood (white arrows) extending from T12-L1 to sacral level and subarachnoid collection of blood (asterisks) extending from L3-4 to the termination of the thecal sac at S2. Mild backache and neck stiffness is not uncommon after EBPs. Worsening back pain with radicular symptoms suggests blood in the subdural or subarachnoid spaces which can result in meningitis, arachnoiditis, lower extremity weakness and radiculopathy.

Pelvic and central nervous system tuberculosis complicated by a paradoxical response manifesting as a spinal tuberculoma: a case report

BackgroundThe post-partum period is a risk factor for tuberculosis (TB), possibly including the period after miscarriage as illustrated here. This case demonstrates how non-specific symptoms can hide widely disseminated TB.Case presentationA healthy 26-year-old female with a history of recent miscarriage presented to the emergency department with non-specific symptoms of headache, abdominal pain, and sub-acute fevers. She had immigrated to the United States from the Marshall Islands 9 years prior. Two months prior to presentation she had a miscarriage at 18 weeks of pregnancy. On admission, transvaginal ultrasound revealed retained products of conception and abdominal computed tomography revealed findings consistent with tubo-ovarian abscesses and peritonitis. The obstetrics and gynecology service performed dilation and curettage (D&C) to remove retained products of conception. Acid-fast bacilli cultures from cerebrospinal fluid as well as specimens from D&C and intra-abdominal abscesses subsequently all grew TB. She was diagnosed with TB meningitis, peritonitis, endometritis, and tubo-ovarian abscesses. Her treatment course was complicated by a paradoxical response resulting in a spinal tuberculoma causing lower extremity weakness. The tuberculoma was treated with surgical decompression as well as continuation of treatment with anti-tubercular chemotherapy and steroids.ConclusionDisseminated and extrapulmonary TB can present with non-specific symptoms. Recognition of risk factors for TB is critical for prompt diagnostic evaluation and treatment of this deadly disease. A paradoxical reaction needs to be taken into consideration when any new neurological symptoms occur during TB treatment.

Lumbar Solitary Osteochondroma with Lower Extremity Weakness: A Case Report

Background: Osteochondroma is the most common benign bone tumor. However, osteochondroma of the spine is relatively rare, accounting for only 4% to 7% of primary benign spinal tumors and less than 3% of all osteochondromas. Furthermore, symptomatic osteochondroma in the lumbar spine is much rarer. We herein describe a patient who was diagnosed with a lumbar solitary osteochondroma causing neurological symptoms of the lower extremities. The patient successfully underwent surgical removal of the lesion. Case Presentation: A 79-year-old man presented with a 3-year history of difficulty walking and gradual development of numbness in his left upper extremity, which further exacerbated his difficulty walking. Magnetic resonance imaging and computed tomography revealed spinal stenosis at C3/4, L4/5, and L5/S1 and an osseous solitary lesion. We performed a single-stage operation of both the cervical and lumbar spine. The osseous solitary lesion at L5/S1 was removed, and the pathological diagnosis was osteochondroma. After the operation, the patient’s upper extremity numbness and walking ability were improved. Conclusions: We experienced a rare case of a neurologically symptomatic solitary osteochondroma in the lumbar spinal canal. Moreover, this lesion was found at the same time as cervical spondylosis causing numbness. Therefore, we must perform careful examinations and thoroughly evaluate imaging and postoperative pathological findings.