scholarly journals Prominent Asymmetric Muscle Weakness and Atrophy in Seronegative Immune-Mediated Necrotizing Myopathy

Diagnostics ◽  
2021 ◽  
Vol 11 (11) ◽  
pp. 2064
Author(s):  
Sunha Park ◽  
Dae-Hyun Jang ◽  
Jae-Min Kim ◽  
Nara Yoon
Keyword(s):  
Lower Extremity ◽  
Muscle Weakness ◽  
Inflammatory Myopathy ◽  
Previous History ◽  
Lower Extremity Weakness ◽  
Necrotizing Myopathy ◽  
Left Lower Extremity ◽  
Thigh Muscles ◽  
Immune Mediated ◽  
The Right

Immune-mediated necrotizing myopathy, a new subgroup of inflammatory myopathies, usually begins with subacute onset of symmetrical proximal muscle weakness. A 35-year-old male presented with severe asymmetric iliopsoas atrophy and low back pain with a previous history of left lower extremity weakness. Although his first left lower extremity weakness occurred 12 years ago, he did not receive a clear diagnosis. Magnetic resonance imaging of both thigh muscles showed muscle edema and contrast enhancement in patch patterns, and the left buttock and thigh muscles were more atrophied compared to the right side. Serum creatine kinase levels were elevated, and serologic testings were all negative. Genetic testing using a targeted gene-sequencing panel for neuromuscular disease including myopathy identified no pathogenic variants. Muscle biopsy on the right vastus lateralis showed scattered myofiber necrosis with phagocytosis and an absence of prominent inflammatory cells, consistent with seronegative necrotizing myopathy. Thus, unusual asymmetric muscle weakness and atrophy can be a manifestation of inflammatory myopathy.

scholarly journals Statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like features: A case report

2020 ◽  
Vol 8 ◽  
pp. 2050313X2098412
Author(s):  
Darosa Lim ◽  
Océane Landon-Cardinal ◽  
Benjamin Ellezam ◽  
Annie Belisle ◽  
Annie Genois ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Muscle Weakness ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Intravenous Immunoglobulins ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Therapeutic Implications ◽  
Necrotizing Myopathy ◽  
Immune Mediated

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) immune-mediated necrotizing myopathy is a subtype of idiopathic inflammatory myopathy which may be associated with statin exposure. It presents with severe proximal muscle weakness, high creatine kinase levels and muscle fiber necrosis. Treatment with intravenous immunoglobulins and immunosuppressants is often necessary. This entity is not commonly known among dermatologists as there are usually no extramuscular manifestations. We report a rare case of statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like cutaneous features. The possibility of anti-HMGCR immune-mediated necrotizing myopathy should be considered in patients with cutaneous dermatomyositis-like features associated with severe proximal muscle weakness, highly elevated creatine kinase levels and possible statin exposure. This indicates the importance of muscle biopsy and specific autoantibody testing for accurate diagnosis, as well as significant therapeutic implications.

Abstract 18343: Antisynthetase Syndrome-Associated Myocarditis

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Kavita Sharma ◽  
Ana-Maria Orbai ◽  
Dipan Desai ◽  
Oscar H Cingolani ◽  
Marc K Halushka ◽  
...  
Keyword(s):  
Muscle Weakness ◽  
Cardiac Involvement ◽  
Troponin I ◽  
Acute Decompensated Heart Failure ◽  
Inflammatory Myopathy ◽  
Palliative Therapy ◽  
Left Ventricular ◽  
Caucasian Woman ◽  
Free T4 ◽  
Lower Extremity Weakness

Background: The antisynthetase (AS) syndrome is characterized by fever, non-erosive arthritis, inflammatory myopathy, interstitial lung disease, cutaneous involvement, and antibody specificity. Cardiac involvement is extremely rare; we present one of two cases of AS syndrome-associated myocarditis from our institution. Clinical Case: A 51 year old woman with history of inflammatory arthritis and hypothyroidism presented with 6 months of increasing fatigue, bilateral proximal muscle weakness, leg edema, dyspnea, and orthopnea. Physical examination revealed a Caucasian woman, markedly dyspneic while speaking, with signs of acute decompensated heart failure (HF) including elevated jugular venous pulse, bilateral rales, S3 gallop, and massive peripheral edema with bilateral proximal upper and lower extremity weakness. Diagnostic Testing: Her muscle weakness was concerning for myopathy along with new HF symptoms. Laboratory studies revealed elevations of serum creatine kinase, aldolase, and cardiac troponin I. Thyroid stimulating hormone and free T4 were consistent with subclinical hypothyroidism. MRI and electromyography of the lower extremities and skeletal muscle biopsy were consistent with myositis. Anti-Jo1 antibody was positive, confirming the diagnosis of AS syndrome. Electrocardiogram showed sinus rhythm and low-voltages in all leads. Echocardiography showed severely depressed biventricular function, left ventricular (LV) ejection fraction of 10-15%, normal LV cavity size and wall thickness. Cardiac MRI and endomyocardial biopsy revealed active myocarditis. She received pulse dose corticosteroid therapy, intravenous furosemide, inotropic therapy, and hemofiltration therapy for renal failure. She was discharged home on intravenous dobutamine palliative therapy. Two months later, she presented in cardiogenic shock, failed intensive medical therapy, and died after a cardiac arrest. Conclusions: AS syndrome is a rare entity not typically associated with cardiac involvement. We report one of only two known cases of AS syndrome-associated myocarditis, to our knowledge. Based on these observations, we suggest that myocarditis be considered and evaluated for in patients with AS syndrome presenting with HF.

Endovascular Treatment of an Aneurysm of a Persistent Primitive Hypoglossal Artery With Complete Resolution of Brainstem Compressive Symptoms: Case Report

Neurosurgery ◽  
2011 ◽  
Vol 68 (3) ◽  
pp. E854-E857 ◽  
Author(s):  
Ferdinand K. Hui ◽  
Albert J. Schuette ◽  
Charles M. Cawley
Keyword(s):  
Lower Extremity ◽  
Complete Resolution ◽  
Mass Effect ◽  
Neurological Deficits ◽  
Lower Extremity Weakness ◽  
Computed Tomographic ◽  
Hypoglossal Artery ◽  
Patient Reported ◽  
Persistent Primitive Hypoglossal Artery ◽  
The Right

Abstract BACKGROUND AND IMPORTANCE: Aneurysms of the posterior circulation may manifest with neurological deficits related to mass effect on the brainstem. We present an unusual case of an aneurysm resulting in selective lower-extremity weakness and gait instability. CLINICAL PRESENTATION: A 61-year-old man presents with progressively worsening gait instability over the course of several months. A magnetic resonance image and computed tomographic angiogram demonstrate a persistent hypoglossal artery associated with an aneurysm invaginating into the pontomedullary junction. The patient manifested only lower-extremity symptoms. An endovascular approach through the right internal carotid artery and persistent primitive hypoglossal artery was assayed, coiling off the aneurysm with complete angiographic occlusion. One month after the procedure, the patient reported marked improvement in symptoms with residual difficulty walking. At the 1-year postprocedure interval, he reported nearly complete resolution of symptoms. CONCLUSION: Endovascular therapy of an aneurysm invaginating into the brainstem is safe and efficacious.

Clinical Characteristics of Immune-Mediated Necrotizing Myopathy with Autoantibodies Recognizing the Signal Recognition Particle: A Retrospective Study of a Chinese Cohort

2021 ◽  
Author(s):  
Qi Tang ◽  
Jinshen He ◽  
Feng Li ◽  
Jinwei Chen ◽  
Jing Tian ◽  
...  
Keyword(s):  
Logistic Regression ◽  
Inflammatory Myopathy ◽  
Signal Recognition Particle ◽  
Idiopathic Inflammatory Myopathy ◽  
Lower Limbs ◽  
Hunan Province ◽  
Signal Recognition ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Chinese Cohort

Abstract Objective: Immune-mediated necrotizing myopathy (IMNM) with autoantibodies recognizing the signal recognition particle (SRP) patients tend to have prominent proximal weakness and infrequent extra-muscular involvement, especially interstitial lung disease (ILD). However, we reported a Chinese cohort of anti-SRP IMNM patients with relatively more frequent ILD.Methods: Anti-SRP IMNM patients from September 2016 to November 2019 were included according to the most recent European Neuromuscular Center criteria for IMNM. All sera for anti-SRP autoantibody and other myositis-related autoantibodies detection were obtained before the treatment initiation. Muscle strength, coexisting autoimmunity, complications including ILD, treatment and follow-up outcomes were also recorded. Univariate logistic regression was performed to determine variables predicting bad outcomes.Results: Of 271 patients with idiopathic inflammatory myopathy tested, we diagnosed 23 (8.5%) patients with anti-SRP IMNM. Muscle weakness was presented in 23 patients (100%) and generally worse in the lower limbs. ILD was observed in 50% anti-SRP IMNM patients. Predictor of bad outcomes identified by univariate logistic regression analysis was complicated ILD (odds ratio, 3.8).Conclusion: ILD tends to be more frequent in this Chinese anti-SRP IMNM cohort from Hunan province. Complicated ILD represents a risk factor for bad outcomes for anti-SRP IMNM.

Night Sweats and Paraparesis

2021 ◽  
pp. 199-201
Author(s):  
David N. Abarbanel ◽  
Ivan D. Carabenciov
Keyword(s):  
Lower Extremity ◽  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Lower Extremity Weakness ◽  
Bilateral Lower Extremity ◽  
Left Lower Extremity ◽  
Large B Cell Lymphoma ◽  
Night Sweats ◽  
Large B Cell

A 78-year-old man sought care for saddle anesthesia, left lower extremity numbness, and bilateral lower extremity weakness. The sensory loss occurred suddenly, starting initially in the left perianal region and over the course of 3 hours extending down to involve the entirety of the left lower extremity. Symptoms were stable until 3 weeks later, when he had a few episodes of urinary incontinence. Diffuse, severe, bilateral, lower extremity weakness developed. The patient reported 6 months of intermittent night sweats. Serum studies were notable for pancytopenia and increased erythrocyte sedimentation rate and levels of ferritin and lactate dehydrogenase. Lumbar puncture showed a mildly increased protein concentration with normal blood cell count, glucose value, and cytologic and flow cytometry findings. Magnetic resonance imaging showed multifocal regions of increased T2 signal throughout the central nervous system including the cerebrum, cerebellum, upper cervical cord, lower thoracic cord, and conus medullaris. Gadolinium enhancement was present in the corpus callosum, cerebellum, and dorsal lower thoracic cord. One week later, 18F-fludeoxyglucose–positron emission tomography/computed tomography showed patchy 18F-fludeoxyglucose activity in the cerebral parenchyma, as well as 2 cutaneous, 18F-fludeoxyglucose-avid soft-tissue nodules. Fine-needle aspiration of 1 of these nodules indicated diffuse large B-cell lymphoma, with no dysplastic abnormalities identified on subsequent bone marrow biopsy. Incisional biopsy of the second soft-tissue nodule showed foci of diffuse large B-cell lymphoma adherent to the lumina of a few small arteries, consistent with a diagnosis of intravascular lymphoma. The patient was diagnosed with intravascular large B-cell lymphoma. At initial evaluation at an outside facility, empiric intravenous corticosteroids were administered. After the biopsy findings of intravascular large B-cell lymphoma, he was started on intermediate-dose methotrexate followed by rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone therapy. He continued to experience severe, bilateral, lower extremity weakness and sensory loss. Two months after diagnosis of intravascular large B-cell lymphoma, he died of medical complications from chemotherapy. Intravascular lymphoma is a rare lymphoma subtype that is typically of B-cell origin. The neoplastic cells preferentially grow within the lumen of blood vessels, potentially due to a lack of cellular machinery required for cellular extravasation and parenchymal invasion.

scholarly journals Case of the Week: Preoperative MR/CT Diagnosis of Left L2-L3 Disc Surgically Documented As Massive Synovial Cyst

2019 ◽  
Vol 10 ◽  
pp. 168 ◽  
Author(s):  
Nancy E. Epstein
Keyword(s):  
Lower Extremity ◽  
Synovial Cyst ◽  
Normal Function ◽  
Herniated Disc ◽  
Lower Extremity Weakness ◽  
Left Lower Extremity ◽  
Degenerative Lumbar Disease ◽  
Disc Herniations ◽  
Contrast Ct ◽  
Ct Studies

Background: The diagnosis of a lumbar herniated disc, stenosis, and other degenerative findings are typically established preoperatively with MR scans, supplemented with non-contrast CT studies. Here, a 77-year-old female, diagnosed as having L2-S1 stenosis and a large left-sided L2-L3 herniated disc was found at surgery to have a massive left-sided L2-L3 synovial cyst. Case Description: A 77-year-old female was followed by pain management for 6-months with proximal left lower extremity weakness. The lumbar MR at that time was read as demonstrating a large left L2-L3 disc herniation with inferior migration to the L3 mid pedicle level, accompanied by L2-S1 lumbar stenosis. When she finally consulted neurosurgery, she exhibited severe left iliopsoas and quadriceps weakness (2/5), absent lower extremity reflexes, and profound decreased pin appreciation in the left L2-L3 distributions. The repeat MR and new CT studies confimred a large left L2-L3 disc accompanied by moderate/marked L2-S1 stenosis. However, at surgery, consisting of a laminectomy L2-S1, the supposed left L2-L3 disc proved to be a massive synovial cyst. Postoperatively, the patient regained normal function, and remained neurologically intact 6 months later. Conclusion: In this 77 year-old female, the preoperative MR and CT scans were interpreted as showing a “typical” large left L2-L3 herniated disc. This proved at surgery to be a massive left L2-L3 synovial cyst. As demonstrated in this case, older patients with degenerative lumbar disease/stenosis, may have synovial cysts that mimic disc herniations both clinically and on preoperative diagnostic studies.

scholarly journals Complex Lumbosacral Fracture-Dislocation with Sacral Fracture: Report of a Case and Review of the Literature

2020 ◽  
Author(s):  
Liyan Qu ◽  
Jiashi Song ◽  
Fangcai Li ◽  
Bing Liu
Keyword(s):  
Lower Extremity ◽  
Pelvic Ring ◽  
Sacral Fracture ◽  
Vertical Shear ◽  
Fracture Dislocation ◽  
X Rays ◽  
Severe Injuries ◽  
Pattern Of Injury ◽  
Left Lower Extremity ◽  
The Right

Abstract Purpose: Traumatic lumbosacral fracture-dislocation is a very rare pattern of injury. We report a patient who has been suffered from a traffic accident and resulted in concomitant severe injuries: complex lumbosacral fracture-dislocation with pelvic ring disruption and vertical shear sacral fracture; Pneumothorax. Methods: She was transferred to the emergency department at the local provincial hospital and treated with the pelvic external fixation and closed drainage of thoracic cavity. For the further therapy, she was transferred to our hospital. The neurological examination revealed incomplete S1 paraplegia in the right lower extremity, the left lower extremity was normal, and rectal tone was existed. Standard x-rays and computed tomography revealed L5-S1 fracture-dislocation and fractures of the L5 transverse processes. Results: She was treated with S1 laminectomy and L5-S1 interbody fusion using lumbo-iliac internal fixation. Conclusions: Available literatures on lumbosacral fracture-dislocation were also reviewed which illustrate the pathogenic, clinical, radiological and therapeutic aspects of lumbosacral fracture dislocation.

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