Z-line degradation and phagocytosis of sarcomeric fragments in myonecrosis

Author(s):  
Melinda L. Estes ◽  
Samuel M. Chou

Many muscle diseases show common pathological features although their etiology is different. In primary muscle diseases a characteristic finding is myofiber necrosis. The mechanism of myonecrosis is unknown. Polymyositis is a primary muscle disease characterized by acute and subacute degeneration as well as regeneration of muscle fibers coupled with an inflammatory infiltrate. We present a case of polymyositis with unusual ultrastructural features indicative of the basic pathogenetic process involved in myonecrosis.The patient is a 63-year-old white female with a one history of proximal limb weakness, weight loss and fatigue. Examination revealed mild proximal weakness and diminished deep tendon reflexes. Her creatine kinase was 1800 mU/ml (normal < 140 mU/ml) and electromyography was consistent with an inflammatory myopathy which was verified by light microscopy on biopsy muscle. Ultrastructural study of necrotizing myofiber, from the right vastus lateralis, showed: (1) degradation of the Z-lines with preservation of the adjacent Abands including M-lines and H-bands, (Fig. 1), (2) fracture of the sarcomeres at the I-bands with disappearance of the Z-lines, (Fig. 2), (3) fragmented sarcomeres without I-bands, engulfed by invading phagocytes, (Fig. 3, a & b ), and (4) mononuclear inflammatory cell infiltrate in the endomysium.

2019 ◽  
Vol 34 (6) ◽  
pp. 831-831
Author(s):  
S John ◽  
M Silva ◽  
N Newman ◽  
D Loring

Abstract Objective We present a patient with rapidly progressive visual decline of 2-year duration that interfered with daily functioning. She was evaluated by neuro-ophthalmology and neurology prior to neuropsychological referral. A series of evaluations led to diagnosis of posterior cortical atrophy, demonstrating the importance of inter-departmental collaboration. Method A 66-year old white female presented with a 2-year history of progressive changes to vision and memory. Medical history included hypertension, dyslipidemia, and a strong family history of optic neuropathy causing blindness. She was diagnosed with a left homonymous hemianopia. MRI revealed "significant cortical atrophy more remarkable on the right temporal, parietal, and occipital regions." She reported dressing apraxia, unsteady gait, declines in reading and writing, and difficulty recalling well-learned information. Results She was a good historian, had fluent speech and no apparent comprehension difficulty. Neuropsychological evaluation revealed relatively preserved language and verbal abilities, including confrontation naming, in the presence of otherwise impaired performances across all domains of functioning. She demonstrated agraphia, acalculia, left-right confusion, and difficulties with motor programming. Perceptual and constructional tasks revealed prominent deficits in visual integration, map orientation, form discrimination, and construction of simple geometric designs. She was perseverative and susceptible to verbal and visual stimulus pull. Conclusions The pattern on neuropsychological testing, with prominent visual spatial and perceptual difficulties, was consistent with posterior cortical atrophy. The decline in visual ability is likely exacerbated but not entirely explained by left hemianopia. Neuropsychological, neuroimaging, and visual field evidence demonstrated posterior cortical atrophy in the absence of positive biomarker evidence, leading to initiation of anti-cholinesterase therapy.


2014 ◽  
Vol 05 (04) ◽  
pp. 399-400 ◽  
Author(s):  
Amita N. Bhargava ◽  
Gaurav M. Kasundra ◽  
Subhakaran Khichar ◽  
Bharat S. K. Bhushan

ABSTRACTWe describe a patient who presented with a history of lightning strike injury. Following the injury, he sustained acute right upper limb weakness with pain. Clinically, the lesion was located to the upper and middle trunk of the right brachial plexus, and the same confirmed with electrophysiological studies. Nerve damage due to lightning injuries is considered very rare, and a plexus damage has been described infrequently, if ever. Thus, the proposed hypothesis that lightning rarely causes neuropathy, as against high-voltage electric current, due to its shorter duration of exposure not causing severe burns which lead to nerve damage, needs to be reconsidered.


2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Nedal Bukhari ◽  
Bachar Harfouch ◽  
Majid Shallal Alotaibi ◽  
Hulayel Al-Harbi ◽  
Omar Chamdine

We report a case of a 31-year-old female patient with high-risk neuroblastoma (NBL) who presented with a history of static back pain and bilateral lower limb weakness for almost a month. Her primary tumor was located in the right paraspinal region, causing spinal cord compression (SCC). Chemotherapy was administered with an immediate clinical improvement noted after 24 hours of starting treatment. We herein report the efficacy of chemotherapy in an adult neuroblastoma (aNBL) patient presenting with spinal cord compression.


2020 ◽  
Vol 6 (5) ◽  
pp. 328-332
Author(s):  
Hardeep Singh Mudhar ◽  
Madalina Pavel ◽  
Andrew K.K. Chung ◽  
Thomas Elliott Jackson

An 84-year-old male presented with a history of a slowly growing lesion beneath the right lower lid. Examination showed a right-sided mass in the inferior bulbar conjunctiva, measuring approximately 10 × 6 mm. The lesion was excised and showed a giant cell-rich solitary fibrous tumour (GCR-SFT) with pseudo-vascular spaces with benign cytology, with STAT6, expressed in the spindle, ovoid, and tumour giant cells. A second case, a 26-year-old white female, presented with a 12-month history of a gradually expanding lesion in left inferior bulbar conjunctiva. Examination showed a well-defined, dome-shaped pink fleshy mass of 10 × 7 × 2 mm, on the inferior bulbar conjunctiva and abutting the inferior fornix. Excision followed by histology showed a similar appearance to the first case except that the multinucleate giant cells closely resembled Touton giant cells and did not show the rather hyperchromatic crowded nuclei of the giant cells observed in the first case. No pseudo-vascular spaces were identified. The tumour cells, including the Touton-like multinucleate giant cells, displayed nuclear positivity for STAT6. An unexpected feature was the presence of a Melan A- and Sox10-positive population amongst the GCR-SFT, postulated to be a subepithelial melanocytic nevus colliding with the GCR-SFT.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Luis Enrique Arzeno ◽  
Kimberly Kochersperger Lessard ◽  
Serge A Jabbour

Abstract Persistent hyperglycemia has been associated with vascular damage in patients with uncontrolled diabetes. Special emphasis has been placed on the heart, kidneys, eyes, and brain since those major organs are vital. However, little has been studied in terms of the vascular supply to the muscle and how it could be affected by high blood glucose. Here we present a 26-year-old female with a history of uncontrolled Type 1 Diabetes Mellitus treated with insulin pump who presented with muscle aches on her right lower extremity. During the evaluation at the Emergency Department (ED), the patient was noted to have diabetes ketoacidosis, intravenous fluids and insulin drip were started. As part of the workup for the muscle aches multiple blood studies were ordered including Creatinine Phosphokinase (CPK) 26 IU/L (25 - 185 IU/L), Erythrocyte Sedimentation Rate (ESR) 102 (0 - 20), C-Reactive Protein (CRP) 3.4 mg/dL (&lt;=0.80 mg/dL), Aldolase 7.5 U/L (&lt;=8.1 U/L), White Blood Cell (WBC) was 13.1 B/L (4.0 - 11.0 B/L). At this point, a muscle biopsy was considered given the lack of evidence to support a definite diagnosis. Before proceeding with the biopsy, a Magnetic Resonance Imaging (MRI) of the low extremities was done, showing diffuse intramuscular edema, predominantly in the right vastus intermedius, with additional patchy intramuscular edema in the right vastus lateralis, vastus medialis, and biceps femoris, as well as the left gluteus maximus, vastus lateralis which were compatible with myositis. Also, discrete areas of myonecrosis in the right vastus intermedialis (1.7 x 1.1 x 3.6 cm), left vastus lateralis (1.7 x 0.8 x 6 cm) and left gluteus maximus (2.8 x 3 cm x 6 cm). Given her previous history of uncontrolled diabetes, the clinical presentation with low CPK levels, lack of data to support another diagnosis, and MRI findings the possibility of diabetes myonecrosis was raised. The patient was managed with conservative therapy: intravenous fluids, pain control and aspirin with improvement in myalgias and muscle strength. Diabetic myonecrosis is a rare condition that appears to be related to vasculopathic changes on uncontrolled diabetics. The lack of specific diagnostic tools and the nonspecific symptoms could make this condition to be overlooked easily; leading to unnecessary studies like muscle biopsy with consequences from complications and increased health care expenditure. A high index of suspicion is essential for timely treatment, which is limited to rest, optimal glycemic control, pain control and patients who are candidates low-dose aspirin. This condition resolves spontaneously over a few weeks to months in most patients and acknowledging this condition could provide timely relief and reassurance.


2020 ◽  
Author(s):  
Kanyarat Laoyoung ◽  
Phu Waisayarat ◽  
Charungthai Dejthevaporn ◽  
Chaiyos Khongkhatithum ◽  
Jariya Waisayarat

Abstract Objectives To evaluate the correlation between N-CAM expression and diagnosis of common muscle diseases in the Thai population.Methods The expression of N-CAM was interpreted in 75 muscle biopsy specimens diagnosed with myopathies in a 3-year retrospective study. We used anti-CD56 (MRQ-42) rabbit monoclonal primary antibody (1:200, Cell Marque, MilliporeSigma, Rocklin, CA, USA) in our immunohistochemical study.Results Of our 75 patients, 41 cases were female and 34 were male. The mean age of the patients was 35-year-old, with the range from 3 months to 83 years. 35 (46.67%) of the specimens were N-CAM-positive, and 40 (53.33%) were N-CAM-negative. There were 35 (46.67%) cases diagnosed with nonspecific myopathy, 17 (22.67%) with inflammatory myopathy, 9 (12.00%) with neurogenic dystrophy, 7 (9.33%) with muscular dystrophy, 5 (6.67%) with non-diagnostic myopathy, and 2 (2.67%) with mitochondrial myopathy. Chi-squared testing was used to analyze the N-CAM expression data and the diagnosis. There is a statistically significant correlation between inflammatory myopathy and N-CAM positivity (p-value <0.001, OR 14.250, 95% CI 2.960-68.606), with 15 out of 17 cases being N-CAM-positive. N-CAM was also positive in muscular dystrophy, neurogenic myopathy, and nonspecific myopathy but they were not statistically significant. We did not find N-CAM expression in our 2 mitochondrial myopathy cases, nor in the 5 non-diagnostic myopathy cases.Conclusion N-CAM expression can be a complementary tool for diagnostic evaluation in muscle diseases with regeneration or denervation of muscle fibers. We recommend further study in a larger group.


VASA ◽  
2011 ◽  
Vol 40 (3) ◽  
pp. 251-255 ◽  
Author(s):  
Gruber-Szydlo ◽  
Poreba ◽  
Belowska-Bien ◽  
Derkacz ◽  
Badowski ◽  
...  

Popliteal artery thrombosis may present as a complication of an osteochondroma located in the vicinity of the knee joint. This is a case report of a 26-year-old man with symptoms of the right lower extremity ischaemia without a previous history of vascular disease or trauma. Plain radiography, magnetic resonance angiography and Doppler ultrasonography documented the presence of an osteochondrous structure of the proximal tibial metaphysis, which displaced and compressed the popliteal artery, causing its occlusion due to intraluminal thrombosis..The patient was operated and histopathological examination confirmed the diagnosis of osteochondroma.


Commonwealth ◽  
2017 ◽  
Vol 19 (1) ◽  
Author(s):  
John Arway

The challenges of including factual information in public policy and political discussions are many. The difficulties of including scientific facts in these debates can often be frustrating for scientists, politicians and policymakers alike. At times it seems that discussions involve different languages or dialects such that it becomes a challenge to even understand one another’s position. Oftentimes difference of opinion leads to laws and regulations that are tilted to the left or the right. The collaborative balancing to insure public and natural resource interests are protected ends up being accomplished through extensive litigation in the courts. In this article, the author discusses the history of environmental balancing during the past three decades from the perspective of a field biologist who has used the strength of our policies, laws and regulations to fight for the protection of our Commonwealth’s aquatic resources. For the past 7 years, the author has taken over the reins of “the most powerful environmental agency in Pennsylvania” and charted a course using science to properly represent natural resource interests in public policy and political deliberations.


2015 ◽  
Vol 43 (01) ◽  
pp. 44-38
Author(s):  
C.-C. Lin ◽  
K.-S. Chen ◽  
Y.-L. Lin ◽  
J. P.-W. Chan

SummaryA 5-month-old, 13.5 kg, female Corriedale sheep was referred to the Veterinary Medicine Teaching Hospital, with a history of traumatic injury of the cervical spine followed by non-ambulatoric tetraparesis that occurred 2 weeks before being admitted to the hospital. At admission, malalignment of the cervical spine with the cranial part of the neck deviating to the right was noted. Neurological examinations identified the absence of postural reactions in both forelimbs, mildly decreased spinal reflexes, and normal reaction to pain perception tests. Radiography revealed malalignment of the cervical vertebrae with subluxations at C1–C2 and C2–C3, and a comminuted fracture of the caudal aspect of C2. The sheep was euthanized due to a presumed poor prognosis. Necropsy and histopathological findings confirmed injuries of the cervical spine from C1 to C3, which were consistent with the clinical finding of tetraparesis in this case. This paper presents a rare case of multiple subluxations of the cervical spine caused by blunt force trauma in a young sheep. These results highlight the importance of an astute clinical diagnosis for such an acute cervical spine trauma and the need for prompt surgical correction for similar cases in the future.


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