Charcot arthropathy in the elbow caused by hydrosiringomyelia

Introduction: Syringomyelia is a chronic disease of the spinal cord that leads to damage to nerve fibers in the spinothalamic tract. The changes in these structures responsible for the thermal and painful sensitivity lead to an abnormal innervation of the joints, which can lead to neuropathic arthropathy, called Charcot arthropathy. Syringomyelia is the main cause of Charcot arthropathy in the upper limbs, and the most involving joints are the shoulder and elbow. It is a rare condition and its recognition allows for early diagnosis and proper management. Case report: A 50-year-old female patient, with a previous history of spinal cord trauma, who has been in a wheelchair since then, started with edema in the left upper limb, mainly in the elbow, evolved with local ulcer and presence of serosanguinolent secretion, weakness and paresthesia in the left hand. Magnetic resonance imaging of the elbow showed signs of neuroarthropathy and the study of the thoracic cervical spine showed enlargement of the cerebrospinal fluid space and tapering of the spinal cord. Conclusions: Charcot’s arthropathy should be considered as a differential diagnosis of pain, edema and limitation of joint movements. When present in the upper limbs, an investigation should be carried out to exclude syringomyelia.

Novel Pathology Classification System (FEMASK-Score) for Charcot Neuropathic Arthropathy Reveals Intraneural Vasculopathy and Predicts Outcome

Category: Basic Sciences/Biologics; Ankle; Diabetes; Midfoot/Forefoot Introduction/Purpose: Charcot neuropathic arthropathy (CNA) is a debilitating, rapidly destructive degenerative joint disease that occurs in diabetic, neuropathic midfoot. Clinicoradiologic assessment for CNA previously relied on Eichenholtz Stage. There is limited data on CNA histopathology. The goal of this study was to independently develop a histopathologic scoring system for Charcot neuropathic arthropathy. Methods: Retrieval of surgical pathology specimens from neuroarthropathic CNA patients (2012-2019) were analyzed to evaluate joint soft tissue and bone. Considering progression from large to small periarticular bone fragments to resolution, we devised and applied a CNA FEMASK-score (named after coauthors): 0= intraneural arteriolosclerosis; 1= large bone fragments without host histiocytic response; 2= mixed bone fragments with host histiocytic response; 3= small minute bone spicules resorption to fibrosis. Clinical modified Eichenholtz staging and outcome were then compared the CNA FEMASK-score to assess for associations between these three elements. Results: Forty-eight cases of CNA included 34 males and 14 females, mean age 60.3 and age range 28-83 years, with clinical diabetes mellitus (predominantly Type II) and longstanding neuropathy. Elevated HbA1C, Eichenholtz stage, American Society of Anesthesia score, and Charlson comorbidity index were predictive of amputation. Pathologic specimens varied from fixation tissue to amputation. In addition to neurotraumatic, neurovascular and inflammatory findings, a distinctive intraneural hyalinized arteriolosclerosis was observed. FEMASK-scores:1 = 10%, 2= 58%, and 3=32%. FEMASK-score comparisons were 98% accurate compared with the modified Eichenholtz criteria scores of each patient and 98% reproducible among pathologists. FEMASK 2 and 3 correlates strongly with amputation. Conclusion: Our novel CNA FEMASK-score classification, derived from the largest cohort of diabetic neuropathic specimens, is reproducible, explains pathophysiology, correlates with Eichenholtz, and predicts amputation. The unique intraneural vasculopathy observed contributes to CNA etiology.

The many facets of neuropathic arthropathy

Neuropathic arthropathy, also known as Charcot arthropathy, refers to progressive and occasionally rapid joint destruction that results from underlying disorders of the nervous system. This pictorial essay aims to illustrate various radiologic findings in neuropathic arthropathy using various examples in the upper and lower limbs and in the spine. Pearls for radiologic diagnosis, clinical differential considerations and possible complications are discussed individually for each joint. MR imaging techniques for differentiating infection and neuropathic arthropathy are explained with examples. Management issues are outlined.

Multiple sclerosis neuropathic arthropathy of elbow: Report of a rare case and literature review

Neuropathic arthropathy (NA) is a progressive degenerative and destructive joint disease associated with underlying chronic neurologic deficit. A 34-year-old woman was referred to our tumor clinic with swelling and destruction of her left elbow joint. Except for a 5-year history of multiple sclerosis (MS), she had no other underlying diseases. Suspected of tumor involvement, she had been undergone an open biopsy. The pathologic report was fibromatosis, which was not compatible with imaging studies and clinical presentations. Second surgical inspection and cellular study didn’t prove tumoral involvement. Magnetic resonance images (MRIs) showed multiple hyper signal plaques in her cervical spine. MRI findings, clinical features, surgical observations, and disproportionate painless elbow destruction all confirmed the MS NA diagnosis.