Persistent hemiplegia with normal intraoperative neurophysiological monitoring in supratentorial neurosurgery: a case report and review of literature.

Intraoperative neurophysiological monitoring (IONM) is needed for evaluating and demonstrating the integrity of the central and peripheral nervous system during surgical manoeuvres that take place in proximity to eloquent motor and somatosensory nervous structures. The integrity of the monitored motor pathways is not always followed by consistent clinical normality, particularly in the first hours/days following surgery, when surgical resection involves brain structures such as the supplementary motor areas (SMA). We report the case of a patient who underwent surgical excision of a right frontal glioblastoma with normal preoperative, intraoperative (IONM), and postoperative central motor conduction, but with persistent postoperative hemiplegia (> 6 months). The literature regarding SMA syndrome and its diagnosis and prognosis is reviewed.

[Surgical treatment of tumors of the supplementary motor area]. / Khirurgicheskoe lechenie opukholei dopolnitel'noi motornoi oblasti.

After surgical treatment of tumors of the supplementary motor area (SMA) post-operative speech and/or motor neurological deficit may occur. OBJECTIVE: To determinate frequency and reversibility of such deficit and identify risk factors for its development. MATERIAL AND METHODS: We retrospectively analyzed postoperative outcomes in 34 patients with SMA tumors. Pre- and postoperative neurological status, localization of tumors, extent of resection relative to adjacent regions and relationship of tumor with white matter tracts were assessed. We also analyzed the influence of these factors on the risk of postoperative neurological impairment. RESULTS: Postoperative neurological impairment occurred in 47% of cases. Complete or significant regression was observed in all patients within 5.7 month after surgery. Major risk factors were lesion of dominant hemisphere (p=0.029), tumor spreading to primary motor cortex (p=0.018) and resection of SMA together with cingulate gyrus (p=0.000). Location of frontal aslant tract in dominant hemisphere just near the tumor contributed to disorders regarding speech initiation and fluency (p=0.016). Resection of SMA with cingulate gyrus in dominant hemisphere affected development of more serious speech disorders (p=0.003). CONCLUSION: Surgery for SMA tumors is safe and followed by favorable functional outcomes.

Point-of-care ultrasound findings in the diagnosis and management of Superior Mesenteric Artery (SMA) syndrome.

Superior mesenteric artery (SMA) syndrome is a potentially fatal condition that can be difficult to diagnose for emergency medicine physicians due to its rarity and vague gastrointestinal symptom presentation. Patients arriving at the emergency department (ED) with this condition may encounter delays in proper supportive care and treatment. We present the case of a 21-year-old female who was seen in the ED for nausea, non-bloody vomiting, and rapid weight loss. Through point-of-care ultrasound (POCUS) findings, she was diagnosed with SMA syndrome and received appropriate, supportive care for her condition before catastrophic complications could occur. This case demonstrates the utility of POCUS in SMA syndrome and the importance of considering this diagnosis despite its rarity.

The supplementary motor area syndrome: a neurosurgical review.

The supplementary motor area (SMA) syndrome is a frequently encountered clinical phenomenon associated with surgery of the dorsomedial prefrontal lobe. The region has a known motor sequencing function and the dominant pre-SMA specifically is associated with more complex language functions; the SMA is furthermore incorporated in the negative motor network. The SMA has a rich interconnectivity with other cortical regions and subcortical structures using the frontal aslant tract (FAT) and the frontostriatal tract (FST). The development of the SMA syndrome is positively correlated with the extent of resection of the SMA region, especially its medial side. This may be due to interruption of the nearby callosal association fibres as the contralateral SMA has a particular important function in brain plasticity after SMA surgery. The syndrome is characterized by a profound decrease in interhemispheric connectivity of the motor network hubs. Clinical improvement is related to increasing connectivity between the contralateral SMA region and the ipsilateral motor hubs. Overall, most patients know a full recovery of the SMA syndrome, however a minority of patients might continue to suffer from mild motor and speech dysfunction. Rarely, no recovery of neurological function after SMA region resection is reported.

Avoidant restrictive food intake disorder emerging during COVID-19 pandemic resulting in superior mesenteric artery syndrome.

The COVID-19 pandemic has significantly increased the prevalence of psychiatric disorders within pediatric populations. However, only a limited number of studies have sought to understand the correlation between the pandemic and increased incidence of eating disorders. This case study highlights the hospital course of an 18-year-old female who presented with restrictive eating patterns and intensive exercise regimen, self-attributed to the COVID-19 pandemic, leading to superior mesenteric artery syndrome. In understanding the patient's avoidant restrictive food intake disorder (ARFID), this case study seeks to inform readers of this newer DSM-V diagnosis with the intent of educating pediatric providers of the severity and long-term impact of this disease. Moreover, the case study highlights the importance of gaining a more holistic view of psychiatric disorders emerging as a result of the COVID-19 pandemic.

Free will and neurosurgical resections of the supplementary motor area: a critical review.

BACKGROUND: Research suggests that unconscious activity in the supplementary motor area (SMA) precedes not only certain simple motor actions but also the point at which we become aware of our intention to perform such actions. The extent to which these findings have implications for our understanding of the concepts of free will and personal responsibility has been subject of intense debate during the latest four decades. METHODS: This research is discussed in relation to effects of neurosurgical removal of the SMA in a narrative review. RESULTS: Removal of the SMA typically causes a transient inability to perform non-stimulus-driven, voluntary actions. This condition, known as the SMA syndrome, does not appear to be associated with a loss of sense of volition but with a profound disruption of executive function/cognitive control. CONCLUSIONS: The role of the SMA may be to serve as a gateway between the corticospinal tract and systems for executive function. Such systems are typically seen as tools for conscious decisions. What is known about effects of SMA resections would thus seem to suggest a view that is compatible with concepts of personal responsibility. However, the philosophical question whether free will exists cannot be definitely resolved on the basis of these observations.

Postoperative isolated lower extremity supplementary motor area syndrome: case report and review of the literature.

The supplementary motor area (SMA) syndrome is characterized by transient weakness and akinesia contralateral to the side of the affected hemisphere. The underlying pathology of the syndrome is not fully understood but is thought to be related to lesions in the SMA, residing principally in the mesial superior frontal gyrus (Broadmann's area 6c). Although the SMA syndrome a well-characterized clinical entity, we report herein, to our knowledge, the first case of isolated lower extremity SMA syndrome in the literature. This case highlights the importance of considering this rare clinical entity in the context of new or worsening postoperative neurologic deficits. Moreover, early studies did not support somatotopic organization of the SMA as in the primary motor cortex; emerging evidence suggests that delicate somatotopic representation may underlie distinct presentations like that reported in the present case.

Supplementary motor area syndrome after surgery for parasagittal meningiomas.

BACKGROUND: Resection within the supplementary motor area (SMA) may be accompanied by dramatic motor deficits and speech arrest when the dominant hemisphere is involved, termed the SMA syndrome. Typically, the muscle tone of the paralyzed extremities is preserved, and in most cases, a complete or near complete recovery is seen within a few months. The SMA syndrome has not been recognized for extra-axial tumor surgery in approximation of the SMA. METHODS: We observed the SMA syndrome in a patient operated for a parasagittal meningioma in the posterior frontal region, and this observation intrigued us to prospectively collect similar cases. RESULTS: In the period from January 2010 to December 2015, we observed five patients who developed a partial SMA syndrome after surgery for frontal parasagittal meningiomas. The muscle tone was preserved in the affected extremities. All patients experienced improvement in motor function within a few days, and on follow-up, three out of five patients had recovered completely. Three of the patients had meningioma WHO grade II. CONCLUSIONS: Surgically induced SMA syndrome can easily be confused with pyramidal weakness. This series of cases demonstrate that the syndrome may also develop after removal of extra-axial tumors and is probably underdiagnosed and underreported. The good functional prognosis is helpful in the preoperative counseling and follow-up of these patients.

Rare medical manifestations of severe restricting and purging: "Zebras," missed diagnoses, and best practices.

OBJECTIVE: Medical problems that arise due to severe restricting and/or purging may be misdiagnosed or suboptimally treated, from outpatient clinics to top medical hospitals. A symptom may be presumed to be a psychological manifestation of the eating disorder and inappropriately dismissed for further medical evaluation. Alternatively, a detailed medical workup may be performed, overlooking a classic relationship between starvation and a physical finding, which delays referral to eating disorder care. This review article focuses on rare medical issues (also called "zebras" in medical training), diagnoses that may be missed in patients with eating disorders, and best practices for management, organized by organ system. METHOD: A PubMed search was performed, using search terms "eating disorder," "anorexia nervosa," and "bulimia nervosa" in combination with different words for each organ system and known medical manifestations of severe eating disorders, with high quality and relevant studies from the past 20 years cited. DISCUSSION: Adults with eating disorders may present with extreme organ dysfunction and atypical signs and symptoms of typical medical problems. Timely diagnosis, risk awareness, appropriate treatment, and avoidance of harm are all vital. With judicious management and nutritional rehabilitation, most of these complications will significantly improve or resolve. ©

Minimally invasive surgery for superior mesenteric artery syndrome: a case report and literature review.

INTRODUCTION: Superior mesenteric artery syndrome is a rare condition characterised by nausea, vomiting, postprandial pain, anorexia and early satiety. Conservative management is tried initially, but if this fails, surgery is indicated. There are few reports in the literature concerning superior mesenteric artery syndrome in children, and fewer still managed surgically by minimally invasive means. CASE PRESENTATION: A 12-year-old girl presented with weight loss, early satiety and vomiting after corrective scoliosis surgery. After upper gastrointestinal endoscopy, contrast study and computed tomography imaging, a diagnosis of superior mesenteric artery syndrome was made. Conservative management by nasojejunal feeding failed; therefore, a laparoscopic duodeno-jejunostomy was undertaken. At follow-up, her symptoms had improved. CONCLUSION: This report describes the youngest child to undergo laparoscopic duodeno-jejunostomy for superior mesenteric artery syndrome. Laparoscopic duodeno-jejunostomy appears to be the most widely employed and reliable minimally invasive approach to superior mesenteric artery syndrome with a high success rate and acceptably low complication rate.

Severe gastric dilatation due to superior mesenteric artery syndrome in anorexia nervosa.

Forty-seven year old female, with a history of anorexia nervosa, was admitted to a medical stabilization unit (ACUTE) complaining of abdominal pain exacerbated by oral intake, associated with nausea, and relieved by emesis. Admission body mass index was 10.6. Labs were notable for hepatitis and hypoglycemia. On her progressive oral refeeding plan, she suddenly developed severe abdominal pain. Computed tomography (CT) revealed gastric dilatation and superior mesenteric artery (SMA) syndrome. SMA syndrome is a rare complication of severe malnutrition resulting from compression of the duodenum between the aorta and the SMA. It is diagnosed by an upper gastrointestinal series or an abdominal CT. Gastric dilatation, in turn, is a rare complication of SMA syndrome to be included in the differential diagnoses of abdominal pain in severely malnourished patients as it is potentially life-threatening. The patient was switched to an oral liquid diet, began weight restoring, and had resolution of symptoms.

Superior mesenteric artery syndrome and its associated gastrointestinal implications.

The superior mesenteric artery (SMA) syndrome is a rare but potentially life-threatening gastrointestinal condition. Over the years, it has been referenced by several names, the most common of which is Wilkie's syndrome. These numerous terminologies have made it difficult to estimate its true frequency in the general population. Common symptoms associated with this syndrome include intermittent postprandial abdominal pain, nausea, and bilious vomiting. Our review revealed that although it is currently well-defined in the literature, the diagnosis of SMA syndrome remains challenging as other disorders can mimic its presentation. However, CT angiography is currently favored in the literature for diagnosis as it can not only show the narrowed aorto-mesenteric angle and distance, but also the extent of duodenal obstruction. In addition, we found no consensus on the preferred mode of therapy once SMA syndrome is diagnosed. The agreement among authors is that the treatment options should be based on severity of the disease, using conservative measures as the first line of therapy in mild SMA syndrome. Duodenojejunostomy is the preferred surgical approach when conservative management fails, or in severe cases.

Superior mesenteric artery syndrome managed laparoscopically: a case report.

BACKGROUND: Superior mesenteric artery syndrome is a rare condition that has only around 400 reported cases so far. Typically, the superior mesenteric artery branches off the abdominal aorta at 45° to create an aortomesenteric distance of 10-28 mm, with the duodenum passing through. However, if this aortomesenteric angle reduces to less than 25°, the third portion of the duodenum becomes compressed between the SMA and aorta, causing mechanical obstruction. CASE PRESENTATION: This case report aims to demonstrate the diagnostic difficulties and the laparoscopic management of a 52-year-old Indian male presenting with abdominal pain and vomiting, with associated weight loss. Imaging was further suggestive of high intestinal obstruction, and he was later found to have superior mesenteric artery syndrome. CONCLUSION: Taking into account a significant reduction in morbidity, we propose laparoscopic duodenojejunostomy to be the new procedure of choice for superior mesenteric artery syndrome.

Duodenojejunostomy following failed gastrojejunostomy in superior mesenteric artery syndrome: A case report.

INTRODUCTION: Superior mesenteric artery (SMA) syndrome is a rare duodenal-vascular anatomic disorder leading to external compression on the duodenum. The first step of treatment usually is conservative, and in the case of failure, surgical management is the treatment choice. Treatment success with duodenojejunostomy after failure in gastrojejunostomy can show the uniqueness of this article. CASE PRESENTATION: A 14-year-old boy came to our hospital with a complaint of epigastric pain, nausea, bilious vomiting, and weight loss since 6 months ago. Conservation therapy and laparotomic Braun anastomosis and gastrojejunostomy was performed due to the SMA syndrome diagnosis 2.5 months before the admission. At our hospital, an alteration of gastrojejunostomy by duodenojejunostomy employing a diamond-shaped anastomosis between the third portion of the duodenum (D3) and a part of jejunum that was placed 15 cm away from the ligament Treitz was done. A significantly dilated stomach and the first three parts of the duodenum were observed during the procedure. After the second surgical intervention, the general condition of the patient dramatically improved. CLINICAL DISCUSSION: Conservative treatment, including nasogastric tube decompression, postural changes, and nutritional support with hyperalimentation, has a variable success rate. However, in some cases, surgery may be necessary. Surgeons prefer laparoscopic duodenojejunostomy due to its outstanding success rate, ranging from 80 % to 100 %. But, in some case reports it is suggested that gastrojejunostomy could be done in cases with severe duodenal dilation instead of duodenojejunostomy. The initial gastrojejunostomy failed because of ongoing symptoms, which was finally revised with a duodenojejunostomy. CONCLUSION: It is suggested to use duodenojejunostomy after failure of gastrojejunostomy or it can be employed as the first surgical option even in cases with severe dilation. Because it is a more efficient correction with fewer complications than gastrojejunostomy.

Presentation, surgical outcome, and supplementary motor area syndrome risk of posterior superior frontal gyrus tumors.

OBJECTIVE: Following resection of posterior superior frontal gyrus (PSFG) tumors, patients can experience supplementary motor area (SMA) syndrome consisting of contralateral hemiapraxia and/or speech apraxia. Given the heterogeneity of PSFG tumors, the authors sought to determine the risk of postoperative deficits and assess predictors of outcomes for all intraparenchymal PSFG tumors undergoing surgery (biopsy or resection), regardless of histology. METHODS: This was a retrospective single-center cohort study of adult PSFG-region tumors undergoing biopsy or resection by a single surgeon. RESULTS: A total of 106 consecutive patients undergoing 123 procedures (21 biopsies, 102 resections) fulfilled inclusion and exclusion criteria. Anaplastic astrocytomas were the most frequent among resected tumors (39% vs 29%), while glioblastomas were most common among biopsies (38% vs 27%) (p < 0.0001). The biopsy cohort was more likely to have tumor involvement outside the PSFG (90% vs 62%) (p = 0.011), most commonly in the motor cortex (67% vs 31%) (p = 0.005). Seizures were the most common presenting symptom in the resection cohort (p = 0.017), while motor deficits were more common in the biopsy cohort (58% vs 29%) (p < 0.001). Immediate postoperative neurological deficits occurred in 71 cases (58%), but only 3 of the deficits were permanent at 6 months of follow-up (2%). Postoperative SMA syndrome occurred in 48 cases (47%) and was significantly associated with involvement of the motor cortex (p = 0.018) or cingulate gyrus (p = 0.023), which were also significant in multivariate analysis as risk factors for SMA syndrome. However, postoperative SMA syndrome was not significantly associated with overall survival (p = 0.51). There were no perioperative deaths, but corpus callosum involvement (p < 0.001), contrast enhancement (p = 0.003), and glioblastoma pathology (p = 0.038) predicted worse overall survival in patients undergoing resection. CONCLUSIONS: Nearly half of all patients undergoing resection of PSFG-region tumors experience a postoperative SMA syndrome. Individuals with corpus callosum and/or motor cortex involvement may be at an increased risk of experiencing SMA syndrome. However, these deficits are usually transient, and the risk of permanent new deficits is very low (3%). Preoperative characteristics including corpus callosum involvement and tumor enhancement-in addition to pathology-might serve as predictors of overall survival within this patient population.

COVID-19 infection with severe hypocalcaemia and superior mesenteric artery syndrome-a case report.

Background: Coronavirus disease 2019 (COVID-19) infection has been associated with various endocrinopathies. Few literatures have reported cases of transient hypoparathyroidism in acute COVID-19 infections, or even exacerbation of hypocalcaemia in previously known hypoparathyroidism patients. The mechanism of hypocalcaemia in COVID-19 infection remains unclear. Case Description: Our patient is a young gentleman who was incidentally diagnosed with superior mesenteric artery (SMA) syndrome and symptomatic primary hypoparathyroidism while presenting with an acute COVID-19 infection. He initially presented with high-grade fever, followed by multiple episodes of vomiting and abdominal pain and subsequently hypocalcaemic symptoms such as tonic-clonic seizures and carpopedal spasms. A computed tomographic scan of his abdomen revealed a SMA syndrome while his blood investigation showed a parathyroid hormone (PTH)-dependent hypocalcaemia. His SMA syndrome was a result of severe malnourishment and improved with refeeding, but his primary hypoparathyroidism persisted despite having recovered for 6 months from the initial COVID-19 infection. There was no evidence to suggest a congenital cause of hypoparathyroidism. Conclusions: To the best of our knowledge this is the first case report that describe this unique case of persistent primary hypoparathyroidism related to COVID-19 infection. Parathyroid gland involvement in a COVID-19 infection is rare but not impossible. Further studies are needed to determine the mechanism and extent of damage of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) to the parathyroid glands.

Understanding Superior Mesenteric Artery Syndrome: Etiology, Symptoms, Diagnosis, and Management.

Superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, poses a diagnostic challenge due to its rarity and varied clinical manifestations. This review explores the syndrome's etiology, symptoms, diagnostic challenges, and management strategies. Symptoms range from early satiety to severe abdominal pain, often leading to malnutrition. Diagnosis involves a thorough gastrointestinal evaluation and various imaging modalities. Management includes medical interventions like nasogastric decompression and nutritional support, along with surgical interventions such as duodenojejunostomy. A thorough understanding of SMA syndrome's complexities is crucial for its timely diagnosis and effective management, especially considering its potential overlap with other gastrointestinal disorders or eating disorders. Further research is needed to enhance understanding and improve patient outcomes.

Superior Mesenteric Artery Syndrome: A Report of a Rare Case.

Superior mesenteric artery (SMA) syndrome is a rare disease in which the third part of the duodenum between the SMA and the abdominal aorta is compressed, leading to small bowel obstruction. Treatment is usually conservative, such as parenteral and nasojejunal nutrition. The pathophysiology includes loss of the retroperitoneal fat layer and subsequent duodenal compression. We present a 53-year-old malnourished female patient who came with complaints of vomiting, constipation, abdominal pain, and distension for four days. This article highlights the diagnostic challenges associated with SMA syndrome and emphasizes the importance of early diagnosis and intervention.

Superior mesenteric artery syndrome treatment strategies: A case report.

Superior mesenteric artery syndrome is a rare manifestation of small bowel obstruction caused by the compressed third part of the duodenum between the superior mesenteric artery and the abdominal aorta. We present the case of an 18-year-old female and presented with symptoms of duodenal outflow obstruction. Upon investigation, her cross-sectional imaging revealed partial obstruction of the distal duodenum at the crossing of superior mesenteric artery forming an acute angle between the superior mesenteric artery and aorta. Failing initial attempts at conservative management, the patient underwent laparotomy and duodenojejunostomy with complete relief of symptoms. Superior mesenteric artery syndrome is a rare but a sinister diagnosis in patients presenting with features of duodenal outflow obstruction. Cross-sectional imaging plays an important role in diagnosis.