A case of Guillain-Barré Syndrome complicated with Fat Embolism syndrome

A 22-year-old man was admitted to our ICU for treatment of fat embolism syndrome complicated with multiple bone fractures. Acute respiratory distress syndrome developed, and the patient received steroid therapy. During the steroid therapy, symmetrical proximal muscle weakness of all four limbs without sensory abnormalities and autonomic nervous system dysfunction developed. Cerebrospinal fluid showed albuminocytologic dissociation. Blood examination showed herpes simplex virus and cytomegalovirus infection. The patient was diagnosed as having Guillain-Barré syndrome, but he recovered completely one month after admission. It is thought that the prolonged steroid therapy for acute respiratory distress syndrome in this case induced viral infection that might have caused Guillain-Barré syndrome.     

Risk of fracture in patients with Guillain-Barré syndrome

Summary

The aim of this study was to evaluate fracture risk in patients with Guillain-Barré syndrome (GBS). No association with risk of fracture was observed for GBS patients compared with controls. Only GBS patients using pain treatment had a doubled risk of fracture.

Introduction

Symptoms of Guillain-Barré syndrome (GBS) may vary from mild difficulty in walking to complete paralysis, which may increase the risk of fractures. Therefore, the aim of this study was to evaluate fracture risk in patients with GBS.

Methods

We conducted a retrospective cohort study using the UK Clinical Practice Research Datalink (1987–2012). Each patient with GBS was matched by year of birth, sex, and practice, up to six patients without a history of GBS. Outcome measure was any fracture.

Results

There were no associations between GBS and any fracture, adjusted hazard ratio (AHR) 1.01 (95 % confidence interval [CI] 0.77–1.33), or osteoporotic fracture, AHR 0.76 (95 % CI 0.50–1.17), compared with controls. Stratification to gender, age, and duration since diagnosis did not show an association either. Only for GBS patients using pain treatment, risk of fracture was doubled AHR 1.97 (95 % confidence CI 1.21–3.21) compared with controls. The risk of fracture in GBS patients exposed to pain treatment was equivalent to risk of fracture among controls exposed to pain treatment.

Conclusions

No association with risk of fracture was observed for GBS patients compared with controls. Only GBS patients using pain treatment had a doubled risk of fracture, but their risk was equivalent to fracture risk among controls exposed to pain treatment.     

Thoracic outlet syndrome in a patient with SAPHO syndrome – A case report

Introduction and ImportanceThoracic outlet syndrome (TOS) includes disorders caused by compression of the neurovascular structures in the upper thoracic outlet (Roos and Owens, 1996 [1]; Bürger, 2014; Curuk, 2020 [3]). Depending on the compressed structure, it is categorized into neurological, arterial and venous TOS.SAPHO syndrome (synovitis–acne–pustulosis–hyperostosis–osteitis syndrome) is a rare chronic inflammatory disease of unknown etiology. With its typical involvement of sternoclavicular joint and clavicle, complication due to hyperostosis in this region, leading to thrombosis of the subclavian vein have been reported in some cases of SAPHO syndrome.Between 2015 and 2019 488 patients, suffering from neurological, vascular or combined TOS presented at our department. Depending on clinical and diagnostic results surgical therapy was performed in 175 cases via the transaxillary approach, including complete first rib and/or cervical rib resection, neurolysis of plexus brachialis, thoracic sympathectomy and vascular reconstruction if indicated (Curuk, 2020). During this period, only one single patient presented with SAPHO syndrome with thrombosis of the subclavian vein and neurovascular TOS.Case presentationWe present a 50-year-old female patient, in line with the SCARE 2020 criteria (Agha et al., 2020 [12]) suffering from extremely rare combination of neurovascular TOS and SAPHO syndrome with thrombosis of the left subclavian vein due to hyperostosis of the left clavicle.ConclusionProgressive bone changes associated with SAPHO syndrome can lead to narrowing of the thoracic outlet. Pharmacological therapies to avoid the progression of the hyperostosis of the costoclavicular joint and the clavicle do currently not exist. First rib resection is a therapeutic option to widen the space in the upper thoracic region. Surely, it is a rare condition and more long-term follow-up data are required.     

Euthyroid sick syndrome in trauma patients with severe inflammatory response syndrome

Objective： To investigate the alternations of thyroid hormone in traumatic patients with severe inflammatory response syndrome （SIRS）. Methods： Fifty traumatic patients with severe SIRS were enrolled and divided into two groups according to whether they presented multiorgan dysfunction syndrome （MODS）. Thyroid hormone measurements were taken, including total triiodothyronine （ TT3 ）, total thyroxine （TT4）, free triiodothyronine （FT3）, free thyroxine （ FT4 ） and thyroid stimulating hormone （TSH）. The acute physiology and chronic health evaluation II （ APACHE II ） score was calculated according to clinical data. The outcomes of recovery or deterioration were recorded, as well as the length of time from the onset of SIRS to the time thyroid hormones were measured. Results： Euthyroid sick syndrome （ESS） was presented in 45 cases. TT3 level was negatively correlated with APACHE II score （r = -0.330, P 〈0. 05）, and TT3/TI＇4 value was negatively correlated with the duration of SIRS（ r = -0.316, P〈0.05）. TT3, TT4 and levels in MODS patients were significantly lower than those without MODS （ P 〈 0.05 ）. MODS patients got low TT4 or FT4 level more frequently than those without MODS （ P 〈 0.05 ）. Compared with the patients in normal TSH group, the patients with decreased TSH had lower T3, T4, recovery rate and higher APACHE II scores, MODS incidence, but there was no difference between two groups （P〉0.05）. Conclusions： Trauma patients with severe SIRS have high possibility to get ESS, which occurs more frequently and severely in MODS patients. It shows the influences of SIRS on the thyroid axes. With the persistence and aggravation of SIRS, there is a progressive reduction of thyroid hormone.     

Anesthetic management of Guillain-Barré syndrome in pregnancy

We report the case of a 23-year-old woman who was diagnosed with an axonal type of Guillain-Barré syndrome at 16 weeks' gestation. The patient had severe motor loss but she was treated effectively with intravenous immunoglobulin, and she underwent cesarean delivery with epidural anesthesia at full term.     

A case report of Turner syndrome associated with Rathke cleft cyst

It has not been reported that Turner syndromecould be associated with Rathke cleft cyst yet,nowwe report such a case found in our hospital.Clinical data:The patient,social sex female,15.3ys,was a junior high school student.Chief com-plaints:She had short stature for 10~ years and no pu-berche.She was a first full term fetus with cephalicpresentation.The delivery was accomplished by episi-otomy and vacuum extracter with a history of neonatal     

Congenital diplopodia–A rare case of duplicated lower limb: A case report

Introduction and importanceDiplopodia is an extremely rare case in medical history, with an even fewer cases being reported in literature. We intended to enrich the literature about diplopodia with our own case report.Case presentationWe present a case about A boy, aged one year and four months old brought by his mother to the hospital with a chief complaint of a duplicated foot in his right lower leg. Physical examination demonstrated a normal left lower extremity and a relatively well-developed duplicate foot emanating from the posterior-lateral aspect of the mid-lower right leg.Clinical discussionDiplopodia consists of partial duplication of the foot, with or without hypoplasia or positional abnormality of the ipsilateral tibia and fibula. It must be differentiated from polydactyly where the additional structures consist of toes that may or may not have corresponding metatarsals but are devoid of tarsal bone.Treatment should be considered case-by-case basis and tailored appropriately to suit individual needs and circumstances.ConclusionIn our case, operative treatment was done at an early walking age to provide plantigrade, functional foot. Timely surgical intervention will enable patient to adapt over time. The secondary aim is to reconstruct the foot to be more acceptable aesthetically.     

A case of clear cell adenocarcinoma of the müllerian duct in persistent müllerian duct syndrome: the first reported case

We report a case of a 67-year-old man with clear cell adenocarcinoma of the remnant uterus in persistent Müllerian duct syndrome. He had a normal penis, urethra, and scrotum, and there was also a vagina and uterus. He died in a traffic accident, and clear cell adenocarcinoma was discovered incidentally at autopsy. Clear cell adenocarcinoma of the remnant uterus metastasized to the retroperitoneal lymph nodes and bilateral lungs. Persistent Müllerian duct syndrome is characterized by the persistence of Müllerian derivatives in otherwise normally virilized males. A variety of germ cell tumors of the testis have been reported in association with persistent Müllerian duct syndrome. However, no malignant change of the persistent Müllerian duct structures has been reported. This represents the first reported case of malignant change of the persistent Müllerian duct structures in persistent Müllerian duct syndrome.     

Deletion of the mitochondrial DNA in a case of de Toni-Debré-Fanconi syndrome and Pearson syndrome

We report a patient with Pearson syndrome with failure to thrive, exocrine pancreas insufficiency, growth hormone deficiency and severe tubular dysfunction. The patient had no signs of liver involvement. Normal respiratory chain enzyme activity was found in the lymphocytes, but a mitochondrial DNA deletion was demonstrated in lymphocytes and in the kidney. Polymerase chain reaction amplification and sequence analysis revealed the presence of the 4,977 base pair common deletion in the mitochondrial genome. Our findings support the view that tubulopathies of unknown origin may be related to mitochondrial respiratory chain deficiency.     

Could motor imagery be effective in upper limb rehabilitation of individuals with spinal cord injury? A case study

Study design:A case study.Objective:The aim was to investigate whether motor imagery (MI) could be successfully incorporated into conventional therapy among individuals with spinal cord injury (SCI) to improve upper limb (UL) function.Setting:The Physical Medicine and Rehabilitation Unit at the Henry Gabrielle Hospital in Lyon, France.Methods:The participant was an individual with a complete C6 SCI. MI content was focused on functional UL movements, to improve hand transport to reach out and grasp with tenodesis. The participant was tested before and after 15 MI training sessions (45?min each, three times a week during 5 consecutive weeks). MI ability and program compliance were used as indicators of feasibility. The Minnesota and Box and Blocks tests, as well as movement time and hand trajectory during targeted movements were the dependent variables, evaluating motor performance before and after MI training.Results:The participant's ability to generate MI was checked and compliance with the rehabilitation program was confirmed. The time needed to complete the Minnesota test decreased by 1?min 25?s. The Box and Blocks score was improved by three units after MI program. Decreased movement time and enhanced hand trajectory smoothness were still observed 3 months later, despite a slight decrease in performance.Conclusions:This study supports the feasibility for introducing MI in conventional therapy. Further studies should confirm the potential role of MI in motor recovery with a larger sample.     

Rare case of obturator hernia in a patient with Marfan’s syndrome

Obturator hernia is a very rare type of abdominal hernia which constitutes <1 % of all the hernias. It is an important cause of small bowel obstruction which is associated with a high mortality rate if left untreated. Obturator hernia typically occurs in an elderly women or patients with chronically raised intraabdominal pressure or previous multiple pregnancies. We report a case of obstructed obturator hernia in a young female patient with Marfan’s syndrome complicated with dissecting aortic aneurysm and chronic kidney disease. Though recurrent and incisional hernia constitutes one of the minor diagnostic criteria of Marfan’s syndrome, obturator hernia being a very rare entity, has been reported very rarely in a patient with Marfan’s syndrome.     

Does subintimal angioplasty have a role in the treatment of severe lower extremity ischemia?

OBJECTIVE: Subintimal angioplasty (SIA) has been advocated to treat long segment lower extremity arterial occlusions, but many question its value. We evaluated the role of SIA in a group of patients with severe lower extremity arterial occlusive disease. METHODS: During a 2.5-year period, 39 patients with arterial occlusions (median length, 8 cm; range, 2 to 31 cm) were treated on an intention-to-treat basis with SIA. Twenty-five patients had gangrene, five had rest pain, and nine had disabling (相似文献