Delayed diagnosis of parkinsonism hyperpyrexia syndrome resulting in death: a case report

Compartment syndrome has been defined as “a condition in which increased pressure within a limited space compromises the circulation and function of the tissues within that space”.1 It is most commonly seen after injuries to the leg2-5 and forearm6-8 but may also occur in the arm,9 thigh,10 foot,11-13 buttock,14 hand15 and abdomen.16 It typically follows traumatic injury, but may also occur after ischaemic reperfusion injuries,17 burns,18 prolonged limb compression after drug overdose19 or poor positioning during surgery.20-24 Furthermore, subclinical compartment syndromes may explain the occurrence of a variety of postoperative disabilities which have been identified after the treatment of fractures of long bones using intramedullary nails.25 Approximately 40% of all acute compartment syndromes occur after fractures of the tibial shaft26 with an incidence in the range of 1% to 10%.26-30 A further 23% of compartment syndromes are caused by soft-tissue injuries with no fracture and fractures of the forearm account for 18%.26 Acute compartment syndrome is seen more commonly in younger patients, under 35 years of age31 and therefore leads to loss of function and long-term productivity in patients who would otherwise contribute to the country’s workforce for up to 40 years.

Woman with Neck Swelling

The single case report: a valuable contribution to the scientific literature.

Stiff Man Syndrome With Thymoma

BackgroundSystemic sclerosis (SSc)-systemic lupus erythematosus (SLE) overlap syndrome is rarely described in the literature, and its morbidity and mortality remain high after the early onset of pulmonary arterial hypertension (PAH), which determines its severity. The epidemiology of SSc-SLE overlap syndrome is not well known. It is characterized by high clinical polymorphism, making its diagnosis difficult. Through this case, we underline the difficulty and delay in the diagnosis of this syndrome in a country with limited resources, as well as the difficulty of its management given the specificity of the treatment for each pathology and the risk of infections, which could limit their use.Case presentationWe report the case of a 49-year-old North African female patient, initially followed for SSc for 8 years, whose diversity of symptoms masked the distinct disease. Indeed, her initial clinical presentation was in favor of SSc, but the discovery of a pericardial effusion stimulated us to carry out more investigations and correct the initial diagnosis. The involvement of the myocardium and pericardium, as well as the positive antibody serology tests, make it possible to retain the diagnosis of SSc-SLE overlap syndrome. Despite the introduction of treatment, the patient unfortunately died a month later after developing a multi organ failure following an opportunistic infection.ConclusionsThe management of SSc-SLE overlap syndrome can be complex, requiring good knowledge of these two pathologies, especially in immunocompromised patients with complications. Treatments based on corticosteroids and immunosuppressants may differ from one case to another, making their use difficult in a patient developing a cytomegalovirus (CMV) infection. These patients require urgent treatment before the onset of complications, at the risk of worsening the prognosis, with close collaboration between a cardiologist and an internist, given the multisystem involvement.

Diagnostic Aid of Transesophageal Echocardiography in an Adult Case of Scimitar Syndrome: Confirmation of the Findings at Surgery and Review of the Literature

Girls usually experience only mild pain during their first menstrual cycle, and severe pain at menarche is occasionally indicative of a uterine anomaly. Case report: An 11-year-old girl, who had been diagnosed with a left renal defect and ureterocele 2 years previously, presented to our emergency room with severe dysmenorrhea 1 day after her second menstrual cycle began. Imaging revealed the presence of a double uterus, left cervical cyst, and ipsilateral renal defect. Thus, she was diagnosed with Wunderlich syndrome. Although cyst drainage was attempted, the wall was too rigid. In addition, uterus bicornis unicollis was noted during laparoscopy. Finally, an intraoperative diagnosis of Herlyn-Werner syndrome was made during laparoscopic left hysterectomy. Discussion: Herlyn-Werner syndrome, obstructed hemivagina and ipsilateral renal anomaly syndrome, and Wunderlich syndrome are rare Mullerian anomalies that have many common features.Conclusion: Diagnosis of these syndromes in girls is difficult, and the treatment differs in each case. Hence, flexibility in treatment is essential.

PAROXETINE OVERDOSE LEADING TO SEROTONIN SYNDROME

Behcet's Disease with Renal Amyloidosis: Report of a Case from Jordan with Review of the Literature

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe adverse drug reaction presenting with rash, fever, lymphadenopathy, and single or multiple organ involvement. It is most commonly associated with antiepileptics, NSAIDs, and sulfa drugs. We report a 40-year-old man who presented with a 1-week history of fever, sore throat, and a diffuse pruritic macular rash that started on the face and trunk before spreading to all extremities 4 weeks after the use of naproxen. He had lymphadenopathy, hepatosplenomegaly, transaminitis, and peripheral eosinophilia. A Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) score of 8 gave a diagnosis of definite DRESS syndrome. Significant resolution of symptoms and laboratory abnormalities were seen after 2 weeks of corticosteroid therapy. DRESS syndrome is a life-threatening condition, and the clinical status of patients can worsen rapidly. Given the high variability in clinical presentation, the diagnosis of DRESS syndrome requires a high degree of suspicion and clinical judgment. Case reports on this entity will equip physicians in acute medicine to recognize and treat the condition early. This report reinforces the importance of using the RegiSCAR score in the diagnosis of DRESS syndrome.

Background: The parkinsonism hyperpyrexia syndrome (PHS) is a rare but potentially fatal disorder. We reported an advanced Parkinson’s disease (PD) patient who was initially diagnosed with fever of unknown origin and finally with PHS, together with a review of the literature. Case Report: A 67-year-old woman with PD presented with high-grade fever accompanied by altered consciousness and rigidity. Prior to the symptom onset, she had a couple week’s history of poor feeding due to her dental problem. However, she had regularly taken her anti-Parkinsonian medication even during that period, which hampered early recognition of PHS at the emergency room. Her anti-Parkinsonian medication was withheld for more than 30 hours during her stay in our emergency room and extensive diagnostic work-up to identify the fever focus. Her neurologic symptoms deteriorated with concurrent acute hepatic failure and disseminated intravascular coagulopathy. Levodopa treatment was resumed on correction of underlying medical conditions. The patient recovered dramatically with normalization of laboratory tests. Conclusion: This case indicated that PHS could occur in patients without prior history of withdrawal of antiparkinsonian medication, possibly due to poor absorption or poor feeding conditions. Key Words: Parkinson disease; Malignant hyperpyrexia; Neuroleptics

To investigate the usefulness of magnetocardiography (MCG) in the prenatal diagnosis of fetal long QT syndrome. Fetal MCG was recorded in a case of fetal long QT syndrome suspected in utero. The literature on the prenatal diagnosis of fetal long QT syndrome was also reviewed. The MCG was performed at 36 weeks' gestation because sustained fetal bradycardia of 110-120 bpm was detected by cardiotocography. The 64-channel MCG revealed a prolonged fetal corrected QT-interval of 0.57 s. The postnatal electrocardiogram coincided with prenatal MCG. An accumulation of cases of prenatally diagnosed long QT syndrome using MCG indicates that MCG may be the most reliable tool for the prenatal diagnosis of long QT syndrome.

Superior mesenteric artery syndrome is a rare pathological cause of gastric outlet obstruction. Traditionally, computer tomography and magnetic resonance imaging scans have been diagnostic investigations of choice. However, due to vague symptoms of gastric outlet obstruction, there is a delay to diagnosis of superior mesenteric artery syndrome in acute setting. This case describes the use of point-of-care ultrasound scan for diagnosis of superior mesenteric artery syndrome in the emergency department. We present a case of a 25-year-old woman presenting to the emergency department with signs and symptoms of gastric outlet obstruction caused by superior mesenteric artery syndrome. Point-of-care ultrasound scan demonstrated a fluid-filled stomach with continuous bubbling of air (effervescence sign) and a loop of duodenum compressed between the aorta and the superior mesenteric artery, suggesting a diagnosis of superior mesenteric artery syndrome. Subsequent computed tomography scan confirmed the point-of-care ultrasound scan findings. Ultrasound has been described as a reliable diagnostic modality to diagnose the radiological signs of superior mesenteric artery syndrome. Our newly identified sign of continuous bubbling of air (effervescence sign) on point-of-care ultrasound scan, due to fermentation of stomach contents, and its ability to identify diagnostic features of superior mesenteric artery syndrome demonstrate its utility as a diagnostic tool in the emergency department reducing time delays and costs associated with traditional diagnostic modalities. Point-of-care ultrasound scan can aid in the diagnosis of superior mesenteric artery syndrome in the emergency department. Increased awareness of the capabilities of using point-of-care ultrasound scan in the emergency department and our newly identified 'effervescence sign' will enable timely and low-cost diagnoses, while closing current literature gap and establishing point-of-care ultrasound scan as a feasible diagnostic tool for gastric outlet obstruction secondary to superior mesenteric artery syndrome.

Compartment syndrome is a serious complication of fractures of the tibial shaft and its clinical presentation and evaluation have been well documented (Holden 1975; Kikuchi, Hasue and Watanabe 1978). The most important diagnostic feature is the presence of inappropriate pain even after stabilisation of the fracture by a cast or by external or internal fixation. Pain is made worse by passive stretching of the muscles involved and there is an associated sensory disturbance. The prognosis is directly related to the speed at which the diagnosis is made and treatment begun. It has been shown that muscle can tolerate only four hours of ischaemia without injury (Heppenstall et al 1988; Heckman et al 1993). We describe a patient with a fracture of the tibial shaft in whom the diagnosis of compartment syndrome was delayed because of the use of a local nerve block given in the operating theatre after intramedullary nailing. Case report. A 28-year-old man presented after a football injury with a closed fracture of the tibial shaft (42-A2.2 according to the AO classification (Muller et al 1990)). Before operation there were no signs of neurovascular compromise or of a compartment syndrome. The fracture was initially stabilised in a plaster back slab and closed, unreamed locked intramedullary nailing then performed. At the end of the operation, the patient was given a triple nerve block (femoral, obturator, lateral cutaneous nerve of thigh) using bupivacaine 0.5% for pain control. After operation, he was entirely free from pain, although he did complain of areas of altered sensation in his foot and leg, but these varied in site and were initially thought to be a result of the nerve block rather than a symptom of a developing compartment syndrome. Pressure measurements were made 48 hours after operation because symptoms persisted and there was inability actively to extend the big toe. They showed a pressure of 108 mmHg in the anterior compartment. We performed fasciotomy urgently but all the muscles in the anterior compartment were dead. No other compartment was involved. The whole of the anterior tibial compartment was debrided 48 hours later as there was no sign of recovery. The patient now walks with an orthosis. Discussion. The treatment of postoperative pain in patients whose tibial fractures have been stabilised is usually by elevation of the injured leg and the use of oral or intramuscular analgesics, especially non-steroidal anti-inflammatory agents. Local anaesthetic nerve blocks are now also commonly given to patients with fractures of the lower limb; they are effective and especially useful after fracture of the femoral shaft. A nerve block using bupivacaine persists for about eight hours (Macrae and Davies 1993), but recovery from the block may be patchy and erratic over a period of several hours. The diagnosis of compartment syndrome is essentially

Case report - IntroductionAntiphospholipid Syndrome (APS) is an autoimmune condition manifested by vascular thrombosis with or without pregnancy related morbidities. APS classification criteria require detection of persistent antibodies: Anti-Cardiolipin Antibody (aCL), Anti-Beta Glycoprotein I (ab2GPI) and circulating Lupus Anticoagulant (LAC).Criteria for the diagnosis of obstetric antiphospholipid syndrome allow for optimal treatment planning in pregnancy and reduction of foetal and maternal morbidity. There are, however, several “non-criteria” features of APS, including late pre-eclampsia, which may also infer adverse obstetric outcomes.We highlight a treatment dilemma in a young female with lupus and APS with history of previous pregnancy that was complicated by pre-eclampsia.Case report - Case descriptionWe describe a case of a 26-year-old female with systemic lupus erythematosus (SLE), “triple positive” APS markers (aPL) and non-criteria APS obstetric morbidity.During her first pregnancy she presented at 22 weeks with severe and frequent migraine episodes. She had no other active clinical manifestations of SLE. There was no prior history of pregnancy and therefore no previous obstetric morbidity nor had she had any vascular thrombotic episodes. She had been commenced on low-dose aspirin (LDA) at the start of her pregnancy and was already taking hydroxychloroquine (HCQ). The dose of HCQ was increased to 400mg daily leading to improvement in the headaches. She had persistent strong ACL antibodies, positive LAC and ab2GPI antibodies. ANA was positive with hypocomplementemia and neutropenia but normal range double-stranded DNA antibodies.After discussion with a tertiary centre, it was recommended to continue HCQ and LDA. Low molecular weight heparin (LMWH) was not proposed as she did not fulfil criteria for obstetric APS. Her pregnancy was subsequently complicated by pre-eclampsia and post-partum sepsis.Two years later she sought advice to plan her second pregnancy, raising important questions as to whether the same treatment strategy should be followed or if alternative approaches should be considered, given the history of pre-eclampsia and its association with APS. Again, she did not meet the international consensus (revised Sapporo) criteria for diagnosis of obstetric APS.After further discussion with the tertiary centre, she commenced high-dose aspirin 150mg/day alone. Uterine artery dopplers were recommended and LMWH proposed if bilateral notching was noted. Dopplers were normal.Her blood pressure (BP) fluctuates between 130-145mmHg systolic. She has occasional crops of mouth ulcers but no other SLE features. Migraines continue every 2—3 weeks although severe attacks are infrequent. C4 is persistently low with normal Ds DNA and urine protein/creatinine ratio.Case report - DiscussionOur case raises questions on how pregnancy should be safely managed in patients without a prior history of APS-related pregnancy complications but who nevertheless have risk factors, in the form of APS antibody positivity and non-criteria obstetric manifestations of APS. Her previous pregnancy was treated with LDA and HCQ which had proved insufficient in avoiding the outcome of pre-eclampsia.The objective of prophylactic treatments is to prevent adverse outcomes such as miscarriage, which carry significant maternal morbidity including psychological manifestations such as depression and anxiety. In first pregnancies, mothers have no existing obstetric history, such that current treatment guidance provides few recommendations for preventative interventions, even where other risk factors are apparent. Furthermore, non-criteria manifestations of obstetric APS are now well recognised, which may further highlight potential risk for obstetric morbidity but fail to trigger interventions as they are not included within the current international criteria. These manifestations include late pre-eclampsia, placental abruption and late pregnancy loss, unexplained in vitro fertilisation failures, two unexplained miscarriages and three non-consecutive miscarriages. Low positive antibody titres and intermittent aPL are also included.Studies suggest that women with non-criteria APS may benefit from standard treatment for obstetric APS which includes LMWH in addition to aspirin, with good pregnancy outcomes.HCQ has a dose-dependent effect in preventing thrombosis through inhibition of platelet aggregation and aPL Ab-related thrombosis. However, pregnancy itself is a prothrombotic condition and in the presence of persistent triple positive aPL is a risk factor for adverse pregnancy outcomes.Further studies are needed to define the risk of non-criteria APS on pregnancy outcomes and define optimal treatment strategies.Case report - Key learning pointsObstetric APS carries significant risk for maternal foetal morbidity including miscarriage and pre-term birth.Significant maternal psychological effects are recognised even following a single miscarriage.The international consensus (revised Sapporo) criteria for diagnosis of obstetric antiphospholipid syndrome exclude certain non-criteria clinical and laboratory manifestations that may confer adverse pregnancy outcomes.First pregnancies are challenging to risk stratify in patients with identified aPL as there will be no prior pregnancy history to reference.Ultrasound doppler velocimetry is a useful non-invasive tool to diagnose poor placental perfusion, a predictor of obstetric complications, hence identifying high-risk pregnancies.Further research is required to establish the diagnostic validity, predictive value, and treatment implications of non-criteria manifestations of obstetric APS.