脑桥中央髓鞘溶解症(附9例分析)

目的 研究脑桥中央髓鞘溶解症(CPM)的临床特点和影像学及电生理改变。方法对1984～2003年该院诊治的9例CPM患者的临床特点及影像学、电生理表现进行回顾性分析。结果CPM的病因主要与慢性酒精中毒、营养不良、快速纠正低钠血症等有关，临床以假性球麻痹和四肢痉挛性瘫痪为典型表现．MRI扫描可见脑桥中央髓鞘脱失灶，诱发电位也可见阳性发现。结论CPM是一种相对罕见的脱髓鞘疾病，临床症状及体征是诊断CPM的基础，头部MRI是诊断该病的重要手段，诱发电位可发现早期损害。     

肝移植后脑桥中央髓鞘溶解症5例

目的总结原位肝移植术后脑桥中央髓鞘溶解症(CPM)的诊治体会。方法回顾性分析5例原位肝移植术后并发CPM的临床资料。结果4例为肝炎后肝硬化失代偿期患者,1例为重型肝炎患者。术前均有持续低血钠,原位肝移植术后48 h内血钠纠正至正常范围或高于正常。CPM症状出现时间在术后1～2周,主要临床表现有精神异常、不同程度的意识障碍、言语不清及肢体活动障碍,1例伴有抽搐。5例患者均经头颅MRI检查证实为脑桥中央髓鞘溶解症,其中2例合并脑桥外髓鞘溶解(EPM)。经积极对症支持治疗,5例患者无1例死亡,4例患者神经系统症状明显改善,1例患者恢复较差,随访5个月仍处于昏迷状态。结论肝移植后发生CPM并非罕见,它可能是多种因素共同作用的结果,头颅MRI是主要确诊依据,脑电图可协助判断预后,脑脊液无特异性改变。CPM虽预后较差,但并非不可逆。     

桥脑中央髓鞘溶解症MRI表现及其在诊断中的应用

目的:探讨5例桥脑中央髓鞘溶解症(CPM)患者的MRI表现,阐明MRI在诊断CPM方面的应用,以提高其诊断水平。方法:收集5例CPM患者的主要病史和临床表现。应用Philip Achieva 3.0T超导MR,采集从后颅窝底到颅顶的T1WI、T2WI、Fiair和弥散加权像(DWI)序列。结果:CPM的MRI表现为桥脑中央受累,皮质脊髓束的轴索无损害,无占位及水肿; T1WI呈低信号,T2WI及T2 Flair呈高、稍高信号,DWI呈高信号或等信号,增强后周边轻度强化或无强化;病灶呈"凸"字形,栗形或斑片状;少数累及基底节、皮层、小脑等脑桥外结构。结论:CPM的MRI表现较具特征性,MRI检查可为CPM的早期诊断提供帮助。     

大剂量人体丙种球蛋白冲击治疗脑桥中央髓鞘溶解症1例及文献复习

目的:探讨大剂量静滴人体丙种球蛋白(intravenous immunoglobulin,IVIG)治疗脑桥中央髓鞘溶解症(central pontine myelinolysis,CPM)的疗效。方法:利用IVIG冲击治疗1例CPM患者和文献复习并阐述其原理。结果:大剂量IVIG冲击治疗CPM具有一定效果。结论:大剂量IVIG冲击疗法可用于治疗CPM。     

Isoniazid-induced cerebellitis: a disguised presentation

Cerebellitis is a rarely encountered complication of isoniazid therapy. Its occurrence is usually associated with concomitant renal disease and haemodialysis. Herein, we report the case of a patient with this complication who presented with isolated bilateral symmetrical dentate nucleus T2 hyperintensities on magnetic resonance imaging. Isoniazid neurotoxicity has never been reported to cause bilateral dentate hyperintensities, for which the differentials are few and include metronidazole toxicity.     

Prevalence of dysglycaemic events among inpatients with diabetes mellitus: a Singaporean perspective

INTRODUCTION

As the effectiveness of intensive glycaemic control is unclear and recommended glycaemic targets are inconsistent, this study aimed to ascertain the prevalence of dysglycaemia among hospitalised patients with diabetes mellitus in an Asian population and evaluate the current standards of inpatient glycaemic control.

METHODS

A retrospective observational study was conducted at a secondary hospital. Point-of-care blood glucose (BG) values, demographic data, medical history, glycaemic therapy and clinical characteristics were recorded. Dysglycaemia prevalence was calculated as proportions of BG-monitored days with at least one reading exceeding the cut points of 8, 10 and 15 mmol/L for hyperglycaemia, and below the cut point of 4 mmol/L for hypoglycaemia.

RESULTS

Among the 288 patients recruited, hyperglycaemia was highly prevalent (90.3%, 81.3% and 47.6% for the respective cut points), while hypoglycaemia was the least prevalent (18.8%). Dysglycaemic patients were more likely than normoglycaemic patients to have poorer glycated haemoglobin (HbA1c) levels (8.4% ± 2.6% vs. 7.3% ± 1.9%; p = 0.002 for BG > 10 mmol/L) and longer lengths of stay (10.1 ± 8.2 days vs. 6.8 ± 4.7 days; p = 0.007 for BG < 4 mmol/L). Hyperglycaemia was more prevalent in patients on more intensive treatment regimens, such as basal-bolus combination therapy and the use of both insulin and oral hypoglycaemic agents (100.0% and 96.0%, respectively; p < 0.001 for BG > 10 mmol/L).

CONCLUSION

Inpatient glycaemic control is suboptimal. Factors (e.g. type of treatment regimen, discipline and baseline HbA1c) associated with greater prevalence of dysglycaemia should be given due consideration in patient management.     

Bilateral discoid medial menisci: a rare phenomenon

Discoid medial meniscus is a relatively rare pathology of the knee joint, with bilateral cases even rarer. Herein, we report the case of a 25-year-old man diagnosed with discoid medial meniscus in the right knee with a horizontal tear. Increased cupping of the medial condyle of the tibia, widening of the medial joint space and the presence of discoid meniscus in the right knee prompted investigation of the asymptomatic left knee with magnetic resonance imaging. The contralateral asymptomatic knee also showed evidence of discoid medial meniscus. The symptomatic knee was successfully treated by arthroscopic partial meniscectomy, with excellent functional outcome.     

Prolonged hepatitis and jaundice: a rare complication of paediatric Epstein-Barr virus infection

We herein report the case of a 14-year-old girl with Epstein-Barr virus (EBV) infectious mononucleosis who developed prolonged hepatitis and jaundice. At presentation, she had tender hepatomegaly with a markedly deranged liver function test. Abdominal ultrasonography showed hepatomegaly and a thickened gallbladder wall. During the subsequent 11 weeks, her transaminases showed two further peaks, which corresponded with clinical deterioration. Her highest alanine transaminase level was 1,795 µ/L and total bilirubin level was 154 µmol/L. She recovered fully with conservative management. EBV-related liver involvement is typically mild and self-limiting. We believe that tender hepatomegaly and gallbladder thickening may be important predictors of significant liver involvement. Although multiple transaminase peaks may occur, we do not consider this an indication for antiviral or immunosuppressive therapy. In the absence of strong evidence supporting the use of any specific therapy, we recommend a conservative approach for an immunocompetent patient.     

Orbital pseudotumour as a presentation of paediatric ulcerative colitis

A 2-year-old girl presented with a one-day history of acute-onset bilateral painful, swollen eyes and a two-month history of loose stools. Physical examination revealed a right eyelid swelling with proptosis. Magnetic resonance imaging revealed a right orbital pseudotumour. The patient responded well to treatment with intravenous antibiotics and nonsteroidal anti-inflammatory drugs. However, three weeks later, she was readmitted with a vasculitic lesion over her left upper chest, with mucous-bloody diarrhoea. Histopathology confirmed the diagnosis of ulcerative colitis. The patient was treated with intravenous pulse methylprednisolone and sulphasalazine. Two weeks after discharge, she was readmitted for cutaneous vasculitis and worsening diarrhoea. The patient’s bowel and extraintestinal diseases resolved upon addition of infliximab to her treatment regimen. Her inflammatory markers also normalised. Azathioprine was subsequently added. Infliximab was discontinued after four doses and prednisolone was tapered off. The patient remained well without any flare-up after 24 months of follow-up.     

Recurrent and persistent pityriasis rosea: an atypical case presentation

We report a case of atypical pityriasis rosea in a 24-year-old Malay man. He presented with an 11-month history of three recurrent and persistent episodes of pityriasis rosea associated with oral ulcers. The first episode lasted for one month and recurred within 14 days. The second episode lasted for three months and recurred within nine days. The third episode lasted for seven months. Although all three episodes were not preceded by any prodromal symptoms, a herald patch was noted on three different sites (the left iliac fossa, abdomen and chest) on each successive episode. Recurrent pityriasis rosea and its association with oral ulcers, although quite uncommon, have been reported in the literature. However, reports of multiple recurrences, with prolonged duration of each episode and very short remissions in between, have not been made. To the best of our knowledge, this is the first report of such unique presentation.     

Hepatic tuberculosis: a rare cause of fluorodeoxyglucose hepatic superscan with background suppression on positron emission tomography

Fluorodeoxyglucose (FDG) hepatic superscan refers to the diffuse intense uptake of 18F-FDG in the liver on positron emission tomography (PET), with reduced physiological activity in the brain and heart. The common causes include lymphoma and metastasis. In this case report, we describe the imaging features of tuberculosis as a rare cause of FDG hepatic superscan. PET imaging may be the only clue to a diagnosis of hepatic tuberculosis, as other imaging modalities may demonstrate only nonspecific hepatomegaly. It is important to consider this entity in the differential diagnosis of patients presenting with FDG hepatic superscan and proceed with liver biopsy for a definitive diagnosis.     

Dural metastasis of nasopharyngeal carcinoma: rare,but worth considering

Metastasis of nasopharyngeal carcinoma (NPC) to the dura, an extremely rare condition, can be symptomatically silent and mistaken for a benign entity radiographically. Missed diagnosis can lead to serious consequences or prove immediately fatal. We report a woman with dural metastasis of NPC that mimicked a meningioma on radiography. Craniectomy with tumour resection was performed due to rapid progression from the onset of symptoms to disability. The patient was still alive two years after surgery. This case emphasises the need to keep in mind the possibility of dural metastasis of NPC in patients with abnormal imaging features. This would not only avoid wrong and optimistic diagnosis, but also allow for appropriate treatment in a timely manner. To our knowledge, this is the first report of metastasis of NPC to the dura. We provide detailed information on the neoplastic lesion, which masqueraded as a benign entity and caused potentially fatal consequences.     

An unusual presentation of primary malignant B-cell-type dural lymphoma

Primary malignant B-cell-type dural lymphoma is a rare subtype of primary central nervous system lymphoma (PCNSL). We herein report an unusual case of diffuse B-cell lymphoma that presents as a chronic subdural haematoma without extracranial involvement. The notable aspects of this case include the patient’s immunocompetence, a short clinical history of symptom onset, rapid neurological deterioration and a final diagnosis of high-grade PCNSL. This case highlights the challenges neurosurgeons face, especially in the emergency setting, when the disease manifests in varied presentations.     

Challenges in the management of a rare case of extensive retroperitoneal haemangioma in a pregnant woman

Haemangioma of the retroperitoneal space is a rare benign capillary malformation, which can grow significantly in pregnancy due to the multiple associated cardiovascular changes. We herein describe the case of a pregnant woman with an extensive right retroperitoneal haemangioma extending from the level of the renal hilum, across the lateral anterior abdominal wall and into the thigh. We also highlight the challenges faced in the management of the patient’s delivery process. To the best of our knowledge, this is the first case of such nature and severity described in the English literature.     

A rare case of multiple bronchial artery aneurysms associated with a double aortic arch

Bronchial artery aneurysm is uncommon, and the occurrence of multiple aneurysms arising from a bronchial artery is even rarer. To date, there has been only one published case report describing double bronchial artery aneurysms. We herein describe a case of three aneurysms arising from a left bronchial artery, accompanied by multiple bilateral hypertrophied bronchial and intercostobronchial arteries, as well as a double aortic arch. Bronchial artery aneurysm is potentially life-threatening, and immediate treatment is recommended to minimise the potential risk of rupture. The aneurysms in our case were successfully treated via transcatheter arterial embolisation using coils.     

Unusual presentation of adult xanthogranuloma

Xanthogranulomas are the most common form of non-Langerhans cell histiocytosis. Both adult and childhood forms have been described. Adult cutaneous forms can present as solitary or multiple yellowish, orange-red or tan-hued papules. Herein, we present the case of a 28-year-old Chinese man with a skin-coloured nodule on his left nasal ala that persisted for several months. While initial impression was that of a fibrous papule of the nose, the results of an excision biopsy showed histological features corresponding to xanthogranuloma. This case demonstrates the condition''s myriad of dermatological presentations, and adds to the differential diagnoses of a cutaneous lesion found in the head and neck region.     

Dislocation of the penis: a rare complication after traumatic pelvic injury

Traumatic injury to the male external genitalia is frequently encountered, but acute traumatic dislocation of the penile structure is extremely rare, with only a few reports found in the literature. We herein report the case of a 21-year-old man who sustained blunt trauma to the pelvis following a motor vehicle accident, and had features suspicious of penile dislocation. With the use of computed tomography and bedside ultrasonography, a diagnosis of penile dislocation was made, which was subsequently confirmed intraoperatively. Immediate surgical intervention via gentle manipulation of the penile tissue back to its native position was performed in order to restore normal anatomy. The exact mechanism of penile dislocation is not known. However, circumferential laceration around the foreskin causing degloving injury of the penis is suggested in our patient.     

An unusual presentation of recurrent uterine rupture during pregnancy

We describe a case of recurrent uterine rupture at the site of a previous rupture. Our patient had a history of right interstitial pregnancy with spontaneous uterine fundal rupture at 18 weeks of pregnancy. During her subsequent pregnancy, she was monitored closely by a senior consultant obstetrician. The patient presented at 34 weeks with right hypochondriac pain. She was clinically stable and fetal monitoring showed no signs of fetal distress. Ultrasonography revealed protrusion of the intact amniotic membranes in the abdominal cavity at the uterine fundus. Uterine rupture is a rare but hazardous obstetric complication. High levels of caution should be exercised in patients with a history of prior uterine rupture, as they may present with atypical symptoms. Ultrasonography could provide valuable information in such cases where there is an elevated risk of uterine rupture at the previous rupture site.     

Struma ovarii: management and follow-up of a rare ovarian tumour

INTRODUCTION

Struma ovarii represents about 1.0% of all ovarian tumours. While management involves surgery, there is a paucity of data regarding the extent and approach of surgery, and postoperative management. This study aimed to delineate the management of struma ovarii, its associated complications, and postoperative follow-up and investigations.

METHODS

We retrospectively reviewed cases of benign struma ovarii treated at KK Women’s and Children’s Hospital, Singapore, between January 2000 and May 2011.

RESULTS

A total of 68 patients underwent surgical removal of ovarian cyst or mass (24 cystectomy, 20 salpingo-oopherectomy and 24 total hysterectomy and bilateral salpingo-oopherectomy). Of the 68 surgeries, 39 (57.4%) included intraoperative frozen section sampling or procedures for staging of ovarian malignancy. The majority (73.5%) of surgeries were laparotomies. Histology revealed benign struma ovarii in all (98.5%) but one patient. Only 7 (10.3%) patients had postoperative complications – 3 wound-related, 2 thyroid-related, 1 incisional hernia and 1 nonspecific. The mean length of hospital stay was 4.2 days. During follow-up, 45 (66.2%) patients required no additional investigations. The most common investigation done was ultrasonography (n = 18, 26.5%). While no recurrences were diagnosed histologically, two patients were subsequently found to have complex/dermoid ovarian cysts on the ipsilateral side of the previous struma ovarii on ultrasonography.

CONCLUSION

Simple surgery is recommended for patients with struma ovarii, especially if they have fertility potential. Laparoscopic surgery is the recommended approach due to its shorter recovery time and lower morbidity. Most patients do not require extended periods of follow-up or postoperative investigations.     

Blue rubber bleb nevus syndrome: a rare cause of chronic gastrointestinal bleed in adults

Blue rubber bleb nevus syndrome (BRBNS) is a rare condition characterised by venous malformations in the skin, gastrointestinal tract and other parts of the body. Its presentation is usually sporadic, although cases of autosomal dominant inheritance have been reported. Usually seen in children, BRBNS presentation in adults is rare. Symptoms at presentation depend on the organs involved; patients with BRBNS may present with acute or chronic gastrointestinal bleed. We herein report a rare presentation of BRBNS in an adult who suffered from intermittent abdominal pain and melaena for three years. Contrast-enhanced computed tomography revealed a jejunojejunal intussusception with a vascular malformation as the lead point. The patient underwent laparotomy with resection of the intussuscepted bowel segment. Recovery was uneventful. In spite of a wide range of therapeutic options for the management of BRBNS described in the literature, the efficacy of those available therapies, including surgical excision, is not well established.