Kikuchi-Fujimoto disease associated with systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report.

BACKGROUND: Kikuchi-Fujimoto disease, which was originally described in young women, is a benign condition characterized by necrotizing lymphadenitis and fever. Even though the clinical course is usually self-limiting, it can be associated with recurrences and rarely can be associated with systemic lupus erythematosus or can be complicated with hemophagocytic lymphohistiocytosis. We report the case of a 17-year-old Sri Lankan Sinhalese schoolboy who presented with fever and cervical lymphadenopathy diagnosed as Kikuchi-Fujimoto disease and was complicated with hemophagocytic lymphohistiocytosis subsequently. Later he fulfilled the criteria for systemic lupus erythematosus. CASE PRESENTATION: A 17-year-old previously healthy Sinhalese schoolboy presented with high-grade fever associated with chills and rigors associated with loss of appetite and loss of weight for more than 40 days. On examination, he had bilateral firm matted tender cervical lymphadenopathy and firm hepatomegaly. An excision biopsy of his right cervical lymph node revealed necrotizing lymphadenitis and immunohistochemistry of a lymph node biopsy favored Kikuchi disease. Initial antinuclear antibody and anti-double-stranded deoxyribonucleic acid tests were negative and his C3 and C4 levels were normal. An infections screening was negative. He was treated with steroids. While in hospital he developed hemophagocytic lymphohistiocytosis and renal impairment. Later his antinuclear antibody titer became positive in 1:160 and fulfilled the diagnostic criteria for systemic lupus erythematosus. He was managed with steroids and immune suppressive drugs and showed remarkable improvement. CONCLUSION: Although Kikuchi-Fujimoto disease is uncommon in male patients, it needs to be considered in patients with lymphadenopathy and fever. The disease can be complicated with hemophagocytic lymphohistiocytosis and the patients need continuous monitoring for the possible development of systemic lupus erythematosus later in the course.

A patient with neurofibromatosis type 1 presenting with bilateral frontal lobe infarctions following anterior communicating artery aneurysm rupture.

Neurofibromatosis is a neurocutaneous genetic condition with dysplasia of the mesodermal and ectodermal tissues. Vascular abnormalities are well recognized in neurofibromatosis and cerebral aneurysms are rarely reported in literature. Here, we present a 20-year-old Sri Lankan female presented with headache, altered personality, disinhibited behaviour, and urinary incontinence. On imaging, she was found to have infarctions of both frontal lobes and evidence of a ruptured anterior communicating artery aneurysm with a small subarachnoid haemorrhage. Another small middle cerebral artery aneurysm was also seen in the angiogram. She was managed conservatively and gradually recovered. Because aneurysms in neurofibromatosis are usually asymptomatic and as rupture of such an aneurysm is rare, regular vascular screening is not recommended to all patients with neurofibromatosis. This is the first case report in literature in which a patient with neurofibromatosis presented with infarctions of both frontal lobes due to rupture of an anterior communicating artery aneurysm.

Knowledge, attitudes and skills in melanoma diagnosis among doctors: a cross sectional study from Sri Lanka.

OBJECTIVES: This study aimed to assess the knowledge, attitudes and skills of non-specialist doctors on timely referral of suspicious lesions for melanoma diagnosis. RESULTS: One hundred and twenty-three doctors (mean age; 30.4 years, SD ± 8.015) were enrolled. Very few (3.3%) correctly stated all four types of melanoma. Only 8.1% of the total sample had been trained to perform a total body examination for skin cancer detection and a majority (110/123) had never performed one. Almost all (95.2%) were not confident in using a dermatoscope for examination of a skin lesion. Only 17.9% of participants had discussed skin cancer/melanoma risk reduction with patients. Only 13.8% had educated at least one patient regarding skin self-examination for suspicious skin lesions. Knowledge and clinical skills regarding melanoma recognition was unsatisfactory in our sample. Urgent attention is needed to bridge the gap in knowledge and clinical skills on this topic.

A case report of giant pancreatic pseudocyst following acute pancreatitis: experience with endoscopic internal drainage.

BACKGROUND: Pancreatic cysts are being diagnosed more frequently because of the increasing usage of imaging techniques. A pseudocyst with the major diameter of 10 cm is termed as a giant cyst. Asymptomatic pseudo-cysts up to 6 cm in diameter can be safely observed and monitored without intervention, but larger and symptomatic pseudocysts require intervention. CASE PRESENTATION: A 27-year-old Sri Lankan male, with history of heavy alcohol use, presented with progressive abdominal distension following an episode of acute pancreatitis. Contrast enhanced CT scan of the abdomen showed a large multilocular cystic lesion almost occupying the entire abdominal cavity and displacing the liver medially and the right dome of the diaphragm superiorly. The largest locule in the right side measured as 30 cm × 15 cm × 14 cm. Endoscopic ultrasound guided drainage of the cyst was performed. The cyst was entered into with an electrocautery-assisted cystotome and a lumen-opposing metal stent was deployed under fluoroscopic vision followed by dilatation with a 10 mm controlled radial expansion balloon. Repeat endoscopic ultrasound was done a week later due to persistence of the collection and a second stent was inserted. Then 10 French gauge × 10 cm double ended pigtails were inserted through both stents. The cysts were not visualized on subsequent Ultra sound scans. Stent removal was done after 3 weeks, leaving the pigtails insitu. The patient made an uneventful recovery. CONCLUSION: Giant pancreatic pseudocysts are rare and earlier drainage is recommended before clinical deterioration. Some experts suggest that cystogastrostomy may not be appropriate for the treatment of giant pancreatic pseudocysts and in some instances external drainage of giant pancreatic pseudocysts may be safer than cystogastrostomy. Video-assisted pancreatic necrosectomy with internal drainage and laparoscopic cystogastrostomy were also tried with a good outcome. With our experience we suggest endoscopic guided internal drainage as a possible initial method of management of a giant pseudo cyst. However long-term follow up is needed with repeated imaging and endoscopy. In instances where the primary endoscopic internal drainage fails, surgical procedures may be required as a second line option.

A case of anterior spinal cord syndrome in a patient with unruptured thoracic aortic aneurysm with a mural thrombus.

BACKGROUND: Spinal cord infarction is an uncommon condition. Anterior cord syndrome present with paraparesis or quadriparesis with sparing of vibration and proprioceptive senses. The common causes of anterior cord syndrome are aortic dissection and aortic surgical interventions. Spontaneous unruptured nondissected aortic aneurysms with intramural thrombus can rarely cause anterior cord infarctions. CASE PRESENTATION: We report a case of anterior spinal cord syndrome due to aneurysm of the thoracic aorta with a mural thrombus. A 64 year old male presented with sudden onset paraparesis with a sensory level at T1 with preserved sense of proprioception and vibration. The MRI panspine revealed increased T2 intensity in the anterior portion of the spinal cord from C5 to T10 level with characteristic 'owl eye' appearance on axial imaging. The CT aortogram detected aneurysmal dilatation of the ascending aortic, arch and descending thoracic aorta with significant intimal irregularities, calcified atherosclerotic plaques and a small mural thrombus. CONCLUSION: The possible mechanisms postulated are occlusion of ostia of radicular arteries by the atherosclerotic plaques and mural thrombus or thromboembolism to the anterior spinal artery. Nondissected atherosclerotic aortic aneurysms should be considered in patients presenting with spinal cord infarctions especially in the presence of vascular risk factors and smoking.

Erythroderma and extensive poikiloderma - a rare initial presentation of dermatomyositis: a case report.

BACKGROUND: Dermatomyositis is a humoral-mediated inflammatory myopathy with symmetrical proximal muscle weakness and dermatological manifestations such as Gottron's papules, heliotrope rash, periungual abnormalities, and flagellate erythema. Erythroderma is a severe and potentially life-threatening dermatological condition with diffuse erythema and scaling involving more than 90% of the skin surface area. Poikiloderma vasculare atrophicans refers to mottled hyperpigmentation and hypopigmentation of the skin with in-between telangiectases and areas of atrophy and is considered a variant of mycosis fungoides. Poikilodermatomyositis is the term given to the condition with poikiloderma and inflammatory myopathy. Only a few cases are reported on erythroderma in dermatomyositis and poikilodermatomyositis. Erythrodermal pattern of dermatomyositis transforming into poikilodermatomyositis is a recognized rare manifestation of dermatomyositis and we could find only one case report in the literature. CASE PRESENTATION: A 53-year-old Sri Lankan woman presented with intermittent fever of 5 months' duration with erythroderma. Later she developed progressive, symmetrical proximal muscle weakness. Following a short course of small dose steroids, erythroderma settled but changed to extensive poikiloderma involving more than 90% of her skin with her face being relatively spared. She had an early heliotrope rash, shawl sign, and Gottron papules. Electromyography and muscle biopsy were supportive of inflammatory myositis and skin biopsy showed evidence of dermatomyositis. Inflammatory markers and muscle enzymes were also elevated. Autoimmune antibodies and myositis-specific autoantibodies were negative. She was started on orally administered prednisolone 1 mg/kg per day with methotrexate 10 mg once a week and had a good response to treatment with resolution of the skin condition and improvement of muscle power. Imaging studies, endoscopies, and tumor markers did not reveal any malignancy. CONCLUSIONS: This case illustrates a rare presentation of dermatomyositis initially presenting as fever, erythroderma, and proximal muscle weakness and later developing poikiloderma involving more than 90% of the skin. It is important to be aware of this rare presentation to avoid misdiagnosis. With the currently available literature it is not possible to conclude that erythroderma is a bad prognostic factor in dermatomyositis or a predictive factor for a malignancy. Patients have a good response to steroids with a combination of immunosuppressants.

A case report of zinc phosphide poisoning: complicated by acute renal failure and tubulo interstitial nephritis.

BACKGROUND: Run Rat® is a rodenticide widely used against small mammals. It comprises of a minimum of 32% zinc phosphide which is highly toxic in acute exposures to humans. It may be consumed accidentally or intentionally. It enters the body via skin, respiratory and gastrointestinal tracts. Zinc phosphide is hydrolyzed by the gastric acid and is transformed into phosphine gas. Phosphine is a respiratory toxin that inhibits cytochrome C oxidase system resulting in renal failure and liver failure. CASE PRESENTATION: A 35 year old Sri Lankan female presented following ingestion of 2.5 g of Run Rat®, which is a branded preparation of zinc phosphide, resulting in 61 mg/kg poison load. She developed severe acute kidney injury with acute tubular necrosis, subnephrotic ranged proteinuria and tubulointerstitial nephritis for which she underwent haemodialysis three times along with other measures of resuscitation. She also developed elevated liver enzymes with hyperblirubinaemia, hypoalbuminaemia, acute pancreatitis and mild myocarditis. She improved with supportive therapy over a period of 3 weeks. CONCLUSION: Run Rat® is a commonly used rodenticide and the toxic effects are mediated through conversion of phosphide to phosphine gas. The majority of the deaths had occurred in the first 12 to 24 h and the main causes identified are refractory hypotension and arrhythmias. The late deaths (beyond 24 h) had been commonly due to adult respiratory distress syndrome, liver and renal failure. The outcome is poorer with delayed presentation, development of coagulopathy, hyperglycaemia and multiorgan failure with elevated liver enzymes. In our patient, Zinc phosphide poisoning caused severe acute kidney injury, abnormal liver profile, pancreatitis and possible myocarditis. The patient improved with repeated haemodialysis. The renal biopsy revealed acute tubulointerstitial nephritis with acute tubular necrosis. In tropical countries, the rural population engaged in agriculture has easier access to the compound, as it is available at a lower cost. Furthermore, the lack of an antidote and advanced resuscitative measures such as inotropic supportive therapy and renal replacement facilities at most of the peripheral hospitals pose a major challenge in providing timely interventions to prevent deaths.

Electrocardiographic changes mimicking acute coronary syndrome in a large intracranial tumour: A diagnostic dilemma.

BACKGROUND: ST elevation Myocardial infarction is a medical emergency. A variety of noncardiac conditions had been known to mimic the ECG changes that are seen in acute coronary syndrome. Although the common ECG changes that are documented with raised intracranial pressure are T inversions, prolongation of QT interval and sinus bradycardia, ST elevation or depression, arrhythmias and prominent U waves have also been recognized. However, ST elevations in association with primary intracranial tumours are rarely reported. CASE PRESENTATION: A 68-year-old female patient with a large left sided frontoparietal sphenoidal ridge meningioma with mass effect developed sudden onset shortness of breath while awaiting surgery. Her ECG showed ST segment elevations in the inferior leads along with reciprocal T inversions in anterior leads. The patient was treated with dual antiplatelet therapy and unfractionated heparin. The ST elevations in the ECG remained static and the cardiac Troponin assay was repeatedly negative. 2D ECHO, coronary angiogram and CT pulmonary angiography were normal. The repeat noncontract CT scan of the brain revealed two small areas of haemorrhage in the tumour. CONCLUSION: The two mechanisms for ECG changes described in subarachnoid haemorrhage are the neurogenic stunned myocardium due to the catecholamine surge on the myocytes and stress cardiomyopathy. The same mechanisms could be the reasons for the ECG changes seen in intracranial tumours. These ECG changes could be easily misdiagnosed as acute coronary syndrome. This case emphasizes the importance of the cardiac biomarkers, 2D ECHO and coronary angiogram when confronted with such a diagnostic dilemma. Thus a more holistic analysis should be practiced in diagnosing acute coronary events in patients with intracranial pathologies to obviate a myriad of unnecessary investigations, interventions, costly treatment strategies which may well be detrimental to the patient.

Large hepatocellular carcinoma in a non-cirrhotic liver with peritoneal and omental metastasis in a healthy man: a case report.

BACKGROUND: Liver cancer is the second leading cause of cancer death in men worldwide. Hepatocellular carcinoma usually develops in the setting of cirrhosis or chronic inflammation. Major risk factors for developing hepatocellular carcinoma are chronic hepatitis B or C virus infection, alcoholic cirrhosis, and nonalcoholic fatty liver disease. The most frequent locations for hepatocellular carcinoma to metastasize are the lungs, portal vein, bones, and regional lymph nodes. CASE PRESENTATION: A 41-year-old Sri Lankan man presented with progressive abdominal distension and on examination was found to have a palpable irregular mass in the left lobe of his liver with moderate ascites. His ascitic fluid was an exudate without malignant cells. An ultrasound scan and contrast-enhanced computed tomography of his abdomen showed a large contrast-enhancing lesion in the left lobe of his liver without features of cirrhosis. Laparoscopic assessment revealed peritoneal and omental deposits. Histology of the biopsies taken from the liver lesion, omental deposits, and peritoneal deposits supported a diagnosis of hepatocellular carcinoma. His liver biochemistry was normal and hepatitis serology was negative. He is abstinent from alcohol and did not have metabolic syndrome. CONCLUSIONS: It is rare for a young patient to develop hepatocellular carcinoma with a normal liver without chronic hepatitis B or C infection, or any other risk factors. Intraperitoneal metastasis of non-ruptured hepatocellular carcinoma is also very rare. Here we report a rare case of a 41-year-old man with a large hepatocellular carcinoma in a non-cirrhotic liver without chronic hepatitis who presented with peritoneal and omental metastasis.

Amitraz poisoning: A case report of an unusual pesticide poisoning in Sri Lanka and literature review.

BACKGROUND: Amitraz is a pesticide used worldwide on animals and in agriculture. It contains triazapentadiene, which is a centrally acting alpha-2 adrenergic agonist. Amitraz poisoning is fairly uncommon in humans and occurs via oral, dermal or inhalational routes. Only a limited number of case reports of human intoxication have been published and most of them are of accidental ingestion by children. CASE PRESENTATION: A twenty-year-old Sri Lankan female presented following self-ingestion of 20 ml of amitraz resulting in 37.8 mg/ kg of amitraz poisoning. She lost consciousness after 20 min of ingestion, developed bradycardia and hypotension, which needed intravenous fluid resuscitation and dobutamine. Gastric lavage was performed. Her bradycardia persisted for 36 h and she was drowsy for 48 h. She did not develop respiratory depression, convulsions or hypothermia and the urine output was normal. Arterial blood gas revealed mild respiratory alkalosis. She recovered fully within 48 h and was discharged on day 3. CONCLUSION: The clinical manifestations of amitraz (impaired consciousness, drowsiness, vomiting, disorientation, miosis, mydriasis, hypotension, bradycardia, respiratory depression, hypothermia, generalized seizures, hyperglycemia and glycosuria) can be explained by the agonist action of amitraz on α1 and α2 receptors. Management of amitraz poisoning is still considered to be supportive and symptomatic with monitoring of nervous system, cardiovascular and respiratory systems. Activated charcoal may still be considered for treatment and the place for gastric lavage is controversial. Atropine is effective for symptomatic bradycardia and inotropic support is needed for hypotension that does not respond to fluid resuscitation. Diazepam or Lorazepam is used for convulsions and some patients may require intubation and ICU care. Several α2 adrenergic antagonists like yohimbine have been tried on animals, which have successfully reversed the effects of amitraz. Since the majority of amitraz poisoning cases are due to accidental ingestion, manufactures, regulatory authorities and national poisons control centers have a significant role to play in minimizing its occurrence.

Bilateral rectus sheath haematoma complicating dengue virus infection in a patient on warfarin for mechanical aortic valve replacement: a case report.

BACKGROUND: The management of Dengue virus infection can be challenging. Varied presentations and numerous complications intrinsic to dengue by itself increase the complexity of treatment and potential mortality. When burdened with the presence of additional comorbidities and the need to continue compulsory medications, clear stepwise definitive guidance is lacking and patients tend to have more complex complications and outcomes calling to question the clinical decisions that may have been taken. The use and continuation of warfarin in dengue virus infection is one such example. CASE PRESENTATION: We report a 65 year old South Asian female who presented with dengue fever. She had a history bronchial asthma, a prior abdominal surgery, and was on warfarin and maintained a therapeutically appropriate internationalized normalized ratio for a mechanical aortic valve replacement. Though preemptive decision to stop warfarin was taken with decreasing platelet counts, her clinical course was complicated with the development of bilateral rectus sheath haematoma's requiring resuscitation with blood transfusions. CONCLUSION: Though management of dengue viral fever has seen drastic evolution with recent updated guidance, clinical scenarios seen in the course of the illness still pose challenges to the managing physician. The need to continue obligatory anticoagulation which may seem counterintuitive during a complex disease such as dengue virus infection must be considered after understanding the potential risks versus that of its benefits. Though case by case decisions maybe warranted, a clear protocol would be very helpful in making clinical decisions, as the correct preemptive decision may potentially avert catastrophic and unpredictable bleeding events.

Acute pancreatitis complicated with deep vein thrombosis and pulmonary embolism: a case report.

BACKGROUND: Acute pancreatitis is an acute inflammatory process of the pancreas that can trigger a systemic inflammatory response. Pulmonary embolism refers to obstruction of the pulmonary artery or one of its branches by material (usually a thrombus) that originated elsewhere in the body. Extensive lower limb deep vein thrombosis with pulmonary embolism is a rare complication of acute pancreatitis that has been described in a few case reports. Deep vein thrombosis and hypercoagulable states in pancreatitis are thought to be due to release of pancreatic proteolytic enzymes from a cyst that is connected to the pancreatic duct and penetrates into a vessel. Proteolytic damage or inflammation of the vessels may also play a significant part. Acute pancreatitis also causes a systemic inflammatory response that has effects on an endothelium-dependent relaxing response for acetylcholine. CASE PRESENTATION: A 38-year-old Sri Lankan man presented with acute pancreatitis and later he developed progressive abdominal distention with bilateral ankle edema. A contrast-enhanced computed tomographic scan showed two pancreatic pseudocysts and deep vein thrombosis in both lower limbs, as well as a pulmonary embolism involving the right lower lobe pulmonary artery and the left segmental pulmonary arteries. One of the pseudocysts in the head of the pancreas was compressing the inferior vena cava without direct communication. The patient's thrombophilia screen result was negative. He was started on subcutaneous enoxaparin 1 mg/kg twice daily and warfarin to achieve a target international normalized ratio of 2-3. CONCLUSIONS: Deep vein thrombosis with pulmonary embolism is a rare but life-threatening complication of acute pancreatitis. Once diagnosed, early treatment with intravenous heparin or thrombolysis is effective. Patients with severe acute pancreatitis may be at risk of deep vein thrombosis due to immobilization and other mechanisms, but anticoagulation as prophylaxis is often not used. However, it may be considered on a case-by-case basis in patients with pancreatitis who are acutely ill and immobilized, need intensive care unit admission, and have multiple risk factors for deep vein thromboembolism. Further studies must be undertaken to determine guidelines for deep vein thromboembolism prophylaxis in these patients.

Lucio's phenomenon, an uncommon occurrence among leprosy patients in Sri Lanka.

BACKGROUND: Lucio's phenomenon is a rare manifestation of untreated leprosy which is seen almost exclusively in regions surrounding the Gulf of Mexico. Its occurrence elsewhere though documented is considered uncommon. We present a case of Lucio's phenomenon in a previously undiagnosed leprosy patient who presented to us with its classical skin manifestations. CASE PRESENTATION: A 64 year old South Asian (Sri Lankan) male with a history of chronic obstructive airway disease presented to us with fever and cough. He had a generalized smooth and shiny skin with ulcerating skin lesions afflicting the digits of the fingers. The lesions progressed to involve the extremities of the body and healed with crusting. Based on the clinical and investigational findings Tuberculosis and common vasculitic conditions were suspected and excluded. The unusual skin manifestations prompted a biopsy, and wade fite stained revealed Mycobacterium bacilli. In context of the clinical picture and histological findings, Lucio's phenomenon was suspected. A clinical diagnosis of Lucio's phenomenon occurring in the backdrop of lepromatous leprosy was made. CONCLUSION: Though leprosy is still a prevalent disease, it has manifestations that are not easily recognized or fully appreciated. Regional patterns of atypical manifestations should not limit better understanding of rarer manifestations as it will aid in clinching an early diagnosis and instituting prompt treatment, thereby reducing morbidity and mortality.

Tropical pyomyositis as a presenting feature of subclinical leukemia: a case report.

INTRODUCTION: Pyomyositis, though classically considered a tropical disease, has a variable geographic prevalence. Among the predisposing risk factors, immunodeficiency plays an important role. Pyomyositis has a tendency to mimic more commonly considered diseases, and a lack of familiarity with it is a cause of delayed diagnosis. CASE PRESENTATION: A 53-year-old South Asian man with newly diagnosed type 2 diabetes mellitus was referred to our medical unit in an advanced stage of the disease, which was complicated by sepsis and acute kidney injury. Failure of the referring unit to provide prompt treatment, as well as their delay in coming to a diagnosis, led to the patient's complicated state. Antibiotic therapy was initiated, and clinical stabilization was achieved with supportive measures. Following the patient's recovery from sepsis, his persistent leukopenia and anemia was suggestive of an underlying immunodeficiency, and a subsequent bone marrow biopsy revealed acute myeloid leukemia, M2 variant. Multi-disciplinary care was initiated by the medical, surgical and oncological teams. CONCLUSION: Awareness of tropical pyomyositis is lacking. Common predisposing behaviors and conditions should always be sought and investigated. Immunosuppressive state is an important predisposing factor in the pathogenesis of pyomyositis. Early antibiotic treatment is pivotal in management, and surgical intervention, when relevant, should not be delayed. Identifying one cause should not halt the search for others, as pyomyositis may herald underlying sinister diseases.

Inoperable inflammatory myofibroblastic tumour of the para-nasal sinuses and orbit with recurrence responding to methotrexate and prednisolone: a case report.

BACKGROUND: Inflammatory myofibroblastic tumour is a rare neoplasm with a potential to behave in a malignant manner. It can occur anywhere in the body, however involvement of the head, especially the para-nasal sinuses is rare. CASE PRESENTATION: A 33-year-old South Asian male presented with coryzal symptoms including a persistent cough with an asymmetrical swelling of the left side of the face. Imaging revealed a mass lesion involving the para-nasal sinuses eroding into the orbit. Histology and the clinical picture were compatible with inflammatory myofibroblastic tumour. As curative excision of the tumour was not feasible, medical management was offered. Despite early features of remission to glucocorticoids, tapering resulted in recurrence. Hence combination therapy with glucocorticoids and methotrexate was commenced with dramatic reduction of tumour burden and the patient has been in remission to date. CONCLUSION: Inflammatory myofibroblastic tumour has the potential to behave in a malignant manner. Medical management with chemotherapy, glucocorticoids and non-steroidal anti-inflammatory drugs though effective, do not have a uniform response pattern. Surgically unresectable inflammatory myofibroblastic tumour above neck should be treated aggressively with combination regimens. Combination of prednisolone with methotrexate has been shown to have good outcome.

Polyarteritis nodosa complicated by posterior reversible encephalopathy syndrome: a case report.

BACKGROUND: Posterior reversible encephalopathy syndrome is a presentation which is diagnosed clinico-radiologically. The primary aetiological processes leading to posterior reversible encephalopathy syndrome are many, which include autoimmune conditions. Polyarteritis nodosa as an aetiological factor for posterior reversible encephalopathy syndrome is rare. We present a case of polyarteritis nodosa complicated by posterior reversible encephalopathy syndrome. CASE PRESENTATION: A 26-year-old South-Asian female presented with left sided focal seizures with secondary generalization and visual disturbance for 2 days duration. She had a prior history of arthralgia and weight loss with no medically explainable cause for young onset hypertension. Examination revealed a right claw hand with a palpable vasculitic type of rash involving both the palmar surfaces. Symptoms responded to management with anti-hypertensives and anti-epileptics. Whole blood count, iron studies, erythrocyte sedimentation rate and C-reactive protein values portrayed an ongoing chronic inflammatory process. Serological studies such as Anti-nuclear antibody, Anti -double stranded deoxyribonucleic acid, Anti-neutrophil cytoplasmic antibody and Anti-cyclic citrulinated peptide were negative. Magnetic resonance imaging revealed high signal intensity on T2 in both occipital lobes. Skin biopsy of the palm revealed moderate vessel vasculitis. Renal imaging revealed structurally abnormal kidneys with micro aneurysms in the right renal vasculature. Repeat magnetic resonance imaging of the brain two months later showed marked improvement. A diagnosis of polyarteritis nodosa with posterior reversible encephalopathy syndrome was made. CONCLUSIONS: Posterior reversible encephalopathy syndrome should not be missed. Investigations for an aetio-pathological cause should be considered including the rarer associations like polyarteritis nodosa.

Bronchiectasis and hoarseness of voice in takayasu arteritis: a rare presentation.

BACKGROUND: Takayasu arteritis is a large vessel vasculitis occurring in young females. We report a rare presentation of Takayasu arteritis in a Sri Lankan woman. She presented with bronchiectasis and left recurrent laryngeal nerve palsy prior to the onset of vascular symptoms. This case illustrates an atypical presentation of this disease and the diagnostic dilemma that the physician may be faced with. CASE PRESENTATION: A 39-year-old woman presented with chronic cough, haemoptysis and hoarseness of voice. She had left recurrent laryngeal nerve palsy and high inflammatory markers on investigation. CT thorax revealed aortic wall thickening and traction bronchiectasis. 2 D echocardiogram revealed grade 1 aortic regurgitation compatible with aortitis. She did not have weak peripheral pulses or a blood pressure discrepancy and did not meet American College of Rheumatology (ACR) criteria for diagnosis of Takayasu arteritis at this stage. Tuberculosis, syphilis and sarcoidosis was excluded. While awaiting angiography, she developed left arm claudication and a pericardial effusion. Angiography revealed evidence of Takayasu arteritis and absence of flow in the left subclavian artery. Takayasu arteritis was diagnosed at this stage after a period of eight months from the onset of initial symptoms. She is currently on prednisolone, azathioprine and aspirin. CONCLUSION: Bronchiectasis and recurrent laryngeal nerve palsy is a rare presentation of Takayasu arteritis. Atypical presentations can occur in Takayasu arteritis prior to the onset of vascular symptoms. Elevation of inflammatory markers are an early finding. A high degree of suspicion is needed to identify these patients in the early course of the disease.