A Case of Polyarteritis Nodosa Presenting as Rapidly Progressing Intermittent Claudication of Right Leg.

BACKGROUND: Polyarteritis nodosa (PAN) is a medium vessel vasculitis which causes significant morbidity and mortality. Usually, it presents with constitutional symptoms with angiographic evidence of aneurysms or segmental stenosis of arteries of mesenteric or renal vasculature. It is exceedingly uncommon for PAN to present with symptomatic progressive intermittent claudication. CASE PRESENTATION: We describe a 60-year-old male who presented with rapidly progressive intermittent claudication of his right leg. He did not have any significant atherosclerotic risk factors. He had recent onset hypertension and loss of weight. He also had mononeuropathy of right common peroneal nerve and livedo reticularis rash. With negative autoimmune markers and suggestive histology in deep punch skin biopsy and angiographic evidence of segmental stenosis of femoral and renal arteries, we diagnosed PAN. We treated him with aggressive immunosuppressants and vascular bypass surgery of right femoral vessels; he showed a good response. CONCLUSION: Rapidly progressive unilateral intermittent claudication could be a very rare, but noteworthy presentation of PAN. With suggestive histology and exclusion of other comorbidities aggressive immunosuppressants should be instituted. Vascular bypass surgery for critical ischaemia of the limbs is an option that could be considered for limb-threatening disease.

Acute pulmonary melioidosis presenting with multiple bilateral cavitary lesions in a healthy young adult: an authentic case report from Sri Lanka.

BACKGROUND: Melioidosis is an emerging infectious disease in Sri Lanka. This disease usually develops in a host with an immunocompromised state. Acute pulmonary melioidosis commonly presents as a lobar consolidation with or without pulmonary nodules or abscesses involving the upper lobes of the lungs. CASE PRESENTATION: We report a young healthy female who does not have known risk factors or immunocompromised state, presented with bilateral multiple cavitary lesions involving all three zones of the lungs. She used to involve in home gardening. Her initial relevant microbiological investigations were all negative. The diagnosis of melioidosis was made by broncho-alveolar lavage fluid culture positivity combined with a highly positive antibody titre. She showed dramatic response to intravenous high dose Meropenem. CONCLUSIONS: Melioidosis should be suspected early in patients with acute pulmonary involvement who show poor response to conventional antibiotics, even in the absence of known risk factors for disease. Other than known occupational exposures, household exposures such as home gardening should also be considered as a possible mode of exposure.

A case of multiple myeloma presenting as a distal renal tubular acidosis with extensive bilateral nephrolithiasis.

BACKGROUND: At the time of diagnosis, Multiple Myeloma is commonly associated with renal impairment. Renal tubular acidosis without overt renal insufficiency is an uncommon disease presentation of myeloma. Among tubular acidosis types, isolated renal tubular acidosis is a very unusual presentation of multiple myeloma. CASE PRESENTATION: We present a 55 years old female who presented with lower limb weakness due to persistent hypokalaemia caused by distal renal tubular acidosis. On further investigation of her anaemia with high erythrocyte sedimentation rate, we diagnosed IgG myeloma. CONCLUSION: Isolated distal renal tubular acidosis is a rare presentation of multiple myeloma. In the absence of hypercalciuria and demonstrable light chain excretion in urine, we assumed that the distal renal tubular acidosis could have been caused by monoclonal hypergammaglobulinaemic state of multiple myeloma.

Paraneoplastic limbic encephalitis with associated hypothalamitis mimicking a hyperdense hypothalamic tumor: a case report.

BACKGROUND: Paraneoplastic limbic encephalitis is an uncommon association of common malignancies such as small cell lung carcinoma, testicular teratoma, and breast carcinoma. The nonspecific nature of the clinical presentation, lack of freely available diagnostic markers, and requirement for advanced imaging techniques pose a great challenge in the diagnosis of this disease in resource-poor settings. CARE PRESENTATION: A 64-year-old previously healthy Sri Lankan man was admitted to the general medical unit with subacute memory impairment regarding recent events that had occurred during the previous 3 weeks. Initial noncontrast computed tomography of the brain revealed a hyperdensity in the hypothalamic region surrounded by hypodensities extending toward the bilateral temporal lobes; these findings were consistent with a possible hypothalamic tumor with perilesional edema. The patient later developed cranial diabetes insipidus, which was further suggestive of hypothalamic disease. Interestingly, gadolinium-enhanced magnetic resonance imaging of the brain showed no such lesions; instead, it showed prominent T2-weighted signals in the inner mesial region, characteristic of encephalitis. The possibility of tuberculosis and viral encephalitis was excluded based on cerebrospinal fluid analysis results. Limbic encephalitis with predominant hypothalamitis was suspected based on the radiological pattern. Subsequent screening for underlying malignancy revealed a mass lesion in the right hilum on chest radiographs. Histological examination of the lesion showed small cell lung cancer of the "oat cell" variety. CONCLUSION: We suggest that the initial appearance of a hyperdensity in the hypothalamus region on noncontrast computed tomography is probably due to hyperemia caused by hypothalamitis. If hypothalamitis is predominant in a patient with paraneoplastic limbic encephalitis, magnetic resonance imaging will help to differentiate it from a hypothalamic secondary deposit. Limbic encephalitis should be considered in a patient with computed tomographic evidence of a central hyperdensity surrounded by bitemporal hypodensities. This pattern of identification will be useful for early diagnosis in resource-poor settings.

Blindness in a Sri Lankan woman with bilateral breast lumps: a case report.

BACKGROUND: Granulomatosis with polyangiitis is a rare multisystemic autoimmune disorder predominantly affecting the upper and lower respiratory tracts and the kidneys, and rarely affecting other organ systems. Tuberculosis can mimic the presentation of granulomatosis with polyangiitis, and both can occur simultaneously in the same patient. Here we report what we believe to be the first case of concurrent granulomatous breast lesions and hemorrhagic retinal angiopathy in a Sri Lankan woman with refractory granulomatosis with polyangiitis complicated by probable tuberculosis. CASE PRESENTATION: A 48-year-old Sri Lankan Moorish woman presented with a 6-month history of ulcerating bilateral breast lumps, a 3-month history of non-healing painful ulcers on the palate, and sudden bilateral painless loss of vision. Retinoscopy confirmed left-sided retinal hemorrhages and bilateral panuveitis. An examination of her respiratory system showed bilateral coarse crepitations. Histologic examination of the palatal and breast lesions showed chronic granulomatous inflammation. Her levels of immune markers were elevated but her renal function was normal. Chest radiography showed bilateral mid-zone and lower-zone infiltrates with cavitation and small pleural effusions. Her serum proteinase 3 anti-neutrophil cytoplasmic antibody titer and the level of adenosine deaminase in her pleural fluid were significantly elevated. She was diagnosed with generalized granulomatosis with polyangiitis complicated with probable pulmonary tuberculosis, and was started on methylprednisolone and cyclophosphamide pulse therapy with anti-tuberculous treatment. She later developed cerebral vasculitis, indicating refractory disease, and was treated with second-line rituximab with excellent response. CONCLUSION: Proteinase 3 anti-neutrophil cytoplasmic antibody may be a valuable diagnostic marker in patients with atypical symptoms of granulomatosis with polyangiitis or in the presence of probable tuberculosis. Retinal vascular angiopathy needs to be diagnosed and treated early to prevent the development of complete blindness. Concomitant cytotoxic and anti-tuberculous treatments may be safe and effective in patients with simultaneous refractory disease with probable tuberculosis.

Seizures in an immunocompromised adolescent: a case report.

INTRODUCTION: Tuberculosis is a progressive and disabling infection predominantly seen in low-income and middle-income countries. Immunocompromised patients are at a higher risk of contracting tuberculosis than the healthy population. The presentation may also be atypical, leading to delay in diagnosis. We report the first case of tuberculous cerebral vasculitis presenting with epilepsia partialis continua. CASE PRESENTATION: A 17-year-old adolescent boy of Sri Lankan Moor heritage was taking long-term immunosuppressants for nephrotic syndrome. He presented to hospital with focal fits affecting his left arm. He later developed choreiform movements of the same arm, progressing to epilepsia partialis continua and weakness. The gradually evolving focal neurological signs and underlying immunosuppression raised the possibility of localized cerebral infection or inflammation. Analysis of his cerebrospinal fluid showed lymphocytosis with normal cellular morphology. Magnetic resonance imaging was suggestive of progressive vasculitic infarctions of the cerebral cortex and basal ganglia. There was no evidence of active autoimmune or viral disease on hematological investigations, but molecular amplification detected Mycobacterium tuberculosis in his cerebrospinal fluid. Although our patient had been established on isoniazid preventive treatment for eight months before the episode, tuberculosis was nonetheless considered to be the most likely cause of the cerebral vasculitis. He was treated with a trial of anti-tuberculosis treatment, including streptomycin and adjunctive steroids, and made an uneventful recovery. CONCLUSION: Clinicians should have a high index of suspicion for tuberculosis infection in patients with compromised immunity and other risk factors. The pathophysiological mechanisms underpinning cerebral vasculitis and epilepsia partialis continua are not completely understood. The efficacy of isoniazid prophylaxis in patients with immune suppression warrants further study. We present a regimen that successfully treated tuberculous cerebral vasculitis.

Acute myocardial infarction following a Russell's viper bite: a case report.

INTRODUCTION: Snake bite is a common and devastating environmental hazard, especially in rural areas of tropical countries. Acute myocardial infarction caused by snakebite has rarely been reported. To our knowledge we found only 10 cases of Myocardial infarction following a viper bite in English literature. CASE PRESENTATION: We report a case of inferior ST elevation myocardial infarction following a Russell's viper bite in a 37 year old healthy Sri Lankan (South Asian) female with no past history of cardiac disease or cardiac risk factors who died 30 hours following the bite. CONCLUSION: The course of events with respect to myocardium suggests a direct toxic effect of the venom on myocardial tissue or coronary vasoconstriction. Physicians should bear in mind the complications and devastating sequela of Myocardial infarction following Russell's viper bite.