The patient, a 55 yr old female with Type 2 diabetes mellitus for the last 14 years, on insulin for 2 years, pre-sented with the complaint of sudden onset weakness of all four limbs for five days. She had a preceding history of severe muscle cramps and paraesthesia for about a month. History revealed that the patient had severe generalised weakness twenty days back, for which she had been admitted in a local hospital and had improved symptomatically (though some amount of muscle pain persisted) with intravenous fluid and injectable medications, the details of which could not be gathered. The onset of quadriparesis was acute, noticed on getting up in the morning, five days prior to admission in our institute. There was no history of unusually high carbohy-drate intake, cold exposure, excessive exertion or alcohol intake the previous night. She denied unexplained sweating, tremors, heat intolerance, prolonged vomiting, or any drug intake over a prolonged period of time. General examination revealed carpopedal spasm, hypertension(160/100 mm Hg) and mild pallor. Neurological examination revealed bilater-ally symmetrical weakness (Gr 1/5) of all four limbs and generalized hyporeflexia. Muscles of the eyes, face, tongue, pharynx, larynx, diaphragm and sphincters were not involved. Higher functions, cranial nerves and sensory system were intact. Plantar was bilaterally flexor. Apart from mild hepatomegaly, systemic examination was normal.\nInvestigations revealed hypokalemia(1.3 mEq/l), hypocalcemia (corrected 5.8 mg/dl) and hypomagnesemia (1.1 mg/dl) with normal sodium and chloride levels. Routine blood examination was normal. Fasting and post prandial plasma glucose was 171mg/dl and 309mg/dl respectively with an HBA1c of 9.5%. Serum osmolality was normal.\nUrinary ketones were negative. Liver function tests were slightly deranged with AST of 71 mg/dl and ALT of 186 mg/dl. Serum protein was 7.09mg/dl, albumin - 3.2 mg/dl, globulin - 3.8mg/dl, serum bilirubin -0.5mg/dl. Serum creatinine was 1.l mg/dl and microalbuminuria was present. CPK and LDH were within normal limits. Thyroid profile was normal. Serum phosphorus was normal and PTH was 50.3 pg/ml(normal–10 to 55 pg/ml). 24 hour urinary calcium was 61mg (normal - 100 to 300mg/24hrs). Urinary potas-sium excretion was 53 mEq/L /day (normal-25-120 mEq/L/day). Blood gas analysis revealed metabolic alkalosis. Electrocardiogram, ultrasound abdomen, plain picture abdomen, X-ray cervical spine and chest X-ray were normal. MRI of brain, spine and abdomen were also normal.\nInitially, the patient was treated with injectable potas-sium, magnesium and calcium preparations. Normalization of serum potassium and calcium was achieved only after total correction of magnesium level. There was gradual improve-ment in muscle power after 24 hours of therapy. Complete recovery was achieved after five days of treatment, following which she was shifted to oral preparations. Glycemic control was achieved using insulin and hypertension was controlled with Amlodepin 5 mg. She was discharged after two weeks on oral calcium(l gm daily) and advised to take diet rich in potassium and magnesium. On subsequent follow up at one and three months, she was asymptomatic with normal electrolyte levels and good glycemic control.
A 26-year-old female with B. cenocepecia presented with a history of recurrent small joint pains in association with intermittent painful erythematous nodular rash on the lower legs. This came on at the onset of an infective exacerbation of her chest. Biopsy confirmed erythema nodosum. Other causes of erythema nodosum were excluded, and she did not respond to Non-steroidal anti-inflammatory medications. Her symptoms continue to relapse in association with infective exacerbations of her chest.\nOf the remaining patients (n = 3), one presented with an erythematous rash on the lower legs and another had a purpuric rash on the lower legs and both were diagnosed clinically as leucocytoclastic vasculitis by a dermatologist. The third patient presented with purpuric areas and blistering of the lower legs which was confirmed as leucocytoclastic vasculitis on biopsy.\nOverall, two patients had biopsy proven leucocytoclastic vasculitis, three were clinically diagnosed with vasculitis but did not have biopsy confirmation and two had biopsies, which revealed other pathologies (erythema nodosum and capillaritis), giving an overall prevalence of cutaneous vasculitis in patients with Burkholderia cepacia complex of 16.7%. Out of the five patients with vasculitis, three had a raised ESR/CRP ratio.\nThere was no evidence of renal involvement in any patient – all had a negative urine dipstick and normal serum creatinine. The antinuclear antibody and antineutrophil cytoplasmic antibody were negative in all patients apart from one with biopsy proven leucocytoclastic vasculitis who had a transiently positive antineutrophil cytoplasmic antibody. Antistreptolysin O titre was tested in only two out of the five patients and was negative.\nMean forced expiratory volume1 was 30% (range: 18–55) of the predicted value. In four patients (57%), the forced expiratory volume1 was lower at the time of presentation with rash compared to the baseline forced expiratory volume1 values for the previous two years.\nAll patients received antibiotics for a presumed pulmonary exacerbation at onset of the rash. Three patients received specific treatment for vasculitis (prednisolone ± additional immunosuppressive) after non-resolution of the rash.