Age: 44.0
Sex: F
Vaccine: HEP
Symptoms: Dysphagia, Fatigue, Headache, Hypotonic urinary bladder, Influenza like illness
Clinical narrative: It was reported, in the above stated article, that a 44 year old female nurse with a history of TB exposure, uterine dilation and curettage in 1996 followed by a blood transfusion who in 1989 was vaccinated with hep B vaccine recombinant. Subsequently over a two year period following the birth of her son, experienced diffuse painful myalgias, weakness of the upper extremities, weakness of the lower extremities (proximal >distal), dysphagia, lower back spasms and arthralgias, all of a progressive nature. Symptoms began 3 to 4 weeks post-partum and were accompanied by flu-like symptoms, followed by excessive fatigue and polyarthralgias. The pt had intermittent cephalgia thereafter. Lab evaluation before neurologic exam revealed elevated creatine phosphokinase CK at 1675 IU/L with an aldolase of 13. The neurological examination revealed normal cerebral, cerebellar and cranial nerve function. Sensory examination was intact. Motor examination revealed decreased voluntary effort secondary to pain, but with encouragement, nearly normal strength. Repetitive muscle testing revealed fatigability. There was no apparent atrophy or fasciculations. Deep tendon reflexes were 2+. Nerve conduction studies of the left upper and lower extremities were normal, as were repetitive nerve stimulation. Needle examination showed fibrillation potentials and positive waves of the left deltoid with rapidly recruitable, small abundant potentials. Left quadriceps revealed no abnormal spontaneous activity but myopathic appearing untis. Light microscopy revealed dense, focal and multifocal aggragates of large, non-epithelioid histiocytes with fine PAS-positive granules in SC ffat, epimysium, perimysium, and endomysium. The infiltrate appeared as thick epimysial covering, perifascicularly, and more often with centripetal distribution fromt he epimysium and endomysium. Scattered in a few of the aggragates were rare lymphocytes and plasma cells intermixed with histiocytes. Langerhans cell markers CD1a and S100 protein were negative, therefore the major cell type within the aggrate was macrophages. No organisms were noted in the acid-fast stain. PCR analysis for Tropheryma whippelii, Myco baccterium tuberculosis complex and Myco bacterium avium complex was negative. Stains and measurements for the presence of alumimum were negative. Electron microscopy revealed bistiocytes with intracytoplasmic accumulations of fine-needle shaped, randomly oriented dense structures, often bounded by distinct limiting membranes that resembled aparite crystals. No extracellular accumulations were found, and no bacteria or viral particles were present. Because of the complaints of cephalgia, MRI of the brain was done and revealed numerous small patchy signal abnormalities involving supratentorial white matter on the multi-echo and flair sequences. These lesions were more peripheral than central and not associated with mass effect or pathologic contrast enhancement. Abnormal lab tests included, Interleukin-2 (IL-2) was425 (normal range, 25-233 pg/mL), and tumor necrosis factor (TNF) was 25 (normal, 0-10 pg/mL). Cardiovascular evaluation included a normal 24 hr Holter Monitor and electrocardiogram, which revealed thickened aortic and mitral leaflets, as well as left ventricular hypertrophy. The pt was started on high-dose preddnisone (650mg per day) and within 2 weeks had significant improvement. After 2 months, an attempt at reduction in dose caused a return of symptoms. After another month, because of weight gain, the prednisone was reduced to every other day but the continuation of symptoms required additional methotrexate 10 mg per day. The prednisone was reduced to 20mg every other day. The pt's endurance, arthralgias, myalgias, and dysphagia were greatly improved although she continued to have some proximal muscle weakness. The pt remained clinically stable over the next 6 months and continued on the prednisone every other day along with methotraxate daily. CK was reduced at 270IU/L. The MRI of the brain was unchanged following treatment. The pt had multiple hyperintense signal abnormalities in the supratentorial white matter. Despite these findings, she had no clinical evidence of central nervous system involvement. She had been aggressively treated with prednisone and methotrexate, and it was possible that this treatment may have precluded central nervous system manifestation of her white matter pathology. Another unusual finding in this pt's case is the apparent cardiac involvement with the thickening of the aortic and mitral valves, which is found in systemic histiocytosis. Elevated IL-2 and TNF can also be found in systemic histiocytosis. By absence of aluminum in the muscle biopsy, the pt's macrophagic myofascitis could not be attributed to a vaccine reaction, as were a number of cases. Her disorder appears rather to represent systemic histiocytosis of unknown etiology. Improvement with prednisone and methotrexate was encouraging but not complete. Upon internal review, the pt's experience were considered to be important medical events (OMIC). Additional info has been requested. The follow up received on 2/20/03 states the author indicates that the article did not occur as a result of the hep B vaccine recombinant vaccine. It was reported that the pt had the vaccine many years prior to the onset of symptoms. Additional infois not expected.