JBJS | Parrot Pseudoparalysis of the Upper Extremities. A Case Report*

The Journal of Bone & Joint Surgery, Volume 78, Issue 2

Articles | February 01, 1996

Parrot Pseudoparalysis of the Upper Extremities. A Case Report*

MININDER S. KOCHER, M.D.†; MIGUELA CANIZA, M.D.‡, DURHAM, NORTH CAROLINA

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Investigation performed at Duke University Medical Center, Durham

The Journal of Bone & Joint Surgery. 1996; 78:284-7

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Introduction

Introduction | Case Report | Discussion | References

The incidence of congenital syphilis in the United States has been increasing in epidemic proportions since 1986 and is currently the highest since the introduction of penicillin for the treatment of syphilis in the early 1950's^17,37. This increase represents both a rise in the actual number of patients who have congenital syphilis and a broadening of the definition of a case, by the Centers for Disease Control in 1988, to include syphilitic stillbirths and presumptive infections^6,33,36. The incidence of congenital syphilis has paralleled the recent increase in the incidence of primary and secondary acquired syphilis in adults^{5,17,20-22,25,26}.

Clinical manifestations of congenital syphilis are multisystemic, non-specific, and variable in presentation. Skeletal involvement is common, especially in symptomatic infants, and can occasionally be the presenting symptom^{11,27,29,31,34}. This report details the case of an infant with congenital syphilis in whom the chief presenting symptoms were swelling and paralysis of the upper extremities. Originally described by Parrot²³ in 1871, this pseudoparalysis represents decreased movement of the extremity secondary to painful syphilitic periostitis. The clinical presentation, osseous involvement, laboratory testing, and current recommendations for treatment of congenital syphilis are briefly reviewed.

*No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. No funds were received in support of this study.

†Department of Orthopaedic Surgery, Harvard Medical School, Massachusetts General Hospital, Gray 6, Fruit Street, Boston, Massachusetts 02114.

‡Division of Pediatric Infectious Disease, Duke University Medical Center, Durham, North Carolina 27710.

*No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. No funds were received in support of this study.

†Department of Orthopaedic Surgery, Harvard Medical School, Massachusetts General Hospital, Gray 6, Fruit Street, Boston, Massachusetts 02114.

‡Division of Pediatric Infectious Disease, Duke University Medical Center, Durham, North Carolina 27710.

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Radiographs of the left and right upper extremities, made when the infant was four weeks old, demonstrating metaphyseal erosions of the distal part of the humerus and the proximal part of the ulna. In addition, there was periosteal reaction of the humerus, ulna, and radius.

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Case Report

Introduction | Case Report | Discussion | References

A four-week-old black male infant was admitted to the pediatric neurology service at Duke University Medical Center for evaluation of flaccid paralysis of the upper extremities. The infant weighed 2102 grams at birth, which was after a thirty-six-week gestation. The mother was twenty-three years old, single, black, gravida one, and para zero. During the last trimester of the pregnancy, she had had a two-week febrile illness characterized by malaise and a pruritic, macular rash, with subsequent desquamation, on the palms and soles. Laboratory evaluation at the first prenatal visit revealed negative findings for rapid plasma reagin, hepatitis-B surface antigen, and the human immunodeficiency virus. The test for the sickle-cell trait was positive, and the patient had rubella immune status. The infant was born through a normal, spontaneous vaginal delivery, without complications, and had an Apgar score of nine at one minute and at five minutes. The neonatal course was unremarkable except for brief neonatal jaundice that was treated with phototherapy.

The newborn was healthy and vigorous during the first two weeks of life, and there was no history of trauma in this period. The mother noted that the infant had decreased movement of both upper extremities starting at the age of two weeks, swelling of the forearm bilaterally at the age of three weeks, and complete lack of movement of the upper extremities by the age of four weeks. At that time, the mother brought the infant to a local hospital, where a physical examination revealed flaccid paralysis of both upper extremities, with decreased tone and proximal muscle atrophy. Reflexes were reportedly absent bilaterally, and the infant cried when the upper extremities were manipulated. Laboratory evaluation revealed elevated levels of lactate dehydrogenase (737 units per liter [12.29 microkatals per liter]) and alkaline phosphatase (567 units per liter [9.5 microkatals per liter]). Radiographs of the shoulders and humeri revealed no fractures. Magnetic resonance images of the brain and cervical spine revealed findings within normal limits.

The infant was transferred to Duke University Medical Center, where a physical examination revealed that he was alert and vigorous and had no dysmorphic features. Relative to his age, his weight was in the tenth percentile and his length was in the twenty-fifth percentile. Hepatosplenomegaly was apparent on abdominal examination. Cranial nerves II through XII were found to be intact. The upper extremities hung, flaccid and extended, at the sides, and the hands were held half open. There was swelling of both forearms and atrophy of the proximal muscles of the upper limbs. On examination for motor function, the strength of the deltoid, biceps, triceps, forearm flexors, forearm extensors, and hand-grip muscles was grade 2 of 5 bilaterally. On examination for sensory function, the infant withdrew on painful stimuli to the lower extremities but merely cried without any movement on painful stimuli to the upper extremities. The reflexes, tested while the infant was asleep, were grade 2+ of 3 throughout. (A grade of 1 meant hyporeflexic; 2, normoreflexic; and 3, hyperreflexic.) He demonstrated normal rooting, corneal, gag, and step reflexes. No spontaneous movements of the upper extremities were observed. He had a full passive range of motion, but he cried when the upper extremities were manipulated. Laboratory evaluation also showed elevated levels of lactate hydrogenase (832 units per liter [13.87 microkatals per liter]), alkaline phosphatase (559 units per liter [9.3 microkatals per liter]), and aldolase (12.9 units per liter [215 nanokatals per liter]). The findings of a complete blood-cell count, serum chemistry studies, coagulation studies, hemoglobin electrophoresis, evaluation of the level of creatinine kinase, and cultures of blood for bacterial growth were within normal limits. Radiographs of both upper extremities revealed periosteal elevation of the humerus, radius, and ulna bilaterally (Figs. 1-A and 1-B). In addition, there were metaphyseal erosions of the distal part of the humerus and the proximal part of the ulna.

As the clinical presentation was suggestive of congenital or neonatal infection, rapid plasma reagin tests were performed for both the infant and the mother and were found to be positively reactive at a dilution of 1:64. The diagnosis was confirmed when the infant was found to be seropositive for fluorescent treponemal antibody. The findings of a lumbar puncture ruled out neurosyphilis, and enzyme-linked immunosorbent assay testing ruled out infection with human immunodeficiency virus. The patient was managed intravenously with 200,000 units of crystalline penicillin G per kilogram of body weight per day, divided into four doses daily, for ten days. At the two-year follow-up examination, the patient had had normal growth without any apparent skeletal abnormalities. The weight was in the thirty-fifth percentile, and the height was in the forty-fifth percentile. He was seronegative for rapid plasma reagin and seropositive for fluorescent treponemal antibody.

Discussion

Introduction | Case Report | Discussion | References

The causative agent of syphilis is Treponema pallidum, a spirochete that is transmitted either through direct contact with denuded epithelial lesions or through transplacental infection of the developing fetus. Congenital infection can occur during any clinical stage of maternal disease and during any stage of pregnancy, although fetal damage is rare before the eighteenth week of gestation^10,13. The extent of the clinical manifestations of congenital syphilis is extremely variable². Stillbirths represent the most severely affected infants and account for 30 to 40 per cent of fetuses with congenital syphilis^2,4,14. However, as many as two-thirds of newborns (632 in a series of 941) are free of symptoms at birth and are seen later with postnatal manifestations^7,14.

Skeletal manifestations of congenital syphilis were recognized in the 1800's, even before the causative agent of syphilis was identified in 1905¹⁴. In 1871, Parrot described several children in whom pain in the extremities secondary to syphilitic involvement of bone resulted in a lack of movement²³. This condition has come to be known as pseudoparalysis of Parrot. In a report of eighteen infants who had congenital syphilis, Fleming and Bardenstein reported that three had pseudoparalysis¹¹. Although the characteristic rash, rhinitis, and hepatosplenomegaly are common presenting symptoms of congenital syphilis, musculoskeletal involvement can occasionally be the principal finding. In a review of the cases of 302 infants who had congenital syphilis, Rasool and Govender found that thirty-four (11 per cent) had primary musculoskeletal symptoms, including swelling of the joints (fifteen infants), pathological fractures (twelve infants), pseudoparalysis (six infants), and pain in the leg (one infant)²⁴. Skeletal manifestations of congenital syphilis usually include metaphysitis, osteitis, and periostitis¹⁹. These changes are often present at birth, but they may not appear until the infant becomes symptomatic. Autopsies of syphilitic stillborn fetuses have demonstrated the presence of spirochetes in bone³². Involvement is usually evident at sites of active proliferation and endochondral ossification, especially in the metaphyseal region of long bones^15,18,19,34. The femur, tibia, and humerus are often involved, although any bone may be affected. Polyostotic, bilateral, symmetrical involvement is the rule, although unilateral involvement has been reported^9,24,28. In a review of the findings in 107 patients who had congenital syphilis with radiographic changes, Rosen and Solomon reported that each had involvement of multiple bones²⁷.

Radiographic changes are present in approximately 95 per cent of infants (107 of 112 infants in the series of Rosen and Solomon²⁷) who have overt clinical signs of disease and approximately 20 per cent of infants (eighteen of ninety-three infants in the series of Greenberg and Bernal¹²) who have positive serological findings but no clinical signs of disease⁸. Because most other clinical findings in congenital syphilis are less specific and the morbidity is so severe, the Centers for Disease Control recommend that radiographs of the long bones be included in the evaluation of all infants who are suspected of having congenital syphilis^3,8. The most common radiographic presentation of congenital syphilis is a combination of metaphyseal changes and periosteal reaction that is bilateral, symmetrical, and polyostotic^24,27,30. The earliest radiographic changes occur in the metaphysis and result from an interplay between bone reaction and bone destruction. Reactive changes manifest as transverse, saw-tooth, dense bands from altered mineralization at the zone of provisional calcification. Frank destruction of bone appears as focal areas of erosion and rarefaction and is thought to result from direct destruction by syphilitic granulation tissue¹⁸. In particular, focal erosion of the medial aspect of the proximal tibial metaphysis occurs commonly and is known as the Wimberger sign²⁴. Periosteal thickening often occurs in association with diffuse osteochondritis and presents as multiple, irregular layers of periosteal new-bone formation. Other radiographic findings of congenital syphilis include changes in the joints, pathological fractures, and epiphyseal separation. The differential diagnosis of such erosive lesions in an infant includes battered-baby syndrome, chronic sub-acute osteomyelitis, neonatal leukemia, neuroblastoma, cytomegalovirus, rubella, and rickets. The signs of late congenital syphilis are often a result of scarring from this early osseous involvement. Osseous changes of the teeth are common. Characteristic notching of the incisors is known as Hutchinson teeth, and maldevelopment of the first molars is known as mulberry molars. Other osseous signs of late congenital syphilis include saber shins, frontal bossing, saddle nose, and perforation of the hard palate. A remarkable feature of the osseous involvement in congenital syphilis is that treatment often results in complete healing with normal growth and an absence of radiographic changes^16,24.

The diagnosis of syphilis is made either by direct detection of treponemes on dark-field examination of appropriate specimens or by serological detection of antibodies formed in response to treponemal disease. Diagnostic evaluation of an infant with suspected congenital syphilis should include a non-treponemal antibody test (rapid plasma reagin) for screening followed by a specific fluorescent treponemal antibody test for confirmation. Once the diagnosis has been made, the workup should include radiographs of the long bones to assess osseous involvement, a lumbar puncture with serological evaluation of cerebrospinal fluid to rule out the possibility of neurosyphilis, and enzyme-linked immunosorbent assay testing to rule out a concomitant infection with human immunodeficiency virus.

Treponema pallidum is exquisitely sensitive to penicillin, with no evidence of resistance. The Centers for Disease Control and the American Academy of Pediatrics recommend that all patients who have confirmed or presumptive congenital syphilis be managed with intravenous administration of 100,000 to 150,000 units of crystalline penicillin G per kilogram of body weight per day, in divided doses every nine to twelve hours, for ten to fourteen days^3,37. This recommendation is based on reports in which treatment with intramuscular administration of a single dose of 50,000 units of benzathine penicillin G failed in as many as 3 per cent of patients^1,35. Periodic follow-up, with serological testing of all infants and treatment of any infected individuals with whom the mother has had sexual contact, is imperative to ensure adequate therapy. Prenatal screening remains the pivotal factor in the prevention of congenital syphilis because it ensures that pregnant women who have syphilis are diagnosed and treated. Routine serological testing for syphilis is legally required at the beginning of prenatal care in all states, and additional testing at the beginning of the third trimester and at delivery is indicated for women in high-risk populations. Because of inadequate prenatal screening, some states now require screening of newborns for congenital syphilis before discharge from the nursery. Screening at the first prenatal visit was ineffective in the present case as the mother apparently became reinfected during pregnancy and, in fact, demonstrated symptoms of secondary syphilis during the third trimester. Routine maternal screening at delivery or screening of the newborn in the neonatal period would have revealed congenital syphilis.

References

Introduction | Case Report | Discussion | References

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