Case Reports

Indian Pediatrics 1999;36: 1154-1157

Palatal Nerve Palsy and Cervical Adenopathy in a Probable Case with Cat Scratch Disease

From the Division of Pediatric Hematology-Oncology, Advanced Pediatric Center and Department of Pathology*, Postgraduate Institute of Medical Education and Research, Chandigarh 160 012, India.
Reprint requests: Dr. R.K. Marwaha, Additional Professor, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh 160 012, India. Fax: +91-0172-540401
Manuscript Received: December 29, 1998;
Initial review completed: February 2, 1999;
Revision Accepted: June 8, 1999

Cat scratch disease (CSD) was first observed by Debre et al. in 1930 and was designated as `La maladie des griffes de chat' in the 1950's(1). It is a worldwide disease, occurring in all races with a slight predilection for males. It is usually a benign, self-limiting disease and is recognized as one of the more common causes for unilateral regional lymphadenitis in children(2). However, CSD has, occasionally, caused neurological complications such as encephalopathy and transient dysfunction of cranial and/or peripheral nerves(3,4). The relative rarity of the condition prompted us to document the problems faced in establishing a diagnosis of CSD in a young boy who presented with palatal palsy and cervical adenopathy.

Case Report

An 11-year-old boy presented with a history of difficulty in swallowing with nasal regurgitation for 10 days prior to admission. He also complained of a nasal intonation. There was no history of fever, sore throat, headache, vomiting or weakness in any of his limbs.

Examination revealed a well nourished, afebrile and nontoxic child. There were no pus points or membrane over the tonsils. He had two, firm to hard jugulodigastric nodes measuring 1 to 1.5 cm, on the right side. These were non-tender, mobile and not matted. Apart from a bilateral palatal palsy, there was no other neurodeficit. The other systems were unremarkable.

The possibility of a brainstem glioma was entertained. A MRI of the brain did not reveal any pathology. On the third day of admission, he developed moderate grade intermittent fever which persisted for almost a month after the onset. During this period the jugulodigastric nodes progressively increased in size. A fluctuant mass developed in the right submandibular region and prompted a possibility of suppurative cervical adenopathy. Investigations revealed a raised erythrocyte sedimentation rate (60 mm in first hour). Throat swab culture was sterile and Albert stain was negative. Aspiration of the submandibular swelling drew pus. The Gram stain showed no organisms and the culture was sterile. The lymph nodal swelling persisted inspite of a 10 day course of Cloxacillin and Gentamicin. Fine needle aspiration cytology (FNAC), done to exclude a malignant disorder, revealed reactive lymphoid hyperplasia. A normal X-ray chest and a negative tuberculin test (1 TU) excluded a tuberculous etiology. A lymph node biopsy was then done. Histopathological examination showed multiple areas of necrosis surrounded by histiocytes and occasional multinucleated cells (Fig. 1) There was a prominent eosino-philic infiltration. In addition to the necrotic areas, poorly formed granulomas were seen. A diagnosis of necrotizing granulomatous lymph-adenitis consistent with cat scratch disease was suggested. The organism could, however, not be demonstrated by Warthin Starry silver stain. Acid fast and periodic acid Schiff stains were negative for mycobacteria and fungi.

Fig. 1. Low magnification view (hematoxylin and eosin ´ 120) of an abscess showing necrotic area with pallisading histiocytes forming the border.

The child was discharged about 3 weeks after hospitalization, by which time palatal palsy had recovered spontaneously. He became afebrile about 2 weeks after discharge whilst the lymph nodes regressed completely in 8 to 12 weeks. Repeated enquiries failed to elicit a history of contact with cats.

Discussion

CSD was first described more than 40 years ago. Several infectious organisms have been proposed as causative agents. Afipia felis, a Gram negative bacillus was demonstrated by Warthin-Starry stain in tissue specimens. More recent evidence implicates Rochalimae henselae as the most frequent organism responsible for CSD(5) which is also known as Bartonella henselae.

CSD occurs more frequently in children. Carithers observed that 87% of the patients were  less than 18 years of age. A history of contact with cats was reported to be present in 90 to 99% cases(6). The disease is also believed to follow contact with other animals (e.g., like dogs, rabbits) or inanimate objects like wood splinters and fish bones. A history of contact with cats was not forthcoming in our case. It is possible that the disease occurred following contact with other animals.

The site of inoculation is identified in 50-76% of cases(5,7). This was not specifically looked for in our case as CSD was not considered as a possibility initially.

CSD is classically described as a benign self-limiting illness presenting with regional lymphadenopathy. Single nodal group involve-ment was found in 85% of the cases reported by Carithers(6). The distribution of nodal involvement was 46.1% in axillary and epitrochlear regions, 26.1% in the neck and jaw areas, 17.5% in the groin and 6.6% in the preauricular region(6). The lymphadenopathy usually resolved in 2 to 4 months. However, the time of resolution could vary from 2 weeks to 2 years(2). In our case, the right submandibular lymphnodes were involved, with spontaneous resolution occurring in about 3 months.

CSD may, sometimes, be a severe, protracted illness which may require costly, painful diagnostic evaluation because of a broad spectrum of differential diagnosis and lack of a reliable diagnostic test. Atypical manifesta-tions in normal hosts are uncommon and are described in only 5-10% of the cases. These include Parinaud's oculoglandular syndrome, encephalopathy, peripheral neuritis, neuroreti-nitis, thrombocytopenic purpura, erythema nodosum, erythema marginatum, urethritis, granulomatous involvement of liver and spleen, osteitis-osteomyelitis, pneumonia, pleural effu-sion and fever of unknown origin(8-10).

Transient dysfunction of cranial and/or peripheral nerves occurred in 15 out of 1471 patients with CSD observed by Margileth over a period of 15 years. There were 2 children, aged 18 months and 6 years respectively, who had temporary facial nerve paralysis, associated with CSD oculoglandular syndrome, lasting for 4-5 weeks. Three women had evidence of peripheral neuritis. The remaining ten patients, of whom 2 were children, had neuroretinitis. All of them recovered slowly within 4-12 months(3). The propositus presented with palatal palsy which has, hitherto, not been described in association with CSD. The nerve palsy showed spontaneous recovery over a period of about 3 weeks.

Laboratory results are typically normal with an exception of an elevated erythrocyte sedimentation rate, as was seen in the index case(8). Although there is no single test to conclusively diagnose CSD, a reliable diagnosis can be based on historical and clinical findings. A clinical diagnosis of CSD can be made when three of the following four criteria are met: (i) contact with cats in conjunction with the primary dermal lesion, (ii) positive CSD skin test, (iii) negative laboratory results for other  causes of lymphadenopathy and (iv) characteris-tic histologic features in a biopsy specimen of either the skin lesion or enlarged lymph node(2). In our patient, a history of contact with cats was not forthcoming. The inoculation site was not looked for particularly. An absence of contact with cats should not negate a diagnosis of CSD as the history of contact may be forgotten or there may be contact with an object with which a kitten was in recent contact(6). No other cause of palatal palsy and cervical adenopathy could be detected. The course of the illness with spontaneous recovery over a period of 3 months are features which are characteristics of the disease.

The skin test for CSD is done with the pus obtained via a pasteurization process, from patients with CSD. Even though it is considered reliable and highly specific, this mode of antigen testing is currently unlicensed because of the potential for transmission of HIV(11).

Cytologic examination of specimens from patients with CSD reveal findings which vary with the stage of the disease. Early lesions show reactive follicular hyperplasia with proliferation of lymphoid elements caused by microabscesses which usually form near or within germinal centers. More mature lesions have characteristic stellate granulomas with suppurative centers, usually without evidence of caseation(2). In our case, histopathological evidence of the disease was found in the affected right-sided sub-mandibular lymph node.

Serological testing for the presence of antibodies to B. henselae is widely used for the laboratory confirmation of the diagnosis of CSD. Immunofluorescent antibody (IFA) and ELISA IgM tests are significantly specific and sensitive whilst ELISA IgG test has lesser sensitivity(12). Besides these, the PCR test for detecting B. henselae specific DNA sequences is the most sensitive test available(12). Lack of availability precluded these investigations in our patient.

In the majority of patients with CSD, no therapy is required. Conservative management of CSD consists of careful observation over several weeks (usually 8 to 24 weeks) during which spontaneous involution of the lymphadenopathy should occur. For individuals with complications or more severe manifesta- tions of CSD, antibiotic therapy is indicated. In a restrospective assessment of uncontrolled data, Margileth reported the efficacy of rifampin (87%), ciprofloxacin (84%), trimethoprim/sulfamethoxazole (58%) and parenteral genta-micin sulfate (73%) in improving clinical symptoms and shortening the clinical course(12).

The propositus serves to illustrate the diagnostic difficulties faced in establishing a diagnosis of CSD, which was ultimately made on histopathological examination of a lymph node biopsy. The presentation of unilateral lymphadenopathy with a self-limiting cranial nerve palsy, although typical of CSD, did not arouse this clinical suspicion because of the relative rarity of the condition in our country. The diagnostic path was, therefore, tortuous. A diagnosis of CSD needs to be entertained in a child who presents with unilateral adenopathy with or without an associated cranial nerve palsy.

Acknowledgements

The authors acknowledge with gratitude the contribution rendered by Dr. Pratibha Singhi and Dr. Debabrata Ghosh, who were associated with the management of the patient in the early part of his hospitalization.

References

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11. Regnery RL, Olson JG, Perkins BA, Bipp W. Serological response to "Rochalimaea henselae" antigen in suspected cat scratch disease. Lancet 1992; 339: 1443-1445.

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13. Margileth AM. Antibiotic therapy for cat scratch disease: Clinical study of therapeutic outcome in 268 patients and a review of the literature. Pediatr Infect Dis J 1992; 11: 474-478.