Volume 87 Number 5
Letters to the Editor
2. Hansen OE, and Lund MA: Akut cerebellar ataksi ved infektios mononukleose, Nord Med 77:153, 1967. 3. Gilbert JW, and Culebras A: Cerebellitis in infectious mononucleosis, JAMA 220"727, 1972. 4. Bennett DR, and Peters HA: Acute cerebellar syndrome secondary to infectious mononucleosis in a fifty-two year old man, Ann Intern Med 55:147, i961. 5. Hoyne RM: Involvement of the central nervous system in infectious mononucleosis, Arch Neurol Psychiat 63:606, 19501 6. Schoenberger HB: Cerebellar ataxia associated with infectious mononucleosis, J Am Coll Health Assoc 14:213, 1966.
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demonstrate any impairment in the i.lnmunologic functions of neutrophils or lymphocytes from our patient. The observation that these patients have recurrent and severe infections remains, but the basis for their altered resistance to infection is still unanswered. Thomas A. Fleisher, M.D. J. Nevin Isenberg, M.D., Ph.D. Harvey L. Sharp, M.D. Gastroenterology Section Department o f Pediatrics University of Minnesota Minneapolis, Minn~ 55455 REFERENCES
Leukocyte function in aspartylglucosaminuria To the Editor: We have recently described a 5-year-old girl with aspartylglucosaminuria (AGU), a metabolic defect in glycoprotein catabolism resulting from a deficiency of the lysosomal enzyme, aspartylglucosamine amido hydrolase. 1 Clinically, patients with this disorder have been, noted to have recurrent respiratory infections and a variety of other purulent lesions. ~ A theory forwarded to explain this altered resistance to infection proposes that the abnormality in lysosomal enzyme content leads to a secondary disturbance in the leukocyte functions related to the immunologic defense mechanism. 2 We have shown that the ultrastructural morphology of the lysosomes of neutrophils and lymphocytes in this disease is altered with respect to size and matrix content) In order to critically examine this hypothesis, an evaluation of neutrophil and lymphocyte function wa s performed on ce!ls prepared from our patient. LABORATORY
STUDIES
The leukocyte counts and differentials were within normal limits on all testings. In v!yo leukocyte migrations as measured by the skin window technique was interpreted as normal (Dr. Arthur Page). In vitro neutrophil chemotaxis, and phagocytosis and killing of Staphlococcus aureus 502A and Escherichia coli K12 were normal (Dr. Paul Quie's laboratory). In addition, the serum ops0nic activity to the above bacteria was normal. Lymphocyte function was studied in vivo with skin tests which yielded weak responses to SKSD and Candida. In vitro lymphocyte evaluation revealed normal numbers of "B" lymphocytes and "T" lymphocytes. Functional lymphocyte evaluation included normal serum immunoglobulin levels (IgG, IgM, IgA, IgE), normal hemagglutination titers, normal antibody response tO diphtheria and tetanus toxoid, and normal mitogenic stimulation (PHA and Con A). Thus, despite the morphologic abnormalities noted in the lysosomes of lcukocytes in AGU, we have been unable to
1. Isenberg JN and Sharp HL: Aspartylglucosaminuria: psychomotor retardation masquerading as a mucopolysaccharidosis, J PEDXATR86:713, 1975. 2. Autio S: Aspartylglucosaminuria-analysis of thirty-four patients, J Ment Defic Res, Monograph Series I, 1972. 3. Isenberg JN, and Sharp HL: Aspartylglucosam~nuria: Biochemical and ultrastructural characteristics unique to this visceral storage disease, Human Pathol (in press).
Hypolycorrhachia in young infants with viral meningitis To the Editor: From June through August, 1973, 36 children with aseptic meningitis were hospitalized at the Children's Hospital of Philadelphia. An attempt at virus isolation was made with specimens from 24 patients. Positive viral isolates were recovered from 14 patients, 8 of which were coxsackievirus B-2. Virus was isolated from the cerebrospinal fluid ( C S F ) i n three instances, and from the pharynx, throat, and rectum in eleven instances. Alihough acute and convalescent serum were not available from all of the patients studied, paired serum specimens of 12 patients from Philadelphia with aseptic meningitis were submitted to the Pennsylvania State Laboratory: six showed four-fold antibody titer rises to Coxsackie virus B - 2 or high-titer neutralizing antibodies in both specimens. Furthermore, Coxsackie virus B-2 was the strain responsible for most of the cases of aseptic meningitis from the Philadelphia area reporte d to the State Laboratory during the summer of 19731 A striking feature of the outbreak was the age distribution of the patients. Half of the infants affected were less than 2 months old, and more than three-fourths of those affected were less than 2 1/2 years old. Another impressive feature was the degree of hypoglycorrhachia. In one-third of the patients studied, the CSF glucose concentration was less than 50% of a simultaneously obtained blood glucose, and in more than three-fourths of the patients, the
Letters to the Editor
2. Hansen OE, and Lund MA: Akut cerebellar ataksi ved infektios mononukleose, Nord Med 77:153, 1967. 3. Gilbert JW, and Culebras A: Cerebellitis in infectious mononucleosis, JAMA 220"727, 1972. 4. Bennett DR, and Peters HA: Acute cerebellar syndrome secondary to infectious mononucleosis in a fifty-two year old man, Ann Intern Med 55:147, i961. 5. Hoyne RM: Involvement of the central nervous system in infectious mononucleosis, Arch Neurol Psychiat 63:606, 19501 6. Schoenberger HB: Cerebellar ataxia associated with infectious mononucleosis, J Am Coll Health Assoc 14:213, 1966.
833
demonstrate any impairment in the i.lnmunologic functions of neutrophils or lymphocytes from our patient. The observation that these patients have recurrent and severe infections remains, but the basis for their altered resistance to infection is still unanswered. Thomas A. Fleisher, M.D. J. Nevin Isenberg, M.D., Ph.D. Harvey L. Sharp, M.D. Gastroenterology Section Department o f Pediatrics University of Minnesota Minneapolis, Minn~ 55455 REFERENCES
Leukocyte function in aspartylglucosaminuria To the Editor: We have recently described a 5-year-old girl with aspartylglucosaminuria (AGU), a metabolic defect in glycoprotein catabolism resulting from a deficiency of the lysosomal enzyme, aspartylglucosamine amido hydrolase. 1 Clinically, patients with this disorder have been, noted to have recurrent respiratory infections and a variety of other purulent lesions. ~ A theory forwarded to explain this altered resistance to infection proposes that the abnormality in lysosomal enzyme content leads to a secondary disturbance in the leukocyte functions related to the immunologic defense mechanism. 2 We have shown that the ultrastructural morphology of the lysosomes of neutrophils and lymphocytes in this disease is altered with respect to size and matrix content) In order to critically examine this hypothesis, an evaluation of neutrophil and lymphocyte function wa s performed on ce!ls prepared from our patient. LABORATORY
STUDIES
The leukocyte counts and differentials were within normal limits on all testings. In v!yo leukocyte migrations as measured by the skin window technique was interpreted as normal (Dr. Arthur Page). In vitro neutrophil chemotaxis, and phagocytosis and killing of Staphlococcus aureus 502A and Escherichia coli K12 were normal (Dr. Paul Quie's laboratory). In addition, the serum ops0nic activity to the above bacteria was normal. Lymphocyte function was studied in vivo with skin tests which yielded weak responses to SKSD and Candida. In vitro lymphocyte evaluation revealed normal numbers of "B" lymphocytes and "T" lymphocytes. Functional lymphocyte evaluation included normal serum immunoglobulin levels (IgG, IgM, IgA, IgE), normal hemagglutination titers, normal antibody response tO diphtheria and tetanus toxoid, and normal mitogenic stimulation (PHA and Con A). Thus, despite the morphologic abnormalities noted in the lysosomes of lcukocytes in AGU, we have been unable to
1. Isenberg JN and Sharp HL: Aspartylglucosaminuria: psychomotor retardation masquerading as a mucopolysaccharidosis, J PEDXATR86:713, 1975. 2. Autio S: Aspartylglucosaminuria-analysis of thirty-four patients, J Ment Defic Res, Monograph Series I, 1972. 3. Isenberg JN, and Sharp HL: Aspartylglucosam~nuria: Biochemical and ultrastructural characteristics unique to this visceral storage disease, Human Pathol (in press).
Hypolycorrhachia in young infants with viral meningitis To the Editor: From June through August, 1973, 36 children with aseptic meningitis were hospitalized at the Children's Hospital of Philadelphia. An attempt at virus isolation was made with specimens from 24 patients. Positive viral isolates were recovered from 14 patients, 8 of which were coxsackievirus B-2. Virus was isolated from the cerebrospinal fluid ( C S F ) i n three instances, and from the pharynx, throat, and rectum in eleven instances. Alihough acute and convalescent serum were not available from all of the patients studied, paired serum specimens of 12 patients from Philadelphia with aseptic meningitis were submitted to the Pennsylvania State Laboratory: six showed four-fold antibody titer rises to Coxsackie virus B - 2 or high-titer neutralizing antibodies in both specimens. Furthermore, Coxsackie virus B-2 was the strain responsible for most of the cases of aseptic meningitis from the Philadelphia area reporte d to the State Laboratory during the summer of 19731 A striking feature of the outbreak was the age distribution of the patients. Half of the infants affected were less than 2 months old, and more than three-fourths of those affected were less than 2 1/2 years old. Another impressive feature was the degree of hypoglycorrhachia. In one-third of the patients studied, the CSF glucose concentration was less than 50% of a simultaneously obtained blood glucose, and in more than three-fourths of the patients, the
