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December 1962, Vol 104, No. 6 >
Article | December 1962

Clinical Considerations in "Schilder's Disease" FREE

W. ZEMAN, M.D.; J. A. WHIELDON, M.D.
[+] Author Affiliations

Submitted for publication Jan. 4, 1961.


Am J Dis Child. 1962;104(6):635-643. doi:10.1001/archpedi.1962.02080030635008.
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Introduction  The remarkable interest of neuropathologists in the relatively rare conditions generically called Schilder's disease or diffuse sclerosis is reflected by the disproportionately large number of publications. Numerous authors have made valuable contributions, aimed at an understanding of the causes of these diseases, which ultimately enabled Poser and van Bogaert1,2 to arrive at a lucid histologic system of classification incorporating etiologic and prognostic implications. In summary, Poser2 proposed that these diseases fall into 3 different categories, (1) the myelinoclastic group, closely related to multiple sclerosis; (2) the dysmyelinating group or leukodystrophies, probably due to some inherited enzymatic defects; (3) the leukoencephalitides, possibly of viral origin. While this now universally accepted system is of immeasurable value to the pathologist, it is of little help to the clinician in differentiating these clinically similar but etiologically and pathogenically divergent conditions. Obviously, a correct clinical diagnosis will not only permit a more

REFERENCES

1 +
Poser, C. M., and van Bogaert, L.:  Natural History and Evolution of the Concept of Schilder's Diffuse Sclerosis , Acta Psychiat. Scand. 31:285-331, 1956;.
2 +
Poser, C. M.:  The Differential Diagnosis of Diffuse Sclerosis in Children , Amer. J. Dis. Child. 100:380-388, 1960;.
3 +
von Strümpell, A.:  Über diffuse Hirnsklerose , Arch. Psychiat Nervenkr. 9:268-285, 1879;.
4 +
Heubner, O.:  Über diffuse Hirnsklerose , Charite Annalen 22:298, 1897; (quoted by Schilder5).
5 +
Schilder, P.:  Zur Kenntnis der sogennanten diffusen Sklerose (über Encephalitis periaxialis diffusa) , Z. Neurol Psychiat. 10:1-60, 1912;.
6 +
Spielmeyer, W.: Histopathologie des Nervensystems , Berlin, Springer-Verlag, 1922;.
7 +
van Bogaert, L.:  Die klinische Einheit und die pathologische Variationsbreite der subakuten sklerosierenden Leuko-Enzephalitis , Wien. Z. Nervenheilk . 13:185-203, 1957;.
8 +
Schilder, P.:  Zur Frage der Encephalitis periaxialis diffusa (sogenannte diffuse Sklerose) , Z. Neurol. Psychiat. 15:359-376, 1913;.
9 +
Scholz, W.:  Klinische, pathologischanatomische und erbbiologische Untersuchungen bei familiärer, diffuser Hirnsklerose im Kindesalter , Z. Neurol. Psychiat. 99:651-717, 1925;.
10 +
Bielschowsky, M., and Henneberg, R.:  Über familiäre diffuse Sklerose (Leukodystrophia cerebri progressiva hereditaria) , J. Psychol. Neurol. 36: 131-181, 1928;.
11 +
Greenfield, J. G.:  A Form of Progressive Cerebral Sclerosis in Infants Associated with Primary Degeneration of the Interfascicular Glia , Proc. Roy. Soc. Med. 26:690-697, 1923;-1933.
12 +
Jervis, G. A.:  Infantile Metachromatic Leukodystrophy , J. Neuropath. Exp. Neurol. 19:323-341, 1960;.
13 +
Jatzkewitz, H.:  Zwei Typen von Cerebrosid-Schwefelsäuereestern als sog. Prälipoide und Speichersubstanzen bei der Leukodystrophie: Typ Scholz (Metachromatische Form der diffusen Sklerose) , Hoppe Seyler Z. Physiol. Chem. 311: 279-282, 1958;.
14 +
Austin, J. H.:  Metachromatic Form of Diffuse Cerebral Sclerosis: I. Diagnosis During Life by Urine Sediment Examination; II. Diagnosis During Life by Isolation of Metachromatic Lipids from Urine , Neurology (Minneap.) 7:415-426; 716-723, 1957;.
15 +
Pelizaeus, F.:  Über eine eigenthümliche Form spastischer Lähmung mit Cerebraler-Scheinungen auf hereditärer Grundlage (multiple Sklerose) , Arch. Psychiat. Nervenkr. 16:698-710, 1885;.
16 +
Merzbacher, L.:  Eine eigenartige familiärehereditäre Erkrankungsform (Aplasia axialis extra-corticalis congenita) , Z. Neurol. Psychiat. 3: 1-138, 1910;.
17 +
Krabbe, K.:  A New Infantile Form of Diffuse Brain-Sclerosis , Brain 39:74-114, 1916;.
18 +
van Bogaert, L.:  Type orthochromatique (a substance soudanophile) diffus de la leucodystrophie familiale , Acta Neuropath . 1:289-307, 1961;.
19 +
Hallervorden, J.: Die degenerative diffuse Sklerose, in Handbuch der speziellen pathologischen Anatomie und Histologie , Vol. 13, Part 1, pp. 716-782, edited by W. Scholz, Berlin, Springer-Verlag, 1957;.
20 +
Schilder, P.:  Die Encephalitis periaxialis diffusa (nebst Bemerkungen über die Apraxie des Lidschlusses) , Arch. Psychiat. Nervenkr. 71:327-356, 1924;.
21 +
Bodechtel, G., and Guttmann, E.:  Diffuse Encephalitis mit sklerosierender Entzündung des Hemisphärenmarkes , Z. Neurol. Psychiat. 133:601-619, 1931;.
22 +
Dawson, J. R., Jr.:  Cellular Inclusions in Cerebral Lesions of Lethargic Encephalitis , Amer. J. Path. 9:7-16, 1933;.
23 +
Dawson, J. R., Jr.:  Cellular Inclusions in Cerebral Lesions of Epidemic Encephalitis , Arch. Neurol. Psychiat. 31:685-700, 1934;.
24 +
Pette, H., and Döring, G.:  Über einheimische Panencephalomyelitis vom Charakter der Encephalitis japonica , Deutsch. Z. Nervenheilk. 149:7-44, 1939;.
25 +
van Bogaert, L., and de Busscher, J.:  Sur la Sclérose inflammatoire de la substance blanche des hémisphéres (Spielmeyer) , Rev. Neurol. (Par.) 71:679-701, 1939;.
26 +
Dellaert, R.; Maere, M., and van Bogaert, L.:  Deux nouvelles observations des scleroses inflammatoires de la substance blanche des hémisphéres (Spielmeyer) , Mschr. Psychiat. 110:103-124, 1945;.
27 +
van Bogaert, L.:  Une Leuco-Encéphalite sclérosante subaiguë , J. Neurol. Neurosurg. Psychiat. 8:101-120, 1945;.
28 +
Lhermitte, F.: Les Leuco-Encéphalites , Paris, Ernest Flammarion, 1950;.
29 +
Pette, H., and Kalm, H.:  Die entzündlichen Erkrankungen des Gehirns und seiner Haute , in Handbuch der inneren Medizin , Vol. 5, (Part 3) , pp. 106-270, edited by R. Jung, Berlin, Springer-Verlag, 1953;.
30 +
Gagnon, J.; Courtois, A., and Duchastel, Y.:  Encéphalite subaiguë à inclusions: Ses rapports avec la panencéphalite noduleuse et la leucoencéphalite sclérosante subaiguë , Un. Med. Canada 88:921-943, 1959;.
31 +
Tariska, S.:  Zur Pathologie des subakuten progressiven Panencephalitiden, Deutsch . Z. Nervenheilk . 179:363-387, 1959;.
32 +
Pelc, S.; Perier, J. O., and Quersin-Thiry, L.:  Résultats expérimentaux obtenus dans l'encéphalite humaine, type encéphalite subaigué a inclusions, leuco-encéphalite sclérosante subaiguë , Rev. Neurol. (Par.) 98:1-24, 1958;.
33 +
Simpson, J.:  Subacute Inclusion-Body Encephalitis , Lancet 2:685-687, 1961;.
34 +
Sherman, F. E.; Davies, R. L., and Haymaker, W.:  Subacute Inclusion Encephalitis , Acta Neuropath . 1:289-307, 1961;.
35 +
Mossakowski, M.; Mathieson, G., and Cumings, J. N.:  On the Relationship of Metachromatic Leucodystrophy and Amaurotic Idiocy , Brain 84: 585-604, 1951;.
36 +
Norman, R. M.; Oppenheimer, D. R., and Tingey, A. H.:  Histological and Chemical Findings in Krabbe's Leucodystrophy , J. Neurol. Neurosurg. Psychiat. 24:223-232, 1961;.
37 +
Tyler, H. R.:  Pelizaeus-Merzbacher Disease , A.M.A. Arch. Neurol. Psychiat. 80:162-169, 1958;.
38 +
Zeman, W.: Personal observations, unpublished.
39 +
Camp, C. D., and Löwenberg, K.:  An American Family with Pelizaeus-Merzbacher Disease , Arch. Neurol. Psychiat. 45:261-264, 1941;.
40 +
Fisarova, M., and Schwartzova, K.:  Diferenciální diagnosa mezi nemoci Schilderovou a subakutní encefalitidou s inklusemi , Cesk. Neurol. 21: 202-206, 1958;
41 +
cited in Zbl. Neurol. Psychiat. 150: 303, 1959;.
42 +
 Case Records of the Massachusetts General Hospital: Case 6-1962 , New Engl. J. Med. 266: 191-196, 1962;.
43 +
Hagberg, B., and Svennerholm, L.:  Metachromatic Leucodystrophy—A Generalized Lipidosis , Acta Pediat. 49:690-694, 1960;.
44 +
Helfant, M. H.; Borjeson, M., and Hellstrom, B.:  The Value of Urinary Sediment Examination as a Screening Method in Suspected Cases of Metachromatic Leucodystrophy , Acta Pediat. 51:49-54, 1962;.
45 +
Limbos, P.; Macken, J.; Guazzi, G. C., and Lowenthal, A.:  An Early Case of Subacute Sclerosing Leuco-Encephalitis from Congo: Clinical and Pathological Observations , Trop. Geogr. Med. 13: 104-114, 1961;.
46 +
Bauer, H.:  Spinal Fluid Alterations in Panencephalitis, First International Congress on Encephalomyelitides , Antwerp, Belgium , (May) , 1959;.
47 +
Bauer, H.: Personal communication to the authors.
48 +
Lyon, G.; Arthuis, M., and Thieffry, S.:  Leucodystrophie métachromatique infantile familiale: Étude de deux observations, dont une avec examen anatomique et chimique , Rev. Neurol. (Par.) 104: 508-532, 1961;.

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References

1 +
Poser, C. M., and van Bogaert, L.:  Natural History and Evolution of the Concept of Schilder's Diffuse Sclerosis , Acta Psychiat. Scand. 31:285-331, 1956;.
2 +
Poser, C. M.:  The Differential Diagnosis of Diffuse Sclerosis in Children , Amer. J. Dis. Child. 100:380-388, 1960;.
3 +
von Strümpell, A.:  Über diffuse Hirnsklerose , Arch. Psychiat Nervenkr. 9:268-285, 1879;.
4 +
Heubner, O.:  Über diffuse Hirnsklerose , Charite Annalen 22:298, 1897; (quoted by Schilder5).
5 +
Schilder, P.:  Zur Kenntnis der sogennanten diffusen Sklerose (über Encephalitis periaxialis diffusa) , Z. Neurol Psychiat. 10:1-60, 1912;.
6 +
Spielmeyer, W.: Histopathologie des Nervensystems , Berlin, Springer-Verlag, 1922;.
7 +
van Bogaert, L.:  Die klinische Einheit und die pathologische Variationsbreite der subakuten sklerosierenden Leuko-Enzephalitis , Wien. Z. Nervenheilk . 13:185-203, 1957;.
8 +
Schilder, P.:  Zur Frage der Encephalitis periaxialis diffusa (sogenannte diffuse Sklerose) , Z. Neurol. Psychiat. 15:359-376, 1913;.
9 +
Scholz, W.:  Klinische, pathologischanatomische und erbbiologische Untersuchungen bei familiärer, diffuser Hirnsklerose im Kindesalter , Z. Neurol. Psychiat. 99:651-717, 1925;.
10 +
Bielschowsky, M., and Henneberg, R.:  Über familiäre diffuse Sklerose (Leukodystrophia cerebri progressiva hereditaria) , J. Psychol. Neurol. 36: 131-181, 1928;.
11 +
Greenfield, J. G.:  A Form of Progressive Cerebral Sclerosis in Infants Associated with Primary Degeneration of the Interfascicular Glia , Proc. Roy. Soc. Med. 26:690-697, 1923;-1933.
12 +
Jervis, G. A.:  Infantile Metachromatic Leukodystrophy , J. Neuropath. Exp. Neurol. 19:323-341, 1960;.
13 +
Jatzkewitz, H.:  Zwei Typen von Cerebrosid-Schwefelsäuereestern als sog. Prälipoide und Speichersubstanzen bei der Leukodystrophie: Typ Scholz (Metachromatische Form der diffusen Sklerose) , Hoppe Seyler Z. Physiol. Chem. 311: 279-282, 1958;.
14 +
Austin, J. H.:  Metachromatic Form of Diffuse Cerebral Sclerosis: I. Diagnosis During Life by Urine Sediment Examination; II. Diagnosis During Life by Isolation of Metachromatic Lipids from Urine , Neurology (Minneap.) 7:415-426; 716-723, 1957;.
15 +
Pelizaeus, F.:  Über eine eigenthümliche Form spastischer Lähmung mit Cerebraler-Scheinungen auf hereditärer Grundlage (multiple Sklerose) , Arch. Psychiat. Nervenkr. 16:698-710, 1885;.
16 +
Merzbacher, L.:  Eine eigenartige familiärehereditäre Erkrankungsform (Aplasia axialis extra-corticalis congenita) , Z. Neurol. Psychiat. 3: 1-138, 1910;.
17 +
Krabbe, K.:  A New Infantile Form of Diffuse Brain-Sclerosis , Brain 39:74-114, 1916;.
18 +
van Bogaert, L.:  Type orthochromatique (a substance soudanophile) diffus de la leucodystrophie familiale , Acta Neuropath . 1:289-307, 1961;.
19 +
Hallervorden, J.: Die degenerative diffuse Sklerose, in Handbuch der speziellen pathologischen Anatomie und Histologie , Vol. 13, Part 1, pp. 716-782, edited by W. Scholz, Berlin, Springer-Verlag, 1957;.
20 +
Schilder, P.:  Die Encephalitis periaxialis diffusa (nebst Bemerkungen über die Apraxie des Lidschlusses) , Arch. Psychiat. Nervenkr. 71:327-356, 1924;.
21 +
Bodechtel, G., and Guttmann, E.:  Diffuse Encephalitis mit sklerosierender Entzündung des Hemisphärenmarkes , Z. Neurol. Psychiat. 133:601-619, 1931;.
22 +
Dawson, J. R., Jr.:  Cellular Inclusions in Cerebral Lesions of Lethargic Encephalitis , Amer. J. Path. 9:7-16, 1933;.
23 +
Dawson, J. R., Jr.:  Cellular Inclusions in Cerebral Lesions of Epidemic Encephalitis , Arch. Neurol. Psychiat. 31:685-700, 1934;.
24 +
Pette, H., and Döring, G.:  Über einheimische Panencephalomyelitis vom Charakter der Encephalitis japonica , Deutsch. Z. Nervenheilk. 149:7-44, 1939;.
25 +
van Bogaert, L., and de Busscher, J.:  Sur la Sclérose inflammatoire de la substance blanche des hémisphéres (Spielmeyer) , Rev. Neurol. (Par.) 71:679-701, 1939;.
26 +
Dellaert, R.; Maere, M., and van Bogaert, L.:  Deux nouvelles observations des scleroses inflammatoires de la substance blanche des hémisphéres (Spielmeyer) , Mschr. Psychiat. 110:103-124, 1945;.
27 +
van Bogaert, L.:  Une Leuco-Encéphalite sclérosante subaiguë , J. Neurol. Neurosurg. Psychiat. 8:101-120, 1945;.
28 +
Lhermitte, F.: Les Leuco-Encéphalites , Paris, Ernest Flammarion, 1950;.
29 +
Pette, H., and Kalm, H.:  Die entzündlichen Erkrankungen des Gehirns und seiner Haute , in Handbuch der inneren Medizin , Vol. 5, (Part 3) , pp. 106-270, edited by R. Jung, Berlin, Springer-Verlag, 1953;.
30 +
Gagnon, J.; Courtois, A., and Duchastel, Y.:  Encéphalite subaiguë à inclusions: Ses rapports avec la panencéphalite noduleuse et la leucoencéphalite sclérosante subaiguë , Un. Med. Canada 88:921-943, 1959;.
31 +
Tariska, S.:  Zur Pathologie des subakuten progressiven Panencephalitiden, Deutsch . Z. Nervenheilk . 179:363-387, 1959;.
32 +
Pelc, S.; Perier, J. O., and Quersin-Thiry, L.:  Résultats expérimentaux obtenus dans l'encéphalite humaine, type encéphalite subaigué a inclusions, leuco-encéphalite sclérosante subaiguë , Rev. Neurol. (Par.) 98:1-24, 1958;.
33 +
Simpson, J.:  Subacute Inclusion-Body Encephalitis , Lancet 2:685-687, 1961;.
34 +
Sherman, F. E.; Davies, R. L., and Haymaker, W.:  Subacute Inclusion Encephalitis , Acta Neuropath . 1:289-307, 1961;.
35 +
Mossakowski, M.; Mathieson, G., and Cumings, J. N.:  On the Relationship of Metachromatic Leucodystrophy and Amaurotic Idiocy , Brain 84: 585-604, 1951;.
36 +
Norman, R. M.; Oppenheimer, D. R., and Tingey, A. H.:  Histological and Chemical Findings in Krabbe's Leucodystrophy , J. Neurol. Neurosurg. Psychiat. 24:223-232, 1961;.
37 +
Tyler, H. R.:  Pelizaeus-Merzbacher Disease , A.M.A. Arch. Neurol. Psychiat. 80:162-169, 1958;.
38 +
Zeman, W.: Personal observations, unpublished.
39 +
Camp, C. D., and Löwenberg, K.:  An American Family with Pelizaeus-Merzbacher Disease , Arch. Neurol. Psychiat. 45:261-264, 1941;.
40 +
Fisarova, M., and Schwartzova, K.:  Diferenciální diagnosa mezi nemoci Schilderovou a subakutní encefalitidou s inklusemi , Cesk. Neurol. 21: 202-206, 1958;
41 +
cited in Zbl. Neurol. Psychiat. 150: 303, 1959;.
42 +
 Case Records of the Massachusetts General Hospital: Case 6-1962 , New Engl. J. Med. 266: 191-196, 1962;.
43 +
Hagberg, B., and Svennerholm, L.:  Metachromatic Leucodystrophy—A Generalized Lipidosis , Acta Pediat. 49:690-694, 1960;.
44 +
Helfant, M. H.; Borjeson, M., and Hellstrom, B.:  The Value of Urinary Sediment Examination as a Screening Method in Suspected Cases of Metachromatic Leucodystrophy , Acta Pediat. 51:49-54, 1962;.
45 +
Limbos, P.; Macken, J.; Guazzi, G. C., and Lowenthal, A.:  An Early Case of Subacute Sclerosing Leuco-Encephalitis from Congo: Clinical and Pathological Observations , Trop. Geogr. Med. 13: 104-114, 1961;.
46 +
Bauer, H.:  Spinal Fluid Alterations in Panencephalitis, First International Congress on Encephalomyelitides , Antwerp, Belgium , (May) , 1959;.
47 +
Bauer, H.: Personal communication to the authors.
48 +
Lyon, G.; Arthuis, M., and Thieffry, S.:  Leucodystrophie métachromatique infantile familiale: Étude de deux observations, dont une avec examen anatomique et chimique , Rev. Neurol. (Par.) 104: 508-532, 1961;.

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