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January 1989, Vol 46, No. 1 >
Article | January 1989

An Autopsy Case of Atypical Infantile Motor Neuron Disease With Hyaline Intraneuronal Inclusions

Yoshio Morimatsu, MD; Junichi Satoh, MD; Masaya Oda, MD; Kenji Yokochi, MD
[+] Author Affiliations

Accepted for publication Jan 4, 1988.

Reprint requests to the Department of Pediatrics, Seirei-Mikatabara Hospital, 3453 Mikatabara-cho, Hamamatsu, Shizuoka 433, Japan.


Arch Neurol. 1989;46(1):103-107. doi:10.1001/archneur.1989.00520370105031
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• We describe a 5-year-old boy who had had a progressive motor weakness with bulbar palsy and spasticity of the lower extremities since age 3 years and who died of bronchopneumonia after about two and a half years of the illness. Neuropathologic examination revealed combined degenerative processes in the upper and lower motor neurons, the spinocerebellar and olivocerebellar systems, and the ventral thalamic nuclei. Lewy body—like intraneuronal hyaline inclusions, which ultrastructurally showed irregular accumulations of trilaminar membranous profiles, were detected in the spinal anterior horn, Clarke's dorsal nucleus, facial nerve nucleus, inferior olivary nucleus, and substantia nigra. This case could be considered as a unique form in the group of the infantile motor neuron diseases associated with multisystemic degenerations in the central nervous system.

REFERENCES

1 +
Dubowitz V: Muscle Disorders in Childhood . Philadelphia, WB Saunders Co, 1978;.
2 +
Byers RD, Banker BQ:  Infantile muscular atrophy . Arch Neurol 1961;;5:140-164.
3 +
Gomez MR, Clermont V, Bernstein J:  Progressive bulbar paralysis in childhood (Fazio-Londe's disease) . Arch Neurol 1962;;6:317-323.
4 +
Alexander MP, Emery ES III, Koerner FC:  Progressive bulbar paresis in childhood . Arch Neurol 1976;;33:66-68.
5 +
Hudson AJ:  Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: A review . Brain 1981;;104:217-247.
6 +
Tandan R, Bradley WG:  Amyotrophic lateral sclerosis: I. Clinical features, pathology, and ethical issues in management . Ann Neurol 1985;;18:271-280.
7 +
Van Bogaert L:  La sclérose latérale amyotrophique et la paralysie bulbaire progressive chez l'enfant . Rev Neurol 1925;;1:180-192.
8 +
Nelson JS, Prensky AL:  Sporadic juvenile ALS: A clinicopathological study of a case with neuronal cytoplasmic inclusions containing RNA . Arch Neurol 1972;;27:300-306.
9 +
Oda M, Akagawa N, Tabuchi Y, et al:  A sporadic juvenile case of the amyotrophic lateral sclerosis with neuronal intracytoplasmic inclusions . Acta Neuropathol 1978;;44:211-216.
10 +
Steiman GS, Rorke LB, Brown MJ:  Infantile neuronal degeneration masquerading as Werdnig-Hoffmann disease . Ann Neurol 1980;;8:317-324.
11 +
Duffy PE, Tennyson VM:  Phase and electron microscopic observations of Lewy bodies and melanin granules in the substantia nigra and locus caeruleus in Parkinson's disease . J Neuropathol Exp Neurol 1965;;24:398-414.
12 +
Ikeda K, Ikeda S, Yoshimura T, et al:  Idiopathic Parkinsonism with Lewy-type inclusions in cerebral cortex: A case report . Acta Neuropathol 1965;;41:165-168.
13 +
Hirano A, Kurland LT, Sayre GP:  Familial amyotrophic lateral sclerosis: A subgroup characterized by posterior and spinocerebellar tract involvement and hyaline inclusions in the anterior horn cells . Arch Neurol 1967;;16:232-243.
14 +
Takahashi K, Nakamura H, Okada E:  Hereditary amyotrophic lateral sclerosis: Histochemical and electron microscopic study of hyaline inclusions in motor neurons . Arch Neurol 1972;;27:292-299.
15 +
Norman MG:  Hyaline (colloid) cytoplasmic inclusions in motoneurons in association with familial microencephaly, retardation and seizures . J Neurol Sci 1974;;23:63-70.
16 +
Schochet SS Jr, Hardman JM, Ladewig PP, et al:  Intraneuronal conglomerates in sporadic motor neuron disease: A light and electron microscopic study . Arch Neurol 1969;;20:548-553.
17 +
Sun CN, Araoz C, Lucas G, et al:  ALS: Inclusion bodies in a case of the classic sporadic form . Ann Clin Lab Sci 1975;;5:38-44.
18 +
Roessmann U, McFarland DE:  Hyaline cytoplasmic inclusions in motor neurons . J Neuropathol Exp Neurol 1971;;30:551-556.
19 +
Pena CE:  Intracytoplasmic neuronal inclusions in the human thalamus: Light-microscopic, histochemical and ultrastructural observations . Acta Neuropathol 1980;;52:157-159.
20 +
Wisniewski HM, Berry K, Spiro AJ:  Ultrastructure of thalamic neuronal inclusions in myotonic dystrophy . J Neurol Sci 1975;;24:321-329.
21 +
Sung JH, Pamirez-Lassepas M, Mastri AR, et al:  An unusual degenerative disorder of neurons associated with a novel intranuclear hyaline inclusion (neuronal intranuclear hyaline inclusion disease) . J Neuropathol Exp Neurol 1980;;39:107-130.
22 +
Haltia M, Somer H, Palo J, et al:  Neuronal intranuclear inclusion disease in identical twins . Ann Neurol 1984;;15:316-321.
23 +
Garen PD, Powers JM, Young GF, et al:  Neuronal intranuclear hyaline inclusion disease in a nine year old . Acta Neuropathol 1986;;70:327-332.
24 +
Muñoz-Garcia D, Ludwin SK:  Adult-onset neuronal intranuclear hyaline inclusion disease . Neurology 1986;;36:785-790.
25 +
Ghadially FM: Ultrastructural Pathology of the Cell and Matrix , ed 2. Stoneham, Mass, Butterworths, 1982;.

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1 +
Dubowitz V: Muscle Disorders in Childhood . Philadelphia, WB Saunders Co, 1978;.
2 +
Byers RD, Banker BQ:  Infantile muscular atrophy . Arch Neurol 1961;;5:140-164.
3 +
Gomez MR, Clermont V, Bernstein J:  Progressive bulbar paralysis in childhood (Fazio-Londe's disease) . Arch Neurol 1962;;6:317-323.
4 +
Alexander MP, Emery ES III, Koerner FC:  Progressive bulbar paresis in childhood . Arch Neurol 1976;;33:66-68.
5 +
Hudson AJ:  Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: A review . Brain 1981;;104:217-247.
6 +
Tandan R, Bradley WG:  Amyotrophic lateral sclerosis: I. Clinical features, pathology, and ethical issues in management . Ann Neurol 1985;;18:271-280.
7 +
Van Bogaert L:  La sclérose latérale amyotrophique et la paralysie bulbaire progressive chez l'enfant . Rev Neurol 1925;;1:180-192.
8 +
Nelson JS, Prensky AL:  Sporadic juvenile ALS: A clinicopathological study of a case with neuronal cytoplasmic inclusions containing RNA . Arch Neurol 1972;;27:300-306.
9 +
Oda M, Akagawa N, Tabuchi Y, et al:  A sporadic juvenile case of the amyotrophic lateral sclerosis with neuronal intracytoplasmic inclusions . Acta Neuropathol 1978;;44:211-216.
10 +
Steiman GS, Rorke LB, Brown MJ:  Infantile neuronal degeneration masquerading as Werdnig-Hoffmann disease . Ann Neurol 1980;;8:317-324.
11 +
Duffy PE, Tennyson VM:  Phase and electron microscopic observations of Lewy bodies and melanin granules in the substantia nigra and locus caeruleus in Parkinson's disease . J Neuropathol Exp Neurol 1965;;24:398-414.
12 +
Ikeda K, Ikeda S, Yoshimura T, et al:  Idiopathic Parkinsonism with Lewy-type inclusions in cerebral cortex: A case report . Acta Neuropathol 1965;;41:165-168.
13 +
Hirano A, Kurland LT, Sayre GP:  Familial amyotrophic lateral sclerosis: A subgroup characterized by posterior and spinocerebellar tract involvement and hyaline inclusions in the anterior horn cells . Arch Neurol 1967;;16:232-243.
14 +
Takahashi K, Nakamura H, Okada E:  Hereditary amyotrophic lateral sclerosis: Histochemical and electron microscopic study of hyaline inclusions in motor neurons . Arch Neurol 1972;;27:292-299.
15 +
Norman MG:  Hyaline (colloid) cytoplasmic inclusions in motoneurons in association with familial microencephaly, retardation and seizures . J Neurol Sci 1974;;23:63-70.
16 +
Schochet SS Jr, Hardman JM, Ladewig PP, et al:  Intraneuronal conglomerates in sporadic motor neuron disease: A light and electron microscopic study . Arch Neurol 1969;;20:548-553.
17 +
Sun CN, Araoz C, Lucas G, et al:  ALS: Inclusion bodies in a case of the classic sporadic form . Ann Clin Lab Sci 1975;;5:38-44.
18 +
Roessmann U, McFarland DE:  Hyaline cytoplasmic inclusions in motor neurons . J Neuropathol Exp Neurol 1971;;30:551-556.
19 +
Pena CE:  Intracytoplasmic neuronal inclusions in the human thalamus: Light-microscopic, histochemical and ultrastructural observations . Acta Neuropathol 1980;;52:157-159.
20 +
Wisniewski HM, Berry K, Spiro AJ:  Ultrastructure of thalamic neuronal inclusions in myotonic dystrophy . J Neurol Sci 1975;;24:321-329.
21 +
Sung JH, Pamirez-Lassepas M, Mastri AR, et al:  An unusual degenerative disorder of neurons associated with a novel intranuclear hyaline inclusion (neuronal intranuclear hyaline inclusion disease) . J Neuropathol Exp Neurol 1980;;39:107-130.
22 +
Haltia M, Somer H, Palo J, et al:  Neuronal intranuclear inclusion disease in identical twins . Ann Neurol 1984;;15:316-321.
23 +
Garen PD, Powers JM, Young GF, et al:  Neuronal intranuclear hyaline inclusion disease in a nine year old . Acta Neuropathol 1986;;70:327-332.
24 +
Muñoz-Garcia D, Ludwin SK:  Adult-onset neuronal intranuclear hyaline inclusion disease . Neurology 1986;;36:785-790.
25 +
Ghadially FM: Ultrastructural Pathology of the Cell and Matrix , ed 2. Stoneham, Mass, Butterworths, 1982;.

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