HEREDITARY SPASTIC PARAPARESIS WITH DEMENTIA, AMYOTROPHY AND PERIPHERAL NEUROPATHY - A NEUROPATHOLOGICAL STUDY

被引:22
作者
FERRER, I
OLIVE, M
RIVERA, R
POU, A
NARBERHAUS, B
UGARTE, A
机构
[1] HOSP MARE DEU ESPERANCA,SERV NEUROL,BARCELONA,SPAIN
[2] HOSP GEN MANRESA,SERV NEUROL,MANRESA,SPAIN
关键词
HEREDITARY SPASTIC PARAPARESIS; STRUMPELL-LORRAIN; FRONTAL LOBE DEMENTIA; AMYOTROPHY;
D O I
10.1111/j.1365-2990.1995.tb01057.x
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Hereditary, probably autosomal recessive, spastic paraparesis in two siblings was associated with dementia of frontal lobe type, amyotrophy and peripheral sensory and motor polyneuropathy. Neuropathological findings correlate with neurological deficits, although neuron loss in the caudate and putamen, substantia nigra, and loss of Purkinje cells were clinically silent, Loss of neurons occurred in all cortical layers of the prefrontal lobe and superior temporal gyrus, Immunohistochemical studies showed reduced parvalbumin immunoreactivity in dendrites, and reduced numbers of calbindin D28k-immunoreactive cells, thus suggesting involvement of cortical local-circuit neurons. Myelin loss, ubiquitin-immunoreactive granular deposits, and nerve fibre degeneration in the white matter of the frontal lobes and corpus callosum were also observed. Cerebral and subcortical white matter abnormalities, together with atrophy of the thalamic dorsomedial complex and anterior nucleus, may have accounted for the development of severe dementia in this patient.
引用
收藏
页码:255 / 261
页数:7
相关论文
共 35 条
  • [31] Sequeiros J, 1993, HDB CEREBELLAR DISEA, P345
  • [32] SUTHERLAND JM, 1975, HDB CLIN NEUROLOGY, V22, P421
  • [33] NEUROFILAMENT AND GLIAL ALTERATIONS IN THE CEREBRAL-CORTEX IN AMYOTROPHIC-LATERAL-SCLEROSIS
    TROOST, D
    SMITT, PAES
    DEJONG, JMBV
    SWAAB, DF
    [J]. ACTA NEUROPATHOLOGICA, 1992, 84 (06) : 664 - 673
  • [34] HIPPOCAMPAL AND NEOCORTICAL UBIQUITIN-IMMUNOREACTIVE INCLUSIONS IN AMYOTROPHIC-LATERAL-SCLEROSIS WITH DEMENTIA
    WIGHTMAN, G
    ANDERSON, VER
    MARTIN, J
    SWASH, M
    ANDERTON, BH
    NEARY, D
    MANN, D
    LUTHERT, P
    LEIGH, PN
    [J]. NEUROSCIENCE LETTERS, 1992, 139 (02) : 269 - 274
  • [35] [No title captured]