Quantitative evaluation of intranuclear inclusions in SSPE: correlation with disease duration.
نویسندگان
چکیده
Three types of intranuclear inclusions in neurones, oligodendrocytes and astrocytes were quantitatively evaluated by electron microscopy in the autopsy material derived from six cases of subacute sclerosing panencephalitis (SSPE) with different duration of disease. Viral nucleocapsids were found in neurones and oligodendrocytes with the highest incidence (about 38% of nuclei) in two acute cases (adolescent), whereas in two subacute cases only 10% of nuclei of these cells contained nucleocapsids. However, in one acute case (child) and one chronic case, no nucleocapsids were detected at all, despite very intensive study. Two other types of intranuclear inclusions--nuclear bodies (NBs) and granulofilamentous inclusions (GFs) were present in astrocytic nuclei in all cases. Nuclear bodies were found the most frequently (about 66%) in cases of a several-week-long duration, and their incidence decreased with the extended duration of the disease. In the case of a seven-year-long duration, about 31% of nuclei contained NBs. The incidence of certain types of NBs varied also in individual groups of cases, and the same applied to the occurrence of cellular nuclei with different numbers of NBs. Nuclear bodies types IVand V occurred with similar frequency, regardless of the disease duration. The highest incidence of nucleocapsids and NBs was accompanied by the highest (about 25%) frequency of GF in astrocytic nuclei. The incidence of the latter declined with the prolonged duration of the disease, and in the chronic case it was about 16 times lower than in acute cases. In some acute and subacute cases, GF occurred together with NBs. Astrocytic nuclei with both types of inclusions occurred with a similar frequency (about 1.6-1.8%).
منابع مشابه
Neuronal ubiquitinated intranuclear inclusions in familial and non-familial frontotemporal dementia of the motor neuron disease type associated with amyotrophic lateral sclerosis.
Ubiquitinated cytoplasmic inclusions (Ub-CIs) in superficial frontal cortex and dentate gyrus neurons are the hallmark of frontotemporal degeneration of the motor neuron disease-type (FTD-MND-type). To date, 2 reports have described intranuclear ubiquitinated inclusions (Ub-INIs) in 9 cases of familial FTD-MND-type (without clinical or pathologic motor neuron disease, MND). In the current study...
متن کاملSkin biopsy is useful for the antemortem diagnosis of neuronal intranuclear inclusion disease.
BACKGROUND Neuronal intranuclear inclusion disease (NIID) is a progressive neurodegenerative disease characterized by eosinophilic hyaline intranuclear inclusions in neuronal and somatic cells. Because of the variety of clinical manifestations, antemortem diagnosis of NIID is difficult. METHODS Seven skin biopsy samples from patients with familial NIID were evaluated histochemically, and the ...
متن کاملOptineurin immunoreactivity in neuronal and glial intranuclear inclusions in adult-onset neuronal intranuclear inclusion disease.
Optineurin (OPTN) is a multifunctional protein involved in cellular morphogenesis, vesicle trafficking, maintenance of the Golgi complex, and transcription activation through its interactions with the Rab8, myosin 6 (MYO6), huntingtin. Recently, OPTN immunoreactivity has been reported in intranuclear inclusions in patients with neuronal intranuclear inclusions disease (NIID). Other studies have...
متن کاملAre neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions?
Neuronal intranuclear inclusions have been found in the brain of a transgenic mouse model of Huntington's disease and in necropsy brain tissue of patients with Huntington's disease. We suggest that neuronal intranuclear inclusions are the common neuropathology for all inherited diseases caused by expansion of polyglutamine repeats. We also suggest that patients with a pathological diagnosis of ...
متن کاملParadoxical absence of nuclear inclusion in cerebellar Purkinje cells of hereditary ataxias linked to CAG expansion.
Degeneration of cerebellar cortex is one of the principal features of hereditary ataxias linked to expansion of CAG repeat. In an attempt to clarify possible correlation between neuronal depletion and neuronal intranuclear inclusions, both triggered by the pathological expansion of CAG repeat, cerebellar sections from SCA1, SCA2, SCA3, and DRPLA cases were immunostained with anti-ubiquitin or a...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Folia neuropathologica
دوره 39 4 شماره
صفحات -
تاریخ انتشار 2001