Bunina bodies in amyotrophic lateral sclerosis

  title={Bunina bodies in amyotrophic lateral sclerosis},
  author={Koichi Okamoto and Yuji Mizuno and Yukio Fujita},
Bunina bodies, which are small eosinophilic intraneuronal inclusions in the remaining lower motor neurons, are generally considered to be a specific pathologic hallmark of amyotrophic lateral sclerosis (ALS). One year before a publication by Bunina, van Reeth et al. described similar intracytoplasmic inclusions in the anterior horn cells in a patient with Pick's dementia with atypical ALS. At present, only two proteins have been shown to be present in Bunina bodies, one is cystatin C and the… 

A Review of our Neuropathological Studies of Amyotrophic Lateral Sclerosis

Our neuropathological studies on amyotrophic lateral sclerosis (ALS), especially about Bunina bodies, fragmentation of Golgi apparatus and cortical ubiquitin-positive and tau-negative neuronal

Familial amyotrophic lateral sclerosis: a SOD1-unrelated Japanese family of bulbar type with Bunina bodies and ubiquitin-positive skein-like inclusions in lower motor neurons

A new family with adult onset amyotrophic lateral sclerosis (FALS) is described, in which the disease was characterized clinically by relatively rapid progression of bulbar symptoms and the presence of Bunina bodies and ubiquitin-positive skein-like inclusions in the lower motor neuron was of considerable interest.

Transferrin localizes in Bunina bodies in amyotrophic lateral sclerosis

The findings suggest that although the mechanisms underlying transferrin accumulation in Bunina bodies and basophilic inclusions are unknown, transferrin could be involved in forming these inclusions.

Decreased Cystatin C Immunoreactivity in Spinal Motor Neurons and Astrocytes in Amyotrophic Lateral Sclerosis

The findings suggest that the formation of TDP-43 inclusions, but not of Bunina bodies, may be linked to the content of CC in spinal motor neurons and that perturbations in endogenous levels ofCC in neuronal and glial cells may be part of the neurodegenerative processes in ALS.

Grand Rounds Vol 10 Pages L6–l9 Speciality: Landmark Case Report Article Type: Case Report Bunina Bodies

It is intriguing to speculate that Tat'yana Bunina's original description of Bunina bodies may have been the original first clue on a path to unravelling the pathogenetic process in ALS.

T.L. Bunina, Asao Hirano, and the post mortem cellular diagnosis of amyotrophic lateral sclerosis

  • L. Rowland
  • Medicine
    Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
  • 2009
Tat'yana Bunina, a Russian neuropathologist, has been immortalized because she discovered a neuronal inclusion and it was named after her; Bunina bodies and ubiquitinated inclusions have almost equal diagnostic value.

Colocalization of Bunina bodies and TDP‐43 inclusions in a case of sporadic amyotrophic lateral sclerosis with Lewy body‐like hyaline inclusions

There is some relationship between BBs and TDP‐43‐immunoreactive inclusions in terms of their formation processes, and immunohistochemical and ultrastructural examinations reveal the presence of BBs within the skein‐like and round hyaline inclusions, and in the LBHIs.

Bunina bodies in motor and non‐motor neurons revisited: A pathological study of an ALS patient after long‐term survival on a respirator

It was revealed that BBs exhibiting immunoreactivity for CC were a feature of LMNs, but not of non‐motor neurons, and that in the cerebellar dentate nucleus, the ratio of neurons withBBs and TDP‐43 inclusions/neurons with BBs was significantly lower than in other regions.

Protein Aggregates in Pathological Inclusions of Amyotrophic Lateral Sclerosis

Recent progress on genetic analysis has fuelled the identification of other genes responsible for fALS, and studies on mutant SOD1 have served as a “gold standard” for a long time and provided valuable insight into molecular pathomechanisms of ALS.




In an autopsy case of sporadic amyotrophic lateral sclerosis, there were intracytoplasmic eosinophilic inclusions of the Bunina type in motor neurons that seem to be due to deposition of some metabolite, but their nature is still obscure.

Widely distributed Bunina bodies and spheroids in a case of atypical sporadic amyotrophic lateral sclerosis

The autopsy findings of an 81-year-old patient with short-course sporadic amyotrophic lateral sclerosis showed a direct connection between axonal swelling and perikaryon was often seen in the facial and hypoglossal nuclei and in the spinal cord.

Bunina bodies in dendrites of patients with amyotrophic lateral sclerosis.

We studied the brains of two cases of amyotrophic lateral sclerosis with dementia. Bunina bodies were found in the motor neurons of cranial nerve nuclei (trigeminal, facial and hypoglossal nerves) as

Ultrastructure of the Bunina bodies in anterior horn cells of amyotrophic lateral sclerosis

Light and electron microscopic studies were made on the anterior horn cells in a case of amyotrophic lateral sclerosis and revealed the presence of two types of cytoplasmic inclusions, including Bunina body, which appeared to be originating from endoplasmic reticulum.

Selective appearance of Bunina bodies in amyotrophic lateral sclerosis

Bunina inclusion bodies were distributed abundantly in the nerve cell cytoplasm of a case of amyotrophic lateral sclerosis on a routine examination, indicating that this inclusion is related to the primary functional disturbance in the motor neurons of ALS.

Eosinophilic cytoplasmic inclusions in sporadic equine motor neuron disease: an electron microscopic study

It was concluded that, although the distribution and nature of the lesions in EMND appeared similar to those of human motor neuron disease, none of the equine eosinophilic inclusions duplicated the ultrastructure of Bunina bodies.

Anterior horn cell degeneration and Bunina-type inclusions associated with dementia

The ultrastructural features of Bunina type inclusions in the anterior horn cells of a patient dying of amyotrophic lateral sclerosis with dementia appear unique, and appear to be a special type of autophagic vacuole, possibly arising from altered mitochondria.

Fine Structure of Anterior Horns in Patients Without Amyotrophic Lateral Sclerosis

Light and electron microscopic study of the anterior horns in nine patients without amyotrophic lateral sclerosis (ALS) disclosed several features usually described in ALS. Central chromatolysis and

Ubiquitin-immunoreactive intraneuronal inclusions in amyotrophic lateral sclerosis. Morphology, distribution, and specificity.

It is concluded that the presence of ubiquitin-IR inclusions in lower motor neurons represents a characteristic pathological feature of ALS in its various clinical forms.