Loss of Purkinje Cells: Causes & Reasons

Possible Causes for Loss of Purkinje Cells

[…] of Purkinje cells in the cerebellar vermis 0007001 Microcephaly Abnormally small skull Decreased circumference of cranium Decreased size of skull Reduced head circumference [rarediseases.info.nih.gov]

[…] present at birth 0003577 Death in infancy Infantile death Lethal in infancy [ more ] 0001522 Gliosis 0002171 Hypoplasia of the pons 0012110 Infantile encephalopathy 0007105 Loss [rarediseases.info.nih.gov]

Autosomal Recessive Spinocerebellar Ataxia

[…] of Purkinje cells in the cerebellum and often hearing loss and skeletal abnormalities. [experts.umich.edu]

Microscopic examination sows loss of Purkinje cells and neurons in other affected nuclei. [neuropathology-web.org]

Autosomal recessive spinocerebellar ataxia 20 (SCAR20) is a recently described disorder characterized by intellectual disability, ataxia, coarse facial features, progressive loss [experts.umich.edu]

Alcoholic Cerebellar Degeneration

In ACD cases, loss of Purkinje cells, narrowing of the width of the molecular layer, and tissue rarefaction in the granular layer were observed in the anterior and superior [ncbi.nlm.nih.gov]

Kymberly Gyure Microscopic (histologic) description Loss of cerebellar Purkinje cells with corresponding Bergmann gliosis Narrowing of the molecular layer and a reduced number [pathologyoutlines.com]

There is a loss of purkinje cells Multiple system atrophy - Cerebellar also known as Oliviopontocerebellar atrophy. [medexam.net]

Spinocerebellar Ataxia Type 6

The main histopathological findings included a severe neuronal cell loss of Purkinje cells and inferior olivary nuclei. [ncbi.nlm.nih.gov]

Severe loss of Purkinje cells was found, particularly in the vermis, and various morphological changes in Purkinje cells and their dendritic arborizations were demonstrated [ncbi.nlm.nih.gov]

[…] of cerebellar Purkinje cells. [ncbi.nlm.nih.gov]

Spinocerebellar Ataxia Type 1

In SCA1, there is loss of Purkinje cells, neuronal loss in dentate nucleus, olives, and pontine nuclei. [ncbi.nlm.nih.gov]

Our results showed reduced numbers of Bergmann glia in SCA1 patient brains and support the hypothesis that Bergmann glia loss contributes toward Purkinje cell degeneration [ncbi.nlm.nih.gov]

cells. [ncbi.nlm.nih.gov]

Cerebellar Ataxia with Peripheral Neuropathy Type 2

Microscopic examination sows loss of Purkinje cells and neurons in other affected nuclei. [neuropathology-web.org]

There is a loss of purkinje cells Multiple system atrophy - Cerebellar also known as Oliviopontocerebellar atrophy. [medexam.net]

SCA 2 is characterized by cerebellar atrophy and a loss of Purkinje cells and granular olivary neurons of the substantia nigra and ventral horn neurons in the spinal cord [tinnitusjournal.com]

Paraneoplastic Neurologic Syndrome

[…] of Purkinje cells. [en.wikipedia.org]

Pleocytosis common IgG: Moderately high; Some oligoclonal bands Pathology: Loss of Purkinje cells Yo antibody syndrome Yo staining Cerebellar Purkinje cell Epidemiology: [neuromuscular.wustl.edu]

Characteristic pathologic changes include widespread loss of Purkinje cells and lymphocytic cuffing of deep blood vessels. [msdmanuals.com]

Olivopontocerebellar Atrophy

The cerebellum showed hypoplasia with rudimentary dentate nuclei, profound loss of Purkinje cells and external granule cell layer, a sparse internal granule cell layer of [ncbi.nlm.nih.gov]

Decreased expression in the steady state level of EAAT4 mRNA in the OPCA sample was correlated with the selective loss of Purkinje cells. [ncbi.nlm.nih.gov]

GABA concentrations in the dentate nucleus were decreased in all cases of MSA (OPCA) and CCA, and were positively correlated with the degree of loss of Purkinje cells (r = [ncbi.nlm.nih.gov]

Adult-Onset Autosomal Recessive Cerebellar Ataxia

Microscopic examination sows loss of Purkinje cells and neurons in other affected nuclei. [neuropathology-web.org]

[…] of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene. ‏ [books.google.com]

[…] of Purkinje cells in the cerebellar cortex, deep cerebellar nuclei & inferior olivary nuclei  ? [slideshare.net]

Spinocerebellar Ataxia Type 12

RECENT FINDINGS: The first neuropathological examination of a brain from an SCA12 patient revealed both cerebellar and cerebral cortical atrophy, with a noted loss of Purkinje [ncbi.nlm.nih.gov]

[…] of Purkinje cells and no evidence of polyglutamine aggregates (Cohen and Margolis, 2016; pubmed:27748686 ). [flybase.org]

The first neuropathological examination of a brain from an SCA12 patient revealed both cerebellar and cerebral cortical atrophy, with a noted loss of Purkinje cells and no [journals.lww.com]

Autosomal Recessive Primary Microcephaly Type 10

[…] in central nervous system Hypoplasia of the brainstem Congenital contracture Hypoplasia of the pons Infantile encephalopathy Loss of Purkinje cells in the cerebellar vermis [mendelian.co]

[…] of Purkinje cells in the cerebellar vermis Short stature Chorea Dyskinesia Abnormality of the cerebral white matter Abnormal facial shape Delayed speech and language development [mendelian.co]

[…] impairment Growth delay Cerebral atrophy Delayed speech and language development Clonus Gliosis Myoclonus Death in infancy Polyhydramnios Cerebellar hypoplasia Neuronal loss [mendelian.co]

Cogan Syndrome

Neuropathological examination reveals severe loss of Purkinje cells and moderate neuronal loss in the anterior horn and dorsal root ganglia. [ncbi.nlm.nih.gov]

MRI may show cerebellar atrophy, mild brainstem atrophy, and cortical atrophy in advanced cases of AOA1, cerebellar atrophy, loss of Purkinje cells, and demyelination in AOA2 [bredagenetics.com]

AOA2 shows cerebellar atrophy, loss of Purkinje cells, and demyelination. [en.wikipedia.org]

Subacute Cerebellar Degeneration

[…] of Purkinje cells. [en.wikipedia.org]

In addition to this distribution of degenerative lesions, uneven loss of Purkinje cells was apparent. [link.springer.com]

Parenchymatous Cortical Cerebellar Degeneration

[…] of Purkinje cells. [alzforum.org]

cell layers with dentate gliosis → neuronal loss and spongiosis Preceded by neuroleptic malignant syndrome Radiation and Chemotherapy Injury may be divided into acute, early [neupsykey.com]

His cerebellar syndrome was noted to correlate with extensive Aβ deposition in the cerebellar cortex as well as a severe loss of Purkinje cells. [alzforum.org]

Autosomal Recessive Spinocerebellar Ataxia 8

Microscopic examination sows loss of Purkinje cells and neurons in other affected nuclei. [neuropathology-web.org]

The loss of brain cells in the cerebellum causes the movement problems characteristic of ARCA1, but it is unclear how this cell loss is related to impaired Purkinje cell function [ghr.nlm.nih.gov]

[…] of vibration and joint position sense Pathologic examination showing loss of Purkinje cells, granule cells, neurons of the inferior olive nucleus, and dentate nucleus Progressive [emedicine.medscape.com]

Spinocerebellar Ataxia Type 10

Although the primary pathology in SCA10 in humans is reportedly the loss of Purkinje cells, brain MRI revealed frontal lobe atrophy with white matter lesions. [ncbi.nlm.nih.gov]

Purkinje cell loss is the major brain pathology of spinocerebellar ataxia type 10 Guangbin Xia 1, Karen N McFarland 1, Kang Wang 2, Partha S Sarkar 3, Anthony T Yachnis 4, [jnnp.bmj.com]

Neuronal Intranuclear Inclusion Disease

Marked loss of cerebellar Purkinje cells and neurons in the dentate nucleus, red nucleus, and spinal cord anterior horns was accompanied by a modest astrocytosis. [experts.umich.edu]

The cerebellar cortex showed massive Purkinje cell loss with attendant Bergmann gliosis (Figure 3). NII were seen in the majority of rare remaining Purkinje cells. [path.upmc.edu]

The granular cell layer exhibited moderate neuronal loss but no discernible NII. [path.upmc.edu]

Pontocerebellar Hypoplasia Type 2A

Résumé Les troubles du développement du cervelet résultent soit de causes endogènes (anomalies génétiques et métaboliques) ou exogènes (intoxications, infections, irradiations). Ces anomalies peuvent affecter les différents stades de l’histogenèse pré-et postnatale du cervelet [1]: la segmentation rostrocaudale[…] [link.springer.com]

Cerebellar Ataxia - Neuropathy - Vestibular Areflexia Syndrome

Brain and temporal bone pathology in one patient showed marked loss of Purkinje cells and of vestibular, trigeminal, and facial ganglion cells, but not of spiral ganglion [wwww.unboundmedicine.com]

Cerebellar Atrophy

Abstract Cerebellar atrophy, consequent to the postdevelopmental degeneration and loss of Purkinje cells and granular neurons, has been identified in three patients with rhizomelic [ncbi.nlm.nih.gov]

In all four cases, the most obvious pathological finding throughout the cerebellum was loss of Purkinje cells, but the rarefaction of granular cell layers was observed only [ncbi.nlm.nih.gov]

Autopsy of the proband revealed a severe loss of Purkinje cells, degeneration of posterior columns and spinocerebellar tracts of the spinal cord, and a marked loss of myelinated [ncbi.nlm.nih.gov]

Pontocerebellar Hypoplasia Type 8

Loss of Purkinje cells, folial atrophy, degeneration of dentate nuclei, and loss of ventral pontine nuclei and transverse pontine nerve fibers Less common. Spinal cord. [ncbi.nlm.nih.gov]

Degeneration and loss of motor neurons in the anterior spinal horn Cerebellum. [ncbi.nlm.nih.gov]

Fetal Akinesia Sequence

The brain showed focal cerebral and diffuse cerebellar white matter gliosis, and patchy loss of internal granular and Purkinje cells in the cerebellar cortex. [ncbi.nlm.nih.gov]

Ataxia-Oculomotor Apraxia Syndrome

AOA2 shows cerebellar atrophy, loss of Purkinje cells, and demyelination. [en.wikipedia.org]

The available data indicate marked cerebellar atrophy, loss of Purkinje cells, and demyelinization. [frontiersin.org]

Vestibulocerebellar Ataxia

Brain and temporal bone pathology in one patient showed marked loss of Purkinje cells and of vestibular, trigeminal, and facial ganglion cells, but not of spiral ganglion [ncbi.nlm.nih.gov]

[…] of Purkinje cells at cerebellar level and sensory neuronopathy. [elsevier.es]

In the cerebellum, there is pathological evidence of vermian atrophy, with notable loss of vermian Purkinje cells 2,3,5. MRI MRI is the imaging modality of choice 6. [radiopaedia.org]

Infantile Neuronal Ceroid Lipofuscinosis

For juvenile onset NCL, along with neuronal loss there is extensive gliosis and complete loss of purkinje cells. [symptoma.com]

Histological studies of Infantile NCL show neuronal loss. In late onset NCL, there are vacuolated lymphocytes observed. [symptoma.com]

Spinocerebellar Ataxia Type 19

Abstract The dominantly inherited cerebellar ataxias are a heterogeneous group of neurodegenerative disorders caused by Purkinje cell loss in the cerebellum. [ncbi.nlm.nih.gov]

Purkinje cell loss is the major brain pathology of spinocerebellar ataxia type 10. [journals.plos.org]

[…] of Purkinje cells and granular cells, neuron loss in the olivary nucleus, the substantia nigra and anterior horn cells of the spinal cord.3,4 The SCA 2 mutation locus is [demneuropsy.com.br]

Autosomal Recessive Spinocerebellar Ataxia 3

Microscopic examination sows loss of Purkinje cells and neurons in other affected nuclei. [neuropathology-web.org]

[…] of cerebellar granule and Purkinje cells リンク ICD-11: 4A01.31 ICD-10: G11.3 MeSH: D001260 OMIM: 208900 文献 PMID: 17889645 著者 Rass U, Ahel I, West SC. [genome.jp]

Essential Tremor

At postmortem examination, there was segmental loss of Purkinje cells, presence of torpedoes, and Bergmann gliosis in the cerebellar cortex. [ncbi.nlm.nih.gov]

On postmortem examination, there was severe segmental loss of Purkinje cells, Bergmann gliosis and numerous torpedoes in the cerebellum. [ncbi.nlm.nih.gov]

Essential tremor also appears to involve a disruption in the activity of motor pathways, but it's uncertain whether there is any loss of brain cells. [health.harvard.edu]

Cognitive Impairment - Cerebellar Ataxia

Microscopic examination sows loss of Purkinje cells and neurons in other affected nuclei. [neuropathology-web.org]

Purkinje cell loss is the major brain pathology of spinocerebellar ataxia type 10. [journals.plos.org]

Neurodegeneration in the cerebellum is characterized by marked Purkinje cell loss, gliosis and axonal swelling. [radiopaedia.org]

Paraneoplastic Syndrome

[…] of Purkinje cells. [en.wikipedia.org]

Optic neuritis Survival Mean > 9 years Longer than Hu or Yo syndromes Neoplasm: Hodgkins lymphoma (89%): Espeically nodular sclerosis No neoplasm (11%) Cerebellar pathology Loss [neuromuscular.wustl.edu]