Neuronal Loss: Causes & Reasons

Possible Causes for Neuronal Loss

Neuronal loss is maximal in the HIPPOCAMPUS, entorhinal cortex, and AMYGDALA. Some ballooned cortical neurons contain argentophylic (Pick) bodies. [hon.ch]

The characteristic features of Pick disease include 1 : Pick bodies swollen chromatolytic neurons loss of large pyramidal neurons astrocytic gliosis The primary radiographic [radiopaedia.org]

Clinical features include APHASIA; APRAXIA; CONFUSION; ANOMIA; memory loss; and personality deterioration. [connects.catalyst.harvard.edu]

Alpers Syndrome

The pathology of left occipital lobe showed neuronal loss, spongiform degeneration, astrocytosis, and demyelination. [ncbi.nlm.nih.gov]

Pathological findings were of neuronal loss and hepatic cirrhosis. [ncbi.nlm.nih.gov]

Post mortem findings in one patient included neuronal loss and gliosis and hepatic cirrhosis. [ 1 ] COMMENT. [pediatricneurologybriefs.com]

Infantile Neuroaxonal Dystrophy

At autopsy, one child had extensive astrogliosis and neuronal loss with shrinkage of the cerebellar cortex, in addition to typical widespread changes of neuroaxonal dystrophy [ncbi.nlm.nih.gov]

Light-microscopic examination of the brain reveals wide distribution of spheroids, cerebellar atrophy, and neuronal loss with astrocytosis. [ncbi.nlm.nih.gov]

LSB were prominent in the posterior column, gracile nucleus, cuneate nucleus, and the tegmentum of the midbrain and the pons associated with neuronal loss and gliosis. [ncbi.nlm.nih.gov]

Familial Encephalopathy with Neuroserpin Inclusion Bodies

However, neither neuronal loss nor gliosis are apparent. [ncbi.nlm.nih.gov]

Neuronal loss is evident only in cortical layer II, and glial involvement is not seen except for the reactive change in layer II, as well as a low-grade subcortical gliosis [ncbi.nlm.nih.gov]

[…] of neuronal function. [ncbi.nlm.nih.gov]

Neuromyelitis Optica Spectrum Disorder with Anti-AQP4 Antibodies

The astrocytes and the neighboring cells get injured, which leads to demyelination, neuronal loss, and subsequent neurologic deficits.10,-,12 Since the identification of AQP4 [nn.neurology.org]

[…] of astrocytes, and sometimes also a loss of neurons and oligodendrocytes. [en.wikipedia.org]

[…] and neuronal injury7,8,9. [nature.com]

Combined Oxidative Phosphorylation Deficiency Type 24

In addition, CI defects have been linked to Parkinson's disease (PD), a neurodegenerative condition which results from the progressive loss of dopaminergic neurons in the [bioscirep.org]

Ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 616192 601184 Autosomal recessive DNAJC5 20q13.33 Ceroid lipofuscinosis, neuronal, 4, Parry [mnglabs.com]

Distal Spinal Muscular Atrophy Type 4

Loss of motor neurons and hypercellularity due to gliosis. [neuropathology-web.org]

Spinal muscular atrophy is characterised by slowly progressive muscle weakness and atrophy of the limb muscles associated with motor neurone loss in the spinal cord. [patient.info]

[…] fibers in the fasciculus gracilis and marked loss of neurons with severe gliosis in the dentate nucleus. [emedicine.medscape.com]

Upper Motor Neuron Disease

Temporal cortex Abbreviations + Pathological Change (i.e. inclusion bodies, neuronal loss, astrocytosis, UIBs, SCIs ++ Predominant Pathological Change - Uninvolved/Little [wiki.iop.kcl.ac.uk]

Loss of motor neurons and hypercellularity due to gliosis. [neuropathology-web.org]

Turner and Al-Chalabi 2007 Motor neurone disease comprises a number of clinical phenotypes united by the pathological feature of progressive motor neuronal loss. [mndcare.net.au]

Classic Progressive Supranuclear Palsy Syndrome

The clinical heterogeneity is associated with variability of regional distribution and severity of abnormal tau accumulation and neuronal loss. [ingentaconnect.com]

Neuropathologically, all forms are characterized by neuronal loss, gliosis with astrocytic plaques and accumulation of tau-immunoreactive neurofibrillary tangles in specific [orpha.net]

Neuronal Intranuclear Inclusion Disease

[…] intranuclear inclusions (NIT) accompanied by a more restricted pattern of neuronal loss. [experts.umich.edu]

[…] intranuclear inclusions (NII) accompanied by a more restricted pattern of neuronal loss. [ncbi.nlm.nih.gov]

There was no apparent correlation between frequency of the inclusions and neuronal loss. Intranuclear inclusions were found in many morphologically normal neurons. [ncbi.nlm.nih.gov]

Spinal and Bulbar Muscular Atrophy

loss in the spinal cord and brainstem. [ncbi.nlm.nih.gov]

loss in males. [ncbi.nlm.nih.gov]

The loss of motor neurons in SBMA results in muscle atrophy and weakness, involuntary muscle twitching and mild sensory impairment as well as signs of endocrine abnormalities [genpharmservices.com]

Hallervorden-Spatz Syndrome

Sections of neocortex showed mild patchy neuronal loss and occasional spheroids. [jnnp.bmj.com]

Severe neuronal loss was noted in the pars reticulata of the substantia nigra with a moderate degree of iron deposition and neuroaxonal spheroids (fig 2 D). [jnnp.bmj.com]

The pathological findings were isolated pallidal degeneration with demyelination and moderate neuronal loss, iron pigment accumulation and spheroid bodies in both pallidal [ncbi.nlm.nih.gov]

Cerebral Cortical Atrophy

Generalized atrophy refers to neuron loss throughout the entire brain, and focal atrophy refers to neuron loss in a specific brain region. [livestrong.com]

Supporting the atrophic patterns seen here, Oster et al. ( 18 ) and Fisher et al. ( 19 ) detected an overall neuronal loss of 27% and 25%, respectively (≈6 × 10 9 neurons) [pnas.org]

The good news about this study is that the shrinkage was accompanied by only minimal neuronal loss suggesting that proper treatment might reverse this portion of the decreased [practicalpainmanagement.com]

Temporal Lobe Epilepsy

[…] indicator of hippocampal neuron loss. [doi.org]

Statistically significant correlations existed only between percent retention scores and hippocampal neuron loss in CA3 and the hilar area for patients with left temporal [ncbi.nlm.nih.gov]

Patients with severe loss of left hippocampal neurons performed worse than those with mild-moderate neuron loss both before and after surgery. [doi.org]

Progressive Muscular Atrophy

The neuropathological examination revealed not only neuronal loss with gliosis in the facial nucleus, hypoglossal nucleus, and anterior horns of the spinal cord, but also [ncbi.nlm.nih.gov]

Neuropathological examination disclosed not only neuronal loss with gliosis in the hypoglossal nucleus and anterior horns of the spinal cord, but also loss of Betz cells and [ncbi.nlm.nih.gov]

Neuropathologically, the UMN and LMN systems exhibit neuronal loss and gliosis and Bunina bodies are detected in surviving neurons. [bmjopen.bmj.com]

Parenchymatous Cortical Cerebellar Degeneration

Autopsy of the proband’s father showed severe cerebral atrophy, gliosis, and neocortical neuronal loss. The postmortem diagnosis was AD. [alzforum.org]

Intranuclear inclusions, neuronal loss and CAG mosaicism in two patients with Machado-Joseph disease. J Neurol Sci. 2002;15:19-25. Lee Y, Oh MR, Kim CH, et al. [rarediseases.org]

loss and spongiosis Preceded by neuroleptic malignant syndrome Radiation and Chemotherapy Injury may be divided into acute, early delayed injury, late delayed injury Diffuse [neupsykey.com]

Striatonigral Degeneration

SND grade II is characterized by neuronal loss, astrogliosis and presence of GCIs in SNC and posterior/dorsolateral putamen. [ncbi.nlm.nih.gov]

Neuropathologic findings are severe neuronal loss and astrocytic gliosis in the corpus striatum and substantia nigra, with a moderate neuronal loss in the dentate nucleus [ncbi.nlm.nih.gov]

The prominent pathological findings were a severe neuronal loss in the depigmented substantia nigra and in the putamen, which showed a brownish discoloration. [ncbi.nlm.nih.gov]

Neurodegenerative Disorder

Light microscopy revealed severe neuron loss with fibrillary gliosis at both the above-mentioned lobes and the putamen. [ncbi.nlm.nih.gov]

Neurodegenerative disorders, which are chronic and progressive, are characterized by selective and symmetric loss of neurons in motor, sensory, or cognitive systems. [nejm.org]

In six cases, in whom postmortem was performed, neuropathological examination disclosed neuronal loss and widespread Lewy-type pathology in the brain in each case. [ncbi.nlm.nih.gov]

Autosomal Recessive Primary Microcephaly Type 10

loss in central nervous system Congenital onset Cerebral atrophy Acidosis Progressive microcephaly Myopathy Hyperreflexia Optic atrophy Cerebral cortical atrophy Intellectual [mendelian.co]

Neuropathologic examination shows severe loss of neurons as well as neuronal loss of polarity and abnormal dendritic maturation. {ECO:0000269 PubMed:23178126}. [genecards.org]

Neuropathologic examination shows severe loss of neurons as well as neuronal loss of polarity and abnormal dendritic maturation (summary by Yang et al., 2012 ).For a general [mendelian.co]

Progressive Supranuclear Palsy

[…] selected for evaluation of neuronal loss. [doi.org]

In contrast, substantia nigra neuronal loss was greater in PSP/LBD than both PSP and LBD. [ncbi.nlm.nih.gov]

The Purkinje cell layer showed severe neuron loss with Bergmann's gliosis, and the dentate nucleus showed severe neuron loss with grumose degeneration. [ncbi.nlm.nih.gov]

Infantile Neuronal Ceroid Lipofuscinosis

[…] the progressive loss of thalamic neurons that relay different sensory modalities to the cortex. [ncbi.nlm.nih.gov]

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

Early, localised glial activation occurs in all forms of NCL and has been shown to be an accurate predictor of areas in which neuronal loss is most pronounced. [kclpure.kcl.ac.uk]

Neuronal Ceroid Lipofuscinosis

In these disorders, glial (microglial and astrocyte) activation typically occurs early in disease progression and predicts where neuron loss subsequently occurs. [ncbi.nlm.nih.gov]

CLN diseases are rare lysosomal storage diseases characterized by progressive axonal degeneration and neuron loss in the CNS, manifesting in disability, blindness, and premature [ncbi.nlm.nih.gov]

In these cases, the autopsies demonstrated severe atrophy with neuronal loss and gliosis throughout the brain and spinal cord, except the hypothalamic neurons and motor neurons [ncbi.nlm.nih.gov]

Postpoliomyelitis Syndrome

Contributing factors to PPS may be aging (with motor neuron loss), overuse, and disuse. PPS is usually a slowly progressive neuromuscular disease. [ncbi.nlm.nih.gov]

The clinical picture is determined by the extent of motor neuron loss; however, clinically unaffected segments may have suffered substantial viral injury. [journals.lww.com]

A second possible mechanism for PPS is motor neuronal loss due to reactivation of a persistent latent virus. [fmcpaware.org]

Olivopontocerebellar Atrophy

Mild neuronal loss was also found in the anterior vermis and inferior olivary nucleus. [ncbi.nlm.nih.gov]

Neuronal loss and grumose degeneration in the cerebellar dentate nucleus were comparable in CBD-OPCA and PSP. [ncbi.nlm.nih.gov]

Both siblings had identical patterns of neuronal loss consistent with olivopontocerebellar atrophy at postmortem examination. [ncbi.nlm.nih.gov]

Prion Disease

We also demonstrated the ability of TUDCA and UDCA to reduce neuronal loss in prion-infected cerebellar slice cultures. [ncbi.nlm.nih.gov]

Abstract Prion diseases are characterized by accumulation of misfolded protein, gliosis, synaptic dysfunction, and ultimately neuronal loss. [ncbi.nlm.nih.gov]

We found that depleting endogenous neuronal PrPc in mice with established neuroinvasive prion infection reversed early spongiform change and prevented neuronal loss and progression [ncbi.nlm.nih.gov]

Fatal Familial Insomnia

Fatal familial insomnia is characterized by sleep disturbance and loss of neurons, with gliosis in the thalamic nuclei. [ncbi.nlm.nih.gov]

Neuropathological assessment reveals severe neuronal loss and astrogliosis of the anterior medial thalamus and inferior olives, with later cerebral cortical and cerebellar [ncbi.nlm.nih.gov]

Western immunoblot showed a trace of protease-resistant PrP in the thalamus--which had the most significant neuronal loss and gliosis--a moderate amount of PrP in the fronto-temporal [ncbi.nlm.nih.gov]

Lower Motor Neuron Syndrome with Late-Adult Onset

loss in the cord and brain stem- L.M. neuron loss in the cord and brain stem  - bumina bodies in surviving neuron- bumina bodies in surviving neuron  - Betz cells in motor [slideshare.net]

[…] fibers in the fasciculus gracilis and marked loss of neurons with severe gliosis in the dentate nucleus. [emedicine.medscape.com]

Left hippocampus cortex showed neuronal loss as well as cytoplasmic, nuclear and extracellular UI inclusions, in addition to neuronal loss and astrogliosis (not shown). [redalyc.org]

Spinal Muscular Atrophy

Moreover, increased miR-146a was sufficient to induce motor neuron loss in vitro, whereas miR-146a inhibition prevented SMA astrocyte-induced motor neuron loss. [ncbi.nlm.nih.gov]

Infantile spinal muscular atrophy (SMA) caused by homozygous SMN1 gene deletions/mutations is characterized by neuronal loss and axonopathy of motor neurons. [ncbi.nlm.nih.gov]

Autopsy revealed no upper motor neuron and only moderate lower motor neuron loss, but there was distal corticospinal tract degeneration and marked neurogenic muscular atrophy [ncbi.nlm.nih.gov]

Spinal Muscular Atrophy Type 4

Loss of motor neurons leads to a loss of muscle mass, which is translated to muscle atrophy. [peripazienti.roche.it]

They’re missing that SMN protein, and because they’re deficient in SMN, they develop these symptoms of motor neuron loss and weakness. [ajmc.com]

loss in these regions 3. [radiopaedia.org]

Adult-Onset Dystonia - Parkinsonism

The primary treatment is a drug called L-Dopa, which compensates for the neuron loss but eventually becomes less effective as more and more neurons die off. [blogs.brandeis.edu]

When tested in mice, paraquat exposure led to pathological changes and loss of dopamine neurons similar to PD. [dana.org]

loss, Lewy bodies and aberrant neuritis in brainstem, pars compacta and nucleus of Meynert with sparing of the amygdala and locus ceruleus. [es.slideshare.net]