Neuroleptic Malignant Syndrome Associated with Refractory Acute Disseminated Encephalomyelitis

We present the case of a young man who was transferred to our hospital with worsening acute disseminated encephalomyelitis (ADEM) despite treatment with intravenous methylprednisolone, intravenous immunoglobulin and plasma exchange. He developed neuroleptic malignant syndrome (NMS) without the use of dopamine-modulating drugs. His progressive clinical improvement started after treatment with intravenous cyclophosphamide and methylprednisolone. In our patient, acute demyelination with severe bilateral inflammation of the basal ganglia could have caused a state of central dopamine depletion, creating proper conditions for the development of NMS. Significant clinical improvement of our case after treatment with intravenous cyclophosphamide and steroids provides further evidence for a possible role of the inflammatory lesions in the pathogenesis of NMS in association with ADEM.

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Aberrant striatal oscillations after dopamine loss in parkinsonian non-human primates

10.1101/650770 ◽

2019 ◽

Cited By ~ 1

Author(s):

Arun Singh ◽

Stella M. Papa

Keyword(s):

Basal Ganglia ◽

Oscillatory Activity ◽

Field Potentials ◽

Striatal Neurons ◽

Dopamine Depletion ◽

Frequency Bands ◽

Oscillatory Pattern ◽

Motor Abnormalities ◽

Beta Frequency

AbstractDopamine depletion in Parkinson’s disease (PD) is associated with abnormal oscillatory activity in the cortico-basal ganglia network. However, the oscillatory pattern of striatal neurons in PD remains poorly defined. Here, we analyzed the local field potentials in one untreated and five MPTP-treated non-human primates (NHP) to model advanced PD. Augmented oscillatory activity in the alpha (8-13 Hz) and low-beta (13-20 Hz) frequency bands was found in the striatum in parallel to the motor cortex and globus pallidus of the NHP-PD model. The coherence analysis showed increased connectivity in the cortico-striatal and striato-pallidal pathways at alpha and low-beta frequency bands, confirming the presence of abnormal 8-20 Hz activity in the cortico-basal ganglia network. The acute L-Dopa injection that induced a clear motor response normalized the amplified 8-20 Hz oscillations. These findings indicate that pathological striatal oscillations at alpha and low-beta bands are concordant with the basal ganglia network changes after dopamine depletion, and thereby support a key role of the striatum in the generation of parkinsonian motor abnormalities.

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Untangling Basal Ganglia Network Dynamics and Function: Role of Dopamine Depletion and Inhibition Investigated in a Spiking Network Model

eNeuro ◽

10.1523/eneuro.0156-16.2016 ◽

2016 ◽

Vol 3 (6) ◽

pp. ENEURO.0156-16.2016 ◽

Cited By ~ 19

Author(s):

Mikael Lindahl ◽

Jeanette Hellgren Kotaleski

Keyword(s):

Basal Ganglia ◽

Network Model ◽

Network Dynamics ◽

Dopamine Depletion ◽

Spiking Network ◽

Dynamics And Function ◽

And Function

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A Biologically Inspired Computational Model of Basal Ganglia in Action Selection

Computational Intelligence and Neuroscience ◽

10.1155/2015/187417 ◽

2015 ◽

Vol 2015 ◽

pp. 1-24 ◽

Cited By ~ 10

Author(s):

Chiara Baston ◽

Mauro Ursino

Keyword(s):

Basal Ganglia ◽

Previous History ◽

Action Selection ◽

Dopamine Depletion ◽

Behavioral Experiments ◽

Biologically Inspired ◽

Cholinergic Interneurons ◽

Hebb Rule ◽

History Of

The basal ganglia (BG) are a subcortical structure implicated in action selection. The aim of this work is to present a new cognitive neuroscience model of the BG, which aspires to represent a parsimonious balance between simplicity and completeness. The model includes the 3 main pathways operating in the BG circuitry, that is, the direct (Go), indirect (NoGo), and hyperdirect pathways. The main original aspects, compared with previous models, are the use of a two-term Hebb rule to train synapses in the striatum, based exclusively on neuronal activity changes caused by dopamine peaks or dips, and the role of the cholinergic interneurons (affected by dopamine themselves) during learning. Some examples are displayed, concerning a few paradigmatic cases: action selection in basal conditions, action selection in the presence of a strong conflict (where the role of the hyperdirect pathway emerges), synapse changes induced by phasic dopamine, and learning new actions based on a previous history of rewards and punishments. Finally, some simulations show model working in conditions of altered dopamine levels, to illustrate pathological cases (dopamine depletion in parkinsonian subjects or dopamine hypermedication). Due to its parsimonious approach, the model may represent a straightforward tool to analyze BG functionality in behavioral experiments.

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Anti-Myelin Oligodendrocyte Glycoprotein Encephalomyelitis and Extensive Longitudinal Transverse Myelitis Associated with Compound Heterozygous NLRP3 Missense Mutations in a Young Child

Journal of Pediatric Neurology ◽

10.1055/s-0040-1721434 ◽

2020 ◽

Author(s):

Deirdre O'Sullivan ◽

Michael Moore ◽

Susan Byrne ◽

Andreas O. Reiff ◽

Susanna Felsenstein

Keyword(s):

Young Child ◽

Genetic Basis ◽

Transverse Myelitis ◽

Acute Disseminated Encephalomyelitis ◽

Myelin Oligodendrocyte Glycoprotein ◽

Compound Heterozygous ◽

Missense Mutations ◽

Childhood Onset

AbstractAcute disseminated encephalomyelitis in association with extensive longitudinal transverse myelitis is reported in a young child with positive anti-myelin oligodendrocyte glycoprotein (MOG) antibody with heterozygous NLRP3 missense mutations; p.(Arg488Lys) and p.(Ser159Ile). This case may well present an exceptional coincidence, but may describe a yet unrecognized feature of the spectrum of childhood onset cryopyrinopathies that contribute to the understanding of the genetic basis for anti-MOG antibody positive encephalomyelitis. Based on this observation, a larger scale study investigating the role of NLRP3 and other inflammasomes in this entity would provide important pathophysiological insights and potentially novel avenues for treatment.

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The Role of the Basal Ganglia in Somatosensory-Motor Interactions: Evidence from Neurophysiology and Behavior

10.3389/978-2-88963-475-0 ◽

2020 ◽

Keyword(s):

Basal Ganglia ◽

And Behavior

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Faculty Opinions recommendation of Assessment of bone synthetic activity in inflammatory lesions and syndesmophytes in patients with ankylosing spondylitis: the potential role of 18F-fluoride positron emission tomography-magnetic resonance imaging.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.725398590.793505223 ◽

2015 ◽

Author(s):

Conny van der Laken

Keyword(s):

Magnetic Resonance Imaging ◽

Positron Emission Tomography ◽

Potential Role ◽

Emission Tomography ◽

Synthetic Activity ◽

Resonance Imaging ◽

Inflammatory Lesions ◽

Positron Emission ◽

18F Fluoride

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Role of MRI evaluation in acute secondary inability to walk in children

Egyptian Journal of Radiology and Nuclear Medicine ◽

10.1186/s43055-021-00417-0 ◽

2021 ◽

Vol 52 (1) ◽

Author(s):

K. H. Sedeek ◽

K. Aboualfotouh ◽

S. M. Hassanein ◽

N. M. Osman ◽

M. H. Shalaby

Keyword(s):

Transverse Myelitis ◽

High Sensitivity ◽

Acute Disseminated Encephalomyelitis ◽

Limb Weakness ◽

Non Invasive ◽

Highly Sensitive ◽

Common Causes ◽

Bilateral Lower Limb ◽

Severity Of The Disease

Abstract Background Acute bilateral lower limb weakness is a common problem in children which necessitates a rapid method for diagnosis. MRI is a non-invasive imaging technique that produces high-quality images of the internal structure of the brain and spinal cord. Results MRI was very helpful in reaching rapid and prompt diagnosis in children with acute inability to walk. Acute disseminated encephalomyelitis (ADEM), Guillain–Barré syndrome (GBS), and acute transverse myelitis (ATM) were the most common causes in our study. MRI proved to be of high sensitivity in detecting the lesions and reaching the diagnosis in ADEM and GBS; however, there was no significant relation between the lesions’ size, enhancement pattern, and severity of the disease or prognosis, yet in ATM the site of the lesion and number of cord segment affection were significantly related to the severity of the disease and prognosis. Conclusion MRI is a quick tool to reach the diagnosis of children with acute secondary inability to walk, and to eliminate other differential diagnosis which is essential for proper treatment and rapid full recovery. It is highly sensitive in detecting the lesions, their site and size.

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Expression of FoxP2 in the basal ganglia regulates vocal motor sequences in the adult songbird

Nature Communications ◽

10.1038/s41467-021-22918-2 ◽

2021 ◽

Vol 12 (1) ◽

Author(s):

Lei Xiao ◽

Devin P. Merullo ◽

Therese M. I. Koch ◽

Mou Cao ◽

Marissa Co ◽

...

Keyword(s):

Transcription Factor ◽

Basal Ganglia ◽

Dopamine Receptor ◽

Speech Disorders ◽

Receptor Expression ◽

Vocal Development ◽

Motor Actions

AbstractDisruption of the transcription factor FoxP2, which is enriched in the basal ganglia, impairs vocal development in humans and songbirds. The basal ganglia are important for the selection and sequencing of motor actions, but the circuit mechanisms governing accurate sequencing of learned vocalizations are unknown. Here, we show that expression of FoxP2 in the basal ganglia is vital for the fluent initiation and termination of birdsong, as well as the maintenance of song syllable sequencing in adulthood. Knockdown of FoxP2 imbalances dopamine receptor expression across striatal direct-like and indirect-like pathways, suggesting a role of dopaminergic signaling in regulating vocal motor sequencing. Confirming this prediction, we show that phasic dopamine activation, and not inhibition, during singing drives repetition of song syllables, thus also impairing fluent initiation and termination of birdsong. These findings demonstrate discrete circuit origins for the dysfluent repetition of vocal elements in songbirds, with implications for speech disorders.

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