scholarly journals Isolated unilateral lingual paralysis in a supranuclear infarction

2021 ◽  
Vol 26 (3) ◽  
pp. 621-622
Author(s):  
Jiyoung Kim ◽  
Kyoung Jin Hwang
Keyword(s):  
Basal Ganglia ◽  
Motor Neuron ◽  
Motor Neurons ◽  
Brain Mri ◽  
Hypoglossal Nucleus ◽  
Focal Lesion ◽  
The Right

Lingual paralysis can result from damage to both upper and lower motor neurons. However, since the hypoglossal nucleus is innervated from both hemispheres simultaneously, unilateral lingual paralysis caused by upper motor neuron has rarely been reported. We report a case which a patient isolated unilateral lingual paralysis due to supranuclear infarction. A 50-year-old right-handed woman was admitted to our hospital due to suddenly developed dysarthria. Her tongue deviated to the right on protrusion without fasciculation or atrophy. A brain MRI showed focal lesion in the left corona radiate and basal ganglia.

scholarly journals Methadone-Induced Toxic Brain Damage

2013 ◽  
Vol 2013 ◽  
pp. 1-2 ◽  
Author(s):  
Jérôme Corré ◽  
Jérôme Pillot ◽  
Gilles Hilbert
Keyword(s):  
Basal Ganglia ◽  
Brain Damage ◽  
Brain Mri ◽  
Cerebellar Hemisphere ◽  
Basal Ganglia Involvement ◽  
Methadone Overdose ◽  
The Right ◽  
Globi Pallidi

A 29-year-old man presented with comatose after methadone intoxication. Cerebral tomography only showed cortico-subcortical hypodense signal in the right cerebellar hemisphere. Brain MRI showed a rare imaging of FLAIR and DWI hyperintensities in the two cerebellar hemispheres as well as basal ganglia (globi pallidi), compatible with methadone overdose. To our knowledge this is the first reported case of both cerebellar and basal ganglia involvement in methadone overdose.

scholarly journals A longitudinal study of the association between basal ganglia volumes and psychomotor symptoms in subjects with late life depression undergoing ECT

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
M. G. A. Van Cauwenberge ◽  
F. Bouckaert ◽  
K. Vansteelandt ◽  
C. Adamson ◽  
F. L. De Winter ◽  
...  
Keyword(s):  
Nucleus Accumbens ◽  
Basal Ganglia ◽  
Caudate Nucleus ◽  
Globus Pallidus ◽  
Brain Mri ◽  
Late Life ◽  
Late Life Depression ◽  
Core Element ◽  
Left Caudate ◽  
The Right

AbstractPsychomotor dysfunction (PMD) is a core element and key contributor to disability in late life depression (LLD), which responds well to electroconvulsive therapy (ECT). The neurobiology of PMD and its response to ECT are not well understood. We hypothesized that PMD in LLD is associated with lower striatal volume, and that striatal volume increase following ECT explains PMD improvement. We analyzed data from a two-center prospective cohort study of 110 LLD subjects (>55 years) receiving ECT. Brain MRI and assessment of mood, cognition, and PMD was performed 1 week before, 1 week after, and 6 months after ECT. Volumetry of the caudate nucleus, putamen, globus pallidus, and nucleus accumbens was derived from automatically segmented brain MRIs using Freesurfer®. Linear multiple regression analyses were used to study associations between basal ganglia volume and PMD. Brain MRI was available for 66 patients 1 week post ECT and in 22 patients also six months post ECT. Baseline PMD was associated with a smaller left caudate nucleus. One week after ECT, PMD improved and volume increases were detected bilaterally in the caudate nucleus and putamen, and in the right nucleus accumbens. Improved PMD after ECT did not relate to the significant volume increases in these structures, but was predicted by a nonsignificant volume change in the right globus pallidus. No volume differences were detected 6 months after ECT, compared to baseline. Although PMD is related to lower striatal volume in LLD, ECT-induced increase of striatal volume does not explain PMD improvement.

Micrographia after thalamo-mesencephalic infarction

Neurology ◽  
1998 ◽  
Vol 51 (2) ◽  
pp. 625-627 ◽  
Author(s):  
J. S. Kim ◽  
J. H. Im ◽  
S. U. Kwon ◽  
J. H. Kang ◽  
M. C. Lee
Keyword(s):  
Basal Ganglia ◽  
Substantia Nigra ◽  
Dopaminergic System ◽  
Brain Mri ◽  
Ischemic Damage ◽  
Anterior Thalamus ◽  
Right Hand ◽  
Ethyl Cysteinate Dimer ◽  
Perfusion Spect ◽  
The Right

A patient with left thalamo-mesencephalic infarction presented with micrographia in the right hand as the only motor sign. Brain MRI and99m Tc ethyl cysteinate dimer (ECD) perfusion SPECT revealed ischemic lesions in the left midbrain and the anterior thalamus, but not in the basal ganglia, whereas [123I]-IPT SPECT demonstrated decreased activity of the [123I]-IPT in the left striatum. The patient's micrographia may be related to a dysfunctional nigrostriatal dopaminergic system secondary to ischemic damage to the substantia nigra.

scholarly journals SUN-684 The Dance of Diabetes

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Aditi Thakkar ◽  
Fatima Jalil ◽  
Pamela Taxel
Keyword(s):  
Blood Glucose ◽  
Basal Ganglia ◽  
Brain Mri ◽  
Rare Condition ◽  
Calcium Deposition ◽  
Focal Lesion ◽  
High Signal ◽  
Insulin Regimen ◽  
Initial Manifestation ◽  
Vitamin Deficiencies

Abstract Nonketotic hyperglycemic chorea-ballism (NKHCB) is a rare movement disorder typically associated with uncontrolled type 2 diabetes. It is often the result of a focal lesion of the contralateral basal ganglia. Here we present a case of a patient who presented with hyperglycemic chorea as the initial manifestation of their diabetes. A 73 year old caucasian female with no known past medical history was brought to the emergency room with involuntary movements of the left face, arm and leg. These movements were sudden in onset and not suppressible. Her vitals were stable on arrival and exam was benign apart from choreiform movements of the left arm, left leg and left perioral muscles. Her labs were significant for a random blood glucose level of 418 mg/dl and HbA1c of 12%. As part of her workup she underwent a CT head which showed an asymmetric hyperdensity involving the right putamen. She underwent additional workup of chorea to exclude infection, vitamin deficiencies, heavy metal poisoning, electrolyte imbalances, liver dysfunction, all of which returned negative. She eventually underwent an MRI of the brain which showed symmetric, mildly bright T1 signal within both putamen suggestive of mild bilateral calcium deposition bilaterally. The patient was further evaluated by neurology who felt that her overall clinical presentation and imaging findings were consistent with a diagnosis of NKHCB. She was placed on an insulin regimen with significant improvement in blood glucose levels. Her symptoms resolved within two weeks as noted on outpatient follow up. NKHCB is a rare condition that manifests in the setting of uncontrolled nonketotic diabetes mellitus. The exact underlying pathophysiology of changes seen on imaging of patients with NKHCB is not fully understood. The diagnosis is based on typical clinical and radiological features including the presence of ballistic or choreiform movements in the setting of marked hyperglycemia and the absence of ketoacidosis. This case is unusual as her chorea was the presenting symptom of her diabetes which was previously undiagnosed. The characteristic radiological feature is a high signal intensity basal ganglia lesion on the T1-weighted brain MRI. The mainstay of treatment includes normalization of the blood glucose, although in some cases antipsychotic use has been described. In most cases, complete resolution of symptoms has been reported within 10 months. Given the excellent prognosis with management of blood glucose, non ketotic hyperglycemia is an important differential to consider in a patient presenting with chorea-hemiballismus.

scholarly journals Expansion of the phenotype in ALS19

2021 ◽  
Author(s):  
Natália Merten Athayde ◽  
Wladimir Bocca Vieira de Rezende Pinto ◽  
Paulo Victor Sgobbi de Souza ◽  
Acary Souza Bulle Oliveira ◽  
Alzira Alves de Siqueira Carvalho
Keyword(s):  
Family History ◽  
Muscle Weakness ◽  
Motor Neurons ◽  
Brain Mri ◽  
Lower Limbs ◽  
Cortical Atrophy ◽  
High Signal ◽  
Pathogenic Variants ◽  
Neurological Exam ◽  
The Right

Context: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects the upper and lower motor neurons. Most cases are sporadic, being 5-10% familial. Currently, more than 20 genes are described as causing familial ALS, with C9ORF72 and SOD1 the most common. Case report: Woman, 42 years old, with progressive weakness in her left foot for 3 years evolving with difficulty walking. No family history. Neurological exam(NE): asymmetric stepagge gait. Asymmetric proximal, distal and axial muscle weakness and distal atrophy. Hyperactive reflexes. EMG: pre-ganglionic lesion in lower limbs with active denervation. Brain MRI: high signal intensity on left corticospinal tract. ALS genetic panel: c.3878G> A, heterozygois in ErbB4(OMIM* 600543). CASE 2: Female, 55 years old, with parkinsonism for 2 years, evolving with muscle weakness, myalgia, dysphonia and dysphagia. After 7 months, respiratory failure and death. Family history: ALS and atypical parkinsonism. NE: Global amyotrophy, facial hypomimia, dropped head, fasciculations on the tongue. Bradykinesia. Plastic hypertonia in the 4 limbs. Proximal and distal weakness. Babinski sign on the right. Oculomotor apraxia. Dysarthrophonia. EMG: pre-ganglionic lesion in the 4 limbs. Brain MRI: global cortical atrophy with temporal predominance. Exome: pathogenic variant, in heterozygosis c. 2428G>A in ErbB4. Conclusion: There are only 3 cases reported in the literature associated to pathogenic variants in this gene. We suggest an expansion of the clinical phenotype for ALS19.

scholarly journals Global transcriptome profile of the developmental principles of in vitro iPSC-to-motor neuron differentiation

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Emilia Solomon ◽  
Katie Davis-Anderson ◽  
Blake Hovde ◽  
Sofiya Micheva-Viteva ◽  
Jennifer Foster Harris ◽  
...  
Keyword(s):  
Stem Cells ◽  
Neural Stem Cells ◽  
Motor Neuron ◽  
Motor Neurons ◽  
Acetylcholine Receptors ◽  
Gene Networks ◽  
Spinal Cord Injuries ◽  
Regulatory Gene ◽  
Transcriptome Profile

Abstract Background Human induced pluripotent stem cells (iPSC) have opened new avenues for regenerative medicine. Consequently, iPSC-derived motor neurons have emerged as potentially viable therapies for spinal cord injuries and neurodegenerative disorders including Amyotrophic Lateral Sclerosis. However, direct clinical application of iPSC bears in itself the risk of tumorigenesis and other unforeseeable genetic or epigenetic abnormalities. Results Employing RNA-seq technology, we identified and characterized gene regulatory networks triggered by in vitro chemical reprogramming of iPSC into cells with the molecular features of motor neurons (MNs) whose function in vivo is to innervate effector organs. We present meta-transcriptome signatures of 5 cell types: iPSCs, neural stem cells, motor neuron progenitors, early motor neurons, and mature motor neurons. In strict response to the chemical stimuli, along the MN differentiation axis we observed temporal downregulation of tumor growth factor-β signaling pathway and consistent activation of sonic hedgehog, Wnt/β-catenin, and Notch signaling. Together with gene networks defining neuronal differentiation (neurogenin 2, microtubule-associated protein 2, Pax6, and neuropilin-1), we observed steady accumulation of motor neuron-specific regulatory genes, including Islet-1 and homeobox protein HB9. Interestingly, transcriptome profiling of the differentiation process showed that Ca2+ signaling through cAMP and LPC was downregulated during the conversion of the iPSC to neural stem cells and key regulatory gene activity of the pathway remained inhibited until later stages of motor neuron formation. Pathways shaping the neuronal development and function were well-represented in the early motor neuron cells including, neuroactive ligand-receptor interactions, axon guidance, and the cholinergic synapse formation. A notable hallmark of our in vitro motor neuron maturation in monoculture was the activation of genes encoding G-coupled muscarinic acetylcholine receptors and downregulation of the ionotropic nicotinic acetylcholine receptors expression. We observed the formation of functional neuronal networks as spontaneous oscillations in the extracellular action potentials recorded on multi-electrode array chip after 20 days of differentiation. Conclusions Detailed transcriptome profile of each developmental step from iPSC to motor neuron driven by chemical induction provides the guidelines to novel therapeutic approaches in the re-construction efforts of muscle innervation.

scholarly journals Lipidomics study of plasma from patients suggest that ALS and PLS are part of a continuum of motor neuron disorders

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Estela Area-Gomez ◽  
D. Larrea ◽  
T. Yun ◽  
Y. Xu ◽  
J. Hupf ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Motor Neurons ◽  
Cell Structure ◽  
Disease Onset ◽  
Point Of View ◽  
Motor Dysfunction ◽  
Cellular Processes ◽  
Progressive Muscle Weakness ◽  
Human Pathology ◽  
Lateral Sclerosis

AbstractMotor neuron disorders (MND) include a group of pathologies that affect upper and/or lower motor neurons. Among them, amyotrophic lateral sclerosis (ALS) is characterized by progressive muscle weakness, with fatal outcomes only in a few years after diagnosis. On the other hand, primary lateral sclerosis (PLS), a more benign form of MND that only affects upper motor neurons, results in life-long progressive motor dysfunction. Although the outcomes are quite different, ALS and PLS present with similar symptoms at disease onset, to the degree that both disorders could be considered part of a continuum. These similarities and the lack of reliable biomarkers often result in delays in accurate diagnosis and/or treatment. In the nervous system, lipids exert a wide variety of functions, including roles in cell structure, synaptic transmission, and multiple metabolic processes. Thus, the study of the absolute and relative concentrations of a subset of lipids in human pathology can shed light into these cellular processes and unravel alterations in one or more pathways. In here, we report the lipid composition of longitudinal plasma samples from ALS and PLS patients initially, and after 2 years following enrollment in a clinical study. Our analysis revealed common aspects of these pathologies suggesting that, from the lipidomics point of view, PLS and ALS behave as part of a continuum of motor neuron disorders.

scholarly journals Poor Corticospinal Motor Neuron Health Is Associated with Increased Symptom Severity in the Acute Phase Following Repetitive Mild TBI and Predicts Early ALS Onset in Genetically Predisposed Rodents

2021 ◽  
Vol 11 (2) ◽  
pp. 160
Author(s):  
Mor R. Alkaslasi ◽  
Noell E. Cho ◽  
Navpreet K. Dhillon ◽  
Oksana Shelest ◽  
Patricia S. Haro-Lopez ◽  
...  
Keyword(s):  
Risk Factor ◽  
Motor Neuron ◽  
Motor Neurons ◽  
Disease Onset ◽  
Poor Health ◽  
Disease Symptoms ◽  
Established Risk Factor ◽  
Early Injury ◽  
Corticospinal Motor Neurons ◽  
Lateral Sclerosis

Traumatic brain injury (TBI) is a well-established risk factor for several neurodegenerative disorders including Alzheimer’s disease and Parkinson’s disease, however, a link between TBI and amyotrophic lateral sclerosis (ALS) has not been clearly elucidated. Using the SOD1G93A rat model known to recapitulate the human ALS condition, we found that exposure to mild, repetitive TBI lead ALS rats to experience earlier disease onset and shortened survival relative to their sham counterparts. Importantly, increased severity of early injury symptoms prior to the onset of ALS disease symptoms was linked to poor health of corticospinal motor neurons and predicted worsened outcome later in life. Whereas ALS rats with only mild behavioral injury deficits exhibited no observable changes in corticospinal motor neuron health and did not present with early onset or shortened survival, those with more severe injury-related deficits exhibited alterations in corticospinal motor neuron health and presented with significantly earlier onset and shortened lifespan. While these studies do not imply that TBI causes ALS, we provide experimental evidence that head injury is a risk factor for earlier disease onset in a genetically predisposed ALS population and is associated with poor health of corticospinal motor neurons.

scholarly journals Gasperini Syndrome: A Case Report and Systematic Review and Proposing a New Definition

2020 ◽  
Vol 12 (3) ◽  
pp. 433-439
Author(s):  
Riwaj Bhagat ◽  
Siddharth Narayanan ◽  
Marwa Elnazeir ◽  
Thong Diep Pham ◽  
Robert Paul Friedland ◽  
...  
Keyword(s):  
Systematic Review ◽  
Brain Mri ◽  
Cranial Nerves ◽  
Clinical Manifestations ◽  
The Other ◽  
Neurological Deficits ◽  
Brainstem Stroke ◽  
Neurological Features ◽  
The Right ◽  
Core Features

Gasperini syndrome (GS), a rare brainstem syndrome, is featured by ipsilateral cranial nerves (CN) V–VIII dysfunction with contralateral hemibody hypoesthesia. While there have been 18 reported cases, the GS definition remains ambiguous. We report a new case and reviewed the clinical features of this syndrome from all published reports to propose a new definition. A 57-year-old man with acute brainstem stroke had right CN V–VIII and XII palsies, left body hypoesthesia and ataxia. Brain MRI showed an acute stroke in the right caudal pons and bilateral cerebellum. After a systematic review, we classified the clinical manifestations into core and associate features based on the frequencies of occurring neurological deficits. We propose that a definitive GS requires the presence of ipsilateral CN VI and VII palsies, plus one or more of the other three core features (ipsilateral CN V, VIII palsies and contralateral hemibody hemihypalgesia). Additionally, GS, similar to Wallenberg’s syndrome, represents a spectrum that can have other associated neurological features. The revised definition presented in this study may enlighten physicians with the immediate recognition of the syndrome and help improve clinical localization of the lesions and its management.

scholarly journals Coexistence of neuronal intranuclear inclusion disease and amyotrophic lateral sclerosis: an autopsy case

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Atsuhiko Sugiyama ◽  
Takahiro Takeda ◽  
Mizuho Koide ◽  
Hajime Yokota ◽  
Hiroki Mukai ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Repeat Expansion ◽  
Cerebellar Hemisphere ◽  
Intranuclear Inclusions ◽  
Intranuclear Inclusion ◽  
Neuronal Intranuclear Inclusion ◽  
Lateral Sclerosis

Abstract Background Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease. Pathologically, it is characterized by eosinophilic hyaline intranuclear inclusions in the cells of the visceral organs as well as central, peripheral, and autonomic nervous system cells. Recently, a GGC repeat expansion in the NOTCH2NLC gene has been identified as the etiopathological agent of NIID. Interestingly, this GGC repeat expansion was also reported in some patients with a clinical diagnosis of amyotrophic lateral sclerosis (ALS). However, there are no autopsy-confirmed cases of concurrent NIID and ALS. Case presentation A 60-year-old Taiwanese woman reported a four-month history of progressive weakness beginning in the right foot that spread to all four extremities. She was diagnosed with ALS because she met the revised El Escorial diagnostic criteria for definite ALS with upper and lower motor neuron involvement in the cervical, thoracic, and lumbosacral regions. She died of respiratory failure at 22 months from ALS onset, at the age of 62 years. Brain magnetic resonance imaging (MRI) revealed lesions in the medial part of the cerebellar hemisphere, right beside the vermis (paravermal lesions). The subclinical neuropathy, indicated by a nerve conduction study (NCS), prompted a potential diagnosis of NIID. Antemortem skin biopsy and autopsy confirmed the coexistence of pathology consistent with both ALS and NIID. We observed neither eccentric distribution of p62-positive intranuclear inclusions in the areas with abundant large motor neurons nor cytopathological coexistence of ALS and NIID pathology in motor neurons. This finding suggested that ALS and NIID developed independently in this patient. Conclusions We describe a case of concurrent NIID and ALS discovered during an autopsy. Abnormal brain MRI findings, including paravermal lesions, could indicate the coexistence of NIID even in patients with ALS showing characteristic clinical phenotypes.

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