Review
Clinical Neurology
Martina Di Pietro, Mirella Russo, Fedele Dono, Claudia Carrarini, Astrid Thomas, Vincenzo Di Stefano, Roberta Telese, Laura Bonanni, Stefano L. Sensi, Marco Onofrj, Raffaella Franciotti
Summary: Consensus criteria for corticobasal degeneration (CBD) include alien limb (AL) phenomena, but the exact nature of AL behavioral features is still debated. CBD-related AL may manifest as involuntary movements, frontal release phenomena, or somatognosia. To make a proper diagnosis of AL in CBD, cognitive and language dysfunctions must be carefully examined. The term "AL" should be used in conjunction with precise descriptions of accompanying involuntary movements, sensory misperceptions, agnosia-asomatognosia contents, and utilization behavior.
FRONTIERS IN NEUROLOGY
(2021)
Article
Multidisciplinary Sciences
Clement M. Garin, Marie Garin, Leonardo Silenzi, Rye Jaffe, Christos Constantinidis
Summary: The size of the prefrontal cortex in humans is not disproportionately enlarged compared to other catarrhini species. However, humans have the most relatively enlarged frontal and parietal lobes in an infraorder exhibiting a disproportionate expansion of these areas.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Article
Neurosciences
Selene Schintu, Catherine A. Cunningham, Michael Freedberg, Paul Taylor, Stephen J. Gotts, Sarah Shomstein, Eric M. Wassermann
Summary: Hemispatial neglect is believed to be caused by disruption of interhemispheric balance, where right hemisphere lesions deactivate the right frontoparietal network and hyperactivate the left. Evidence from neuropsychological research and TMS studies in healthy subjects support this mechanism. Inhibition of the right PPC leads to neglect-like, rightward visuospatial bias.
Article
Clinical Neurology
Anna E. Goodheart, Joseph J. Locascio, Wesley R. Samore, Jessica A. Collins, Michael Brickhouse, Aaron Schultz, Alexandra Touroutoglou, Keith A. Johnson, Matthew P. Frosch, John H. Growdon, Bradford C. Dickerson, Stephen N. Gomperts
Summary: The study found that corticobasal syndrome patients without amyloid deposition showed higher F-18-AV-1451 retention and regional atrophy, particularly in areas associated with corticobasal degeneration pathology, while cases associated with Alzheimer's disease exhibited greater and more diffuse brain involvement on tau-PET imaging. The differences between the two groups were significant.
Review
Geriatrics & Gerontology
Anna Dunalska, Julia Pikul, Katarzyna Schok, Katarzyna Anna Wiejak, Piotr Alster
Summary: Corticobasal syndrome (CBS) is classified as atypical Parkinsonism characterized by motor and higher cortical dysfunctions. Vascular CBS cases are associated with vascular lesions, particularly internal carotid artery stenosis and ischemic strokes, and often occur in elderly patients with cardiovascular risk factors.
FRONTIERS IN AGING NEUROSCIENCE
(2021)
Review
Cell Biology
Federica Arienti, Giulia Lazzeri, Maria Vizziello, Edoardo Monfrini, Nereo Bresolin, Maria Cristina Saetti, Marina Picillo, Giulia Franco, Alessio Di Fonzo
Summary: CBS is a atypical parkinsonian syndrome with heterogeneous clinical features and different underlying neuropathology. The most common gene involved in CBS is GRN, followed by MAPT, C9ORF72, and PRNP. GRN-CBS patients commonly exhibit symptoms such as visuospatial impairment, behavioral changes, aphasia, and language alterations. Specific demographic, clinical, biochemical, and radiological features may suggest mutations in other genes.
Article
Clinical Neurology
Makoto Sainouchi, Mari Tada, Yusran Ady Fitrah, Norikazu Hara, Kou Tanaka, Jiro Idezuka, Izumi Aida, Takashi Nakajima, Akinori Miyashita, Kohei Akazawa, Takeshi Ikeuchi, Osamu Onodera, Akiyoshi Kakita
Summary: The presence of TDP-43 inclusions in the brains of CBD patients has been correlated with clinical phenotypes. This study investigated the relationship between TDP-43 inclusions and neuronal loss in CBD. The results suggest that TDP-43 alterations in neurons, independent of tau pathology, may contribute to neuronal degeneration in CBD.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
(2022)
Review
Clinical Neurology
Katie A. Peterson, Karalyn Patterson, James B. Rowe
Summary: PSP and CBS can present changes in speech and language alongside or before motor symptoms, making their differential diagnosis challenging in the early stages. Language impairment is often an early and persistent issue in CBS and PSP, highlighting the need for improved language screening and detailed language assessments. Improved language assessment may help in differential diagnosis and inform clinical management decisions.
JOURNAL OF NEUROLOGY
(2021)
Article
Neurosciences
Eric Mooshagian, Eric A. Yttri, Arthur D. Loewy, Lawrence H. Snyder
Summary: The canonical view of motor control states that distal muscles are primarily controlled by the contralateral cerebral hemisphere. However, recent studies have shown that the parietal reach region in the contralateral hemisphere plays a significant role in reach preparation.
JOURNAL OF NEUROSCIENCE
(2022)
Article
Neurosciences
Michaela Kaiserova, Katerina Mensikova, Lucie Tuckova, Petr Hlustik, Petr Kanovsky
Summary: Corticobasal syndrome (CBS) presents with progressive asymmetric rigidity and apraxia, often associated with a wide range of neurodegenerative diseases. This case study highlights a 71-year-old woman with CBS showing concomitant pathology corresponding to Lewy-related and Alzheimer's-type pathology, expanding the spectrum of neurodegenerative pathological processes that may manifest with the typical CBS phenotype. Identifying specific biomarkers is crucial for accurate in vivo differential diagnosis and determining the underlying pathological processes of these diseases.
FRONTIERS IN NEUROSCIENCE
(2021)
Article
Public, Environmental & Occupational Health
Diane M. A. Swallow, Carl E. Counsell
Summary: This study estimated the prevalence of progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) in Scotland, UK. The prevalence rates were similar to previous estimates, with little change over the past 20 years.
Editorial Material
Radiology, Nuclear Medicine & Medical Imaging
Don Gueu Park, Young Sil An, Jung Han Yoon
Summary: This rapidly progressing CBS case presented with only resting tremor but showed distinct FDG PET findings involving unilateral severe anterior frontal cortex, caudate nucleus, and contralateral cerebellum. These findings may aid clinicians in recognizing this fulminant type of corticobasal degeneration since no detailed serial functional imaging study has been performed in rapidly progressing CBS cases so far.
CLINICAL NUCLEAR MEDICINE
(2021)
Article
Geriatrics & Gerontology
Kosei Nakamura, Yasuko Kuroha, Masahiro Hatakeyama, Atsushi Michael Kimura, Yukimi Nakamura, Yoshihiro Murakami, Masaki Watanabe, Hironaka Igarashi, Tetsuya Takahashi, Hitoshi Shimada
Summary: We present here an atypical case of CBS that initially presented with clinical features resembling FCMS. TDP-43 proteinopathy was suspected based on early clinical symptoms, but the clinical course and imaging findings, including tau PET, suggested that the pathological background of the patient was 4-repeat tauopathy.
Article
Clinical Neurology
Jacy Bezerra Parmera, Artur Martins Coutinho, Mateus Rozalem Aranha, Adalberto Studart-Neto, Camila de Godoi Carneiro, Isabel Junqueira de Almeida, Davi J. Fontoura Solla, Carla Rachel Ono, Egberto Reis Barbosa, Ricardo Nitrini, Carlos Alberto Buchpiguel, Sonia Maria Dozzi Brucki
Summary: The study aimed to investigate if different brain metabolism patterns could distinguish CBS due to Alzheimer's disease and other pathologies. The results showed that FDG-PET was useful in predicting CBS variants and depicting their specific degeneration patterns, different clinical features, and brain amyloid deposition.
MOVEMENT DISORDERS
(2021)
Review
Clinical Neurology
Shane Lyons, Dominic Trepel, Tim Lynch, Richard Walsh, Sean O'Dowd
Summary: This study conducted a systematic review to investigate the incidence and prevalence of progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). The findings showed heterogeneity in the epidemiology of these conditions, highlighting the need for further research to understand their true burden.
JOURNAL OF NEUROLOGY
(2023)
Editorial Material
Clinical Neurology
Adrian Budhram, Hugh D. Simpson, James H. Bower, Anhar Hassan, Brittani L. Conway, Eoin P. Flanagan
Article
Clinical Neurology
Anna I. Wernick, Ronald L. Walton, Alexandra I. Soto-Beasley, Shunsuke Koga, Michael G. Heckman, Rebecca R. Valentino, Lukasz M. Milanowski, Dorota Hoffman-Zacharska, Dariusz Koziorowski, Anhar Hassan, Ryan J. Uitti, William P. Cheshire, Wolfgang Singer, Zbigniew K. Wszolek, Dennis W. Dickson, Phillip A. Low, Owen A. Ross
Summary: The occurrence of longer TBP CAG/CAA repeat alleles (>38 repeats) is significantly associated with an increased risk of multiple system atrophy, suggesting a possible genetic overlap between multiple system atrophy and SCA17.
CLINICAL AUTONOMIC RESEARCH
(2021)
Article
Clinical Neurology
Anhar Hassan, Shivam Om Mittal, William T. Hu, Keith A. Josephs, Eric J. Sorenson, J. Eric Ahlskog
Summary: The study showed that pure PLS patients did not convert to ALS after a median of 9 years of disease duration follow-up, with the ascending paraparetic phenotype being most common, presenting with early onset and frequent bladder symptoms. A minority of pure PLS patients develop a more pervasive neurodegenerative disorder after many years.
Article
Clinical Neurology
Tatiana Bremova-Ertl, Jens Claassen, Tomas Foltan, Jordi Gascon-Bayarri, Paul Gissen, Andreas Hahn, Anhar Hassan, Anita Hennig, Simon A. Jones, Miriam Kolnikova, Kyriakos Martakis, Jan Raethjen, Uma Ramaswami, Reena Sharma, Susanne A. Schneider
Summary: The study found that N-acetyl-L-leucine (NALL) has a significant and clinically meaningful improvement in symptoms, functioning, and quality of life in pediatric and adult Niemann-Pick disease type C (NPC) patients, with a favorable safety profile. However, the therapeutic effect was lost after a 6-week washout period.
JOURNAL OF NEUROLOGY
(2022)
Article
Clinical Neurology
Anhar Hassan, Shivam O. Mittal, William T. Hu, Keith A. Josephs, Eric J. Sorenson, J. Eric Ahlskog
Summary: This study assessed whether patients with primary lateral sclerosis (PLS) and limited electromyography (EMG) motor unit denervation changes evolve into amyotrophic lateral sclerosis (ALS). The results showed that 2/21 (10%) of these patients later developed ALS. These patients had a more progressive clinical course compared to previously reported pure PLS cases.
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION
(2022)
Review
Clinical Neurology
Guenther Deuschl, Jos S. Becktepe, Michiel Dirkx, Dietrich Haubenberger, Anhar Hassan, Rick C. Helmich, Muthuraman Muthuraman, Pattamon Panyakaew, Petra Schwingenschuh, Kirsten E. Zeuner, Rodger J. Elble
Summary: The various forms of tremor are now classified based on clinical characteristics and etiology. Electrophysiology, as an extension of clinical examination, is valuable in diagnosing and characterizing different types of tremor. It also plays a crucial role in understanding the pathophysiology of tremor.
CLINICAL NEUROPHYSIOLOGY
(2022)
Review
Clinical Neurology
Sarah C. Lidstone, Michael Costa-Parke, Emily J. Robinson, Tommaso Ercoli, Jon Stone
Summary: Functional movement disorder (FMD) is a common manifestation of functional neurological disorder with diverse phenotypes. This meta-analysis examined the relationship between age at onset, phenotype, and gender in FMD. The study found that women had an earlier age of onset than men, and mixed FMD, tremor, and weakness were the most common phenotypes. The data supports the concept of both grouping FMD as a unitary disorder and splitting it into individual phenotypes.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2022)
Article
Clinical Neurology
Hugh D. Simpson, Joseph R. Duffy, Julie A. G. Stierwalt, J. Eric Ahlskog, Anhar Hassan
Summary: This is a retrospective case series on restricted speech-induced action myoclonus. The study found that misdiagnosis is common in these cases, and diagnostic aids such as modulation with different speech tasks and speaking rates, as well as surface electrophysiology, are useful in confirming the diagnosis.
PARKINSONISM & RELATED DISORDERS
(2022)
Article
Clinical Neurology
Kyriakos Martakis, Jens Claassen, Jordi Gascon-Bayari, Nicolina Goldschagg, Andreas Hahn, Anhar Hassan, Anita Hennig, Simon Jones, Richard Kay, Heather Lau, Susan Perlman, Reena Sharma, Susanne Schneider, Tatiana Bremova-Ertl
Summary: This clinical trial investigated the use of N-acetyl-L-leucine (NALL) in patients with GM2 gangliosidoses, and found significant clinical improvements in patient functioning and quality of life.
Article
Neurosciences
Negin Badihian, Lauren M. Jackson, Bryan T. Klassen, Anhar Hassan, Phillip A. Low, Wolfgang Singer, Elizabeth A. Coon
Summary: The course of patients with multiple system atrophy (MSA) who undergo deep brain stimulation (DBS) remains uncertain. In a retrospective review of 1,496 MSA patients evaluated from 1998-2021, only 12 patients underwent DBS, with most experiencing overall worsening of symptoms following the procedure. Prior to surgery, all patients had red flag signs indicative of atypical parkinsonism.
JOURNAL OF PARKINSONS DISEASE
(2022)
Review
Clinical Neurology
Anhar Hassan
Summary: Episodic ataxia (EA) is a group of rare autosomal dominant inherited disorders characterized by recurrent attacks of cerebellar dysfunction. Recent advances in genetic testing have expanded the phenotypes associated with KCNA1 and CACNA1A mutations, as well as identified EA as a manifestation of other genetic disorders. Secondary causes and mimicking disorders further complicate the diagnosis.
TREMOR AND OTHER HYPERKINETIC MOVEMENTS
(2023)
Article
Immunology
Hussam Tabaja, Jason Yuen, Don Bambino Geno Tai, Cristina Corsini Campioli, Supavit Chesdachai, Daniel C. DeSimone, Anhar Hassan, Bryan T. Klassen, Kai J. Miller, Kendall H. Lee, Maryam Mahmood
Summary: This study retrospectively analyzed the incidence of infection in patients undergoing deep brain stimulation (DBS) procedures between 2000 and 2020. Factors associated with higher infection rates included longer operative time, obesity, male sex, and diabetes. Infections typically occurred within a few months of implantation or revision surgery, and were mainly caused by Staphylococcus and Cutibacterium species. Complete removal of the infected device was associated with better outcomes.
OPEN FORUM INFECTIOUS DISEASES
(2023)
Meeting Abstract
Clinical Neurology
Guillaume Lamotte, Farwa Ali, J. Eric Ahlskog, James Bower, Anhar Hassan, Bradley Boeve, William Cheshire, Dennis Dickson, Joseph Parisi, Keith Josephs, Ann Schmeichel, Phillip Low, Wolfgang Singer, Elizabeth Coon
Meeting Abstract
Clinical Neurology
Negin Badihian, Lauren M. Jackson, Bryan T. Klassen, Anhar Hassan, Phillip A. Low, Wolfgang Singer, Elizabeth A. Coon, Wolfgang Singer
ANNALS OF NEUROLOGY
(2022)
Meeting Abstract
Clinical Neurology
Sushma Kola, Sebastian Armasu, Sudhindra Upadhyaya, Owen Ross, Demetrius Maraganore, Anhar Hassan
