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  1. Article ; Online: Spinal Segmental Myoclonus in Primary Progressive Multiple Sclerosis.

    Ruiz-Ortiz, Mariano / Benito-León, Julián

    Tremor and other hyperkinetic movements (New York, N.Y.)

    2024  Volume 14, Page(s) 7

    Abstract: Background: A wide variety of associated movement disorders has been described in multiple sclerosis.: Phenomenology shown: A 57-year-old woman with primary progressive multiple sclerosis developed spinal segmental myoclonus associated with focal ... ...

    Abstract Background: A wide variety of associated movement disorders has been described in multiple sclerosis.
    Phenomenology shown: A 57-year-old woman with primary progressive multiple sclerosis developed spinal segmental myoclonus associated with focal myelitis.
    Educational value: Movement disorders in multiple sclerosis are phenomenologically diverse and have varied pathophysiological mechanisms, making it essential to identify them to initiate appropriate treatment.
    MeSH term(s) Female ; Humans ; Middle Aged ; Myoclonus/drug therapy ; Myoclonus/etiology ; Multiple Sclerosis/complications ; Multiple Sclerosis, Chronic Progressive/complications ; Multiple Sclerosis, Chronic Progressive/drug therapy ; Spinal Cord Diseases ; Movement Disorders
    Language English
    Publishing date 2024-02-26
    Publishing country England
    Document type Case Reports ; Journal Article
    ZDB-ID 2674453-3
    ISSN 2160-8288 ; 2160-8288
    ISSN (online) 2160-8288
    ISSN 2160-8288
    DOI 10.5334/tohm.862
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  2. Article ; Online: Allodynia, rigidity and gait disturbance.

    Ruiz-Ortiz, Mariano / Díaz-Santiáñez, Mario / Azcárate-Díaz, F Javier / Gonzalo-Martínez, Juan Francisco / Moreno-García, Sara / Ruiz-García, Raquel / Calleja-Castaño, Patricia

    Practical neurology

    2020  

    Language English
    Publishing date 2020-10-14
    Publishing country England
    Document type Journal Article
    ZDB-ID 2170881-2
    ISSN 1474-7766 ; 1474-7758
    ISSN (online) 1474-7766
    ISSN 1474-7758
    DOI 10.1136/practneurol-2020-002669
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  3. Article ; Online: Snorting the Brain Away: Cerebral Damage as an Extension of Cocaine-Induced Midline Destructive Lesions.

    García-Pérez, Daniel / Ruiz-Ortiz, Mariano / Panero, Irene / Eiriz, Carla / Moreno, Luis Miguel / García-Reyne, Ana / García, Alfredo / Martín-Medina, Patricia / Salvador-Álvarez, Elena / Hernández-Lain, Aurelio / Serrano, Antonio / Gil-Etayo, Francisco Javier / Castaño-León, Ana María / Paredes, Igor / Pérez-Núñez, Ángel

    Journal of neuropathology and experimental neurology

    2020  Volume 79, Issue 12, Page(s) 1365–1369

    Abstract: Cocaine consumption is associated with a variety of clinical manifestations. Though cocaine intranasal inhalation always determines nasal mucosal damages, extensive septum perforations, and midline destructions-known as cocaine-induced midline ... ...

    Abstract Cocaine consumption is associated with a variety of clinical manifestations. Though cocaine intranasal inhalation always determines nasal mucosal damages, extensive septum perforations, and midline destructions-known as cocaine-induced midline destructive lesions (CIMDL)-affect only a limited fraction of patients. CIMDL is viewed as a cocaine-associated autoimmune phenomenon in which the presence of atypical anti-neutrophil cytoplasmic antibody (ANCA) promotes and/or defines the disease phenotype. A 51-year-old man presented with an intracranial tumor-like lesion by its space-occupying effect. CT also revealed the destruction of the nasal septum and skull base. A diagnosis of CIMDL was made in light of the patient's history as well as findings of the physical and endoscopic examinations, imaging studies, and laboratory testing. There was no evidence of other pathologies. Histopathological results from cerebral biopsy led us to consider the intracranial pathology as an extension of the CIMDL. CIMDL is the result of a necrotizing inflammatory tissue response triggered by cocaine abuse in a subset of predisposed patients. The reported case is the first CIMDL consistent with brain extension mimicking a tumor-like lesion. While the presence of atypical ANCA seems to promote and/or define the disease phenotype, the specific role of these and other circulating autoantibodies needs further investigation.
    MeSH term(s) Brain/diagnostic imaging ; Brain Injuries/diagnostic imaging ; Brain Injuries/etiology ; Cocaine-Related Disorders/complications ; Cocaine-Related Disorders/diagnostic imaging ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged
    Language English
    Publishing date 2020-11-23
    Publishing country England
    Document type Case Reports ; Journal Article
    ZDB-ID 3088-0
    ISSN 1554-6578 ; 0022-3069
    ISSN (online) 1554-6578
    ISSN 0022-3069
    DOI 10.1093/jnen/nlaa097
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  4. Article ; Online: Humoral and Cellular Responses to SARS-CoV-2 in Convalescent COVID-19 Patients With Multiple Sclerosis.

    Zabalza, Ana / Arrambide, Georgina / Tagliani, Paula / Cárdenas-Robledo, Simón / Otero-Romero, Susana / Esperalba, Juliana / Fernandez-Naval, Candela / Trocoli Campuzano, Jesus / Martínez Gallo, Mónica / Castillo, Mireia / Bonastre, Mercè / Resina Sallés, Mireia / Beltran, Jordina / Carbonell-Mirabent, Pere / Rodríguez-Barranco, Marta / López-Maza, Samuel / Melgarejo Otálora, Pedro José / Ruiz-Ortiz, Mariano / Pappolla, Agustin /
    Rodríguez Acevedo, Breogán / Midaglia, Luciana / Vidal-Jordana, Angela / Cobo-Calvo, Alvaro / Tur, Carmen / Galán, Ingrid / Castilló, Joaquín / Río, Jordi / Espejo, Carmen / Comabella, Manuel / Nos, Carlos / Sastre-Garriga, Jaume / Tintore, Mar / Montalban, Xavier

    Neurology(R) neuroimmunology & neuroinflammation

    2022  Volume 9, Issue 2

    Abstract: Background and objectives: Information about humoral and cellular responses to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and antibody persistence in convalescent (COVID-19) patients with multiple sclerosis (PwMS) is scarce. The ... ...

    Abstract Background and objectives: Information about humoral and cellular responses to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and antibody persistence in convalescent (COVID-19) patients with multiple sclerosis (PwMS) is scarce. The objectives of this study were to investigate factors influencing humoral and cellular responses to SARS-CoV-2 and its persistence in convalescent COVID-19 PwMS.
    Methods: This is a retrospective study of confirmed COVID-19 convalescent PwMS identified between February 2020 and May 2021 by SARS-CoV-2 antibody testing. We examined relationships between demographics, MS characteristics, disease-modifying therapy (DMT), and humoral (immunoglobulin G against spike and nucleocapsid proteins) and cellular (interferon-gamma [IFN-γ]) responses to SARS-CoV-2.
    Results: A total of 121 (83.45%) of 145 PwMS were seropositive, and 25/42 (59.5%) presented a cellular response up to 13.1 months after COVID-19. Anti-CD20-treated patients had lower antibody titers than those under other DMTs (
    Discussion: Humoral and cellular responses to SARS-CoV-2 are present in COVID-19 convalescent PwMS up to 13.10 months after COVID-19. The humoral response decreases under anti-CD20 treatment, although the cellular response can be detected in anti-CD20-treated patients, even in the absence of antibodies.
    MeSH term(s) Adult ; Aged ; Antibodies, Viral/analysis ; Antigens, CD20/immunology ; COVID-19/complications ; COVID-19/immunology ; Female ; Humans ; Immunity, Cellular ; Immunity, Humoral ; Immunoglobulin G/analysis ; Interferon-gamma/biosynthesis ; Interferon-gamma/immunology ; Male ; Middle Aged ; Multiple Sclerosis/complications ; Multiple Sclerosis/immunology ; Nucleocapsid/chemistry ; Nucleocapsid/immunology ; Retrospective Studies
    Chemical Substances Antibodies, Viral ; Antigens, CD20 ; Immunoglobulin G ; Interferon-gamma (82115-62-6)
    Language English
    Publishing date 2022-02-01
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2767740-0
    ISSN 2332-7812 ; 2332-7812
    ISSN (online) 2332-7812
    ISSN 2332-7812
    DOI 10.1212/NXI.0000000000001143
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  5. Article ; Online: The kappa free light chain index and oligoclonal bands have a similar role in the McDonald criteria.

    Arrambide, Georgina / Espejo, Carmen / Carbonell-Mirabent, Pere / Dieli-Crimi, Romina / Rodríguez-Barranco, Marta / Castillo, Mireia / Auger, Cristina / Cárdenas-Robledo, Simón / Castilló, Joaquín / Cobo-Calvo, Álvaro / Galán, Ingrid / Midaglia, Luciana / Nos, Carlos / Otero-Romero, Susana / Río, Jordi / Rodríguez-Acevedo, Breogán / Ruiz-Ortiz, Mariano / Salerno, Annalaura / Tagliani, Paula /
    Tur, Carmen / Vidal-Jordana, Angela / Zabalza, Ana / Sastre-Garriga, Jaume / Rovira, Alex / Comabella, Manuel / Hernández-González, Manuel / Montalban, Xavier / Tintore, Mar

    Brain : a journal of neurology

    2022  Volume 145, Issue 11, Page(s) 3931–3942

    Abstract: Intrathecal production of kappa free light chains occurs in multiple sclerosis and can be measured using the kappa free light chain index. Kappa free light chain index values can be determined more easily than oligoclonal bands detection and seem more ... ...

    Abstract Intrathecal production of kappa free light chains occurs in multiple sclerosis and can be measured using the kappa free light chain index. Kappa free light chain index values can be determined more easily than oligoclonal bands detection and seem more sensitive than the immunoglobulin (Ig)G index to diagnose multiple sclerosis. We assessed the value of oligoclonal bands, kappa free light chain index cut-offs 5.9, 6.6 and 10.61, and IgG index to diagnose multiple sclerosis with prospectively acquired data from a clinically isolated syndrome inception cohort. We selected patients with sufficient data to determine oligoclonal bands positivity, MRI dissemination in space and time, IgG index and sufficient quantities of paired CSF and blood samples to determine kappa free light chain indexes (n = 214). We used Kendall's Tau coefficient to estimate concordance, calculated the number of additional diagnoses when adding each positive index to dissemination in space and positive oligoclonal bands, performed survival analyses for oligoclonal bands and each index with the outcomes second attack and 2017 MRI dissemination in space and time and estimated the diagnostic properties of oligoclonal bands and the different indexes for the previously mentioned outcomes at 5 years. Oligoclonal bands were positive in 138 patients (64.5%), kappa free light chain-5.9 in 136 (63.6%), kappa free light chain-6.6 in 135 (63.1%), kappa free light chain-10.61 in 126 (58.9%) and IgG index in 101 (47.2%). The highest concordance was between oligoclonal bands and kappa free light chain-6.6 (τ = 0.727) followed by oligoclonal bands and kappa free light chain-5.9 (τ = 0.716). Combining dissemination in space plus oligoclonal bands or kappa free light chain-5.9 increased the number of diagnosed patients by 11 (5.1%), with kappa free light chain-6.6 by 10 (4.7%), with kappa free light chain-10.61 by 9 (4.2%) and with IgG index by 3 (1.4%). Patients with positive oligoclonal bands or indexes reached second attack and MRI dissemination in space and time faster than patients with negative results (P < 0.0001 except IgG index in second attack: P = 0.016). In multivariable Cox models [adjusted hazard ratio (95% confidence interval)], the risk for second attack was very similar between kappa free light chain-5.9 [2.0 (0.9-4.3), P = 0.068] and kappa free light chain-6.6 [2.1 (1.1-4.2), P = 0.035]. The highest risk for MRI dissemination in space and time was demonstrated with kappa free light chain-5.9 [4.9 (2.5-9.6), P < 0.0001], followed by kappa free light chain-6.6 [3.4 (1.9-6.3), P < 0.0001]. Kappa free light chains-5.9 and -6.6 had a slightly higher diagnostic accuracy than oligoclonal bands for second attack (70.5, 71.1 and 67.8) and MRI dissemination in space and time (85.7, 85.1 and 81.0). Kappa free light chain indexes 5.9 and 6.6 performed slightly better than oligoclonal bands to assess multiple sclerosis risk and in terms of diagnostic accuracy. Given the concordance between oligoclonal bands and these indexes, we suggest using dissemination in space plus positive oligoclonal bands or positive kappa free light chain index as a modified criterion to diagnose multiple sclerosis.
    MeSH term(s) Humans ; Oligoclonal Bands ; Immunoglobulin kappa-Chains ; Demyelinating Diseases/diagnosis ; Multiple Sclerosis/diagnostic imaging ; Immunoglobulin G
    Chemical Substances Oligoclonal Bands ; Immunoglobulin kappa-Chains ; Immunoglobulin G
    Language English
    Publishing date 2022-06-21
    Publishing country England
    Document type Journal Article
    ZDB-ID 80072-7
    ISSN 1460-2156 ; 0006-8950
    ISSN (online) 1460-2156
    ISSN 0006-8950
    DOI 10.1093/brain/awac220
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  6. Article ; Online: Acute hypokinetic-rigid syndrome following SARS-CoV-2 infection.

    Méndez-Guerrero, Antonio / Laespada-García, María Isabel / Gómez-Grande, Adolfo / Ruiz-Ortiz, Mariano / Blanco-Palmero, Víctor Antonio / Azcarate-Diaz, Francisco Javier / Rábano-Suárez, Pablo / Álvarez-Torres, Eva / de Fuenmayor-Fernández de la Hoz, Carlos Pablo / Vega Pérez, Diana / Rodríguez-Montalbán, Raquel / Pérez-Rivilla, Alfredo / Sayas Catalán, Javier / Ramos-González, Ana / González de la Aleja, Jesús

    Neurology

    2020  Volume 95, Issue 15, Page(s) e2109–e2118

    Abstract: Objective: To report a case of a patient infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) who acutely developed a hypokinetic-rigid syndrome.: Methods: Patient data were obtained from medical records from the Hospital ... ...

    Abstract Objective: To report a case of a patient infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) who acutely developed a hypokinetic-rigid syndrome.
    Methods: Patient data were obtained from medical records from the Hospital Universitario 12 de Octubre in Madrid, Spain. [
    Results: We report a previously healthy 58-year-old man who developed hyposmia, generalized myoclonus, fluctuating and transient changes in level of consciousness, opsoclonus, and an asymmetric hypokinetic-rigid syndrome with ocular abnormalities after a severe SARS-CoV-2 infection. DaT-SPECT confirmed a bilateral decrease in presynaptic dopamine uptake asymmetrically involving both putamina. Significant improvement in the parkinsonian symptoms was observed without any specific treatment.
    Conclusion: This case study provides clinical and functional neuroimaging evidence to support that SARS-CoV-2 can gain access to the CNS, affecting midbrain structures and leading to neurologic signs and symptoms.
    MeSH term(s) Betacoronavirus ; Brain/diagnostic imaging ; Brain/metabolism ; COVID-19 ; Consciousness Disorders ; Coronavirus Infections/complications ; Coronavirus Infections/diagnostic imaging ; Coronavirus Infections/physiopathology ; Disease Progression ; Dopamine Plasma Membrane Transport Proteins/metabolism ; Electroencephalography ; Humans ; Hypokinesia/diagnostic imaging ; Hypokinesia/etiology ; Hypokinesia/physiopathology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Muscle Rigidity/diagnostic imaging ; Muscle Rigidity/etiology ; Muscle Rigidity/physiopathology ; Nortropanes ; Ocular Motility Disorders ; Pandemics ; Parkinson Disease, Postencephalitic/diagnostic imaging ; Parkinson Disease, Postencephalitic/etiology ; Parkinson Disease, Postencephalitic/physiopathology ; Pneumonia, Viral/complications ; Pneumonia, Viral/diagnostic imaging ; Pneumonia, Viral/physiopathology ; Putamen/diagnostic imaging ; Putamen/metabolism ; SARS-CoV-2 ; Tomography, Emission-Computed, Single-Photon
    Chemical Substances Dopamine Plasma Membrane Transport Proteins ; Nortropanes ; ioflupane (VF232WE742)
    Keywords covid19
    Language English
    Publishing date 2020-07-08
    Publishing country United States
    Document type Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't ; Video-Audio Media
    ZDB-ID 207147-2
    ISSN 1526-632X ; 0028-3878
    ISSN (online) 1526-632X
    ISSN 0028-3878
    DOI 10.1212/WNL.0000000000010282
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  7. Article: Acute hypokinetic-rigid syndrome following SARS-CoV-2 infection

    Méndez-Guerrero, Antonio / Laespada-García, María Isabel / Gómez-Grande, Adolfo / Ruiz-Ortiz, Mariano / Blanco-Palmero, Víctor Antonio / Azcarate-Diaz, Francisco Javier / Rábano-Suárez, Pablo / Álvarez-Torres, Eva / de Fuenmayor-Fernández de la Hoz, Carlos Pablo / Vega Pérez, Diana / Rodríguez-Montalbán, Raquel / Pérez-Rivilla, Alfredo / Sayas Catalán, Javier / Ramos-González, Ana / González de la Aleja, Jesús

    Neurology

    Abstract: OBJECTIVE: To report a case of a patient infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) who acutely developed a hypokinetic-rigid syndrome. METHODS: Patient data were obtained from medical records from the Hospital ... ...

    Abstract OBJECTIVE: To report a case of a patient infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) who acutely developed a hypokinetic-rigid syndrome. METHODS: Patient data were obtained from medical records from the Hospital Universitario 12 de Octubre in Madrid, Spain. [123I]-ioflupane dopamine transporter (DaT) SPECT images were acquired 4 hours after a single dose of 185 MBq of 123I-FP-CIT. Quantitative analysis was performed with DaTQUANT software providing the specific binding ratio and z score values of the striatum. RESULTS: We report a previously healthy 58-year-old man who developed hyposmia, generalized myoclonus, fluctuating and transient changes in level of consciousness, opsoclonus, and an asymmetric hypokinetic-rigid syndrome with ocular abnormalities after a severe SARS-CoV-2 infection. DaT-SPECT confirmed a bilateral decrease in presynaptic dopamine uptake asymmetrically involving both putamina. Significant improvement in the parkinsonian symptoms was observed without any specific treatment. CONCLUSION: This case study provides clinical and functional neuroimaging evidence to support that SARS-CoV-2 can gain access to the CNS, affecting midbrain structures and leading to neurologic signs and symptoms.
    Keywords covid19
    Publisher WHO
    Document type Article
    Note WHO #Covidence: #638341
    Database COVID19

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  8. Article ; Online: Acute hypokinetic-rigid syndrome following SARS-CoV-2 infection

    Méndez-Guerrero, Antonio / Laespada-García, María Isabel / Gómez-Grande, Adolfo / Ruiz-Ortiz, Mariano / Blanco-Palmero, Víctor Antonio / Azcarate-Diaz, Francisco Javier / Rábano-Suárez, Pablo / Álvarez-Torres, Eva / de Fuenmayor-Fernández de la Hoz, Carlos Pablo / Pérez, Diana Vega / Rodríguez-Montalbán, Raquel / Pérez-Rivilla, Alfredo / Catalán, Javier Sayas / González, Ana Ramos- / González de la Aleja, Jesús

    Neurology

    2020  , Page(s) 10.1212/WNL.0000000000010282

    Abstract: OBJECTIVE: To report a case of a patient infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) who acutely developed a hypokinetic-rigid syndrome. METHODS: Patient data were obtained from medical records from the “Hospital ... ...

    Abstract OBJECTIVE: To report a case of a patient infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) who acutely developed a hypokinetic-rigid syndrome. METHODS: Patient data were obtained from medical records from the “Hospital Universitario 12 de Octubre” in Madrid, Spain. [ 123 I]-ioflupane dopamine transporter single-photon emission CT (DaT-SPECT) images were acquired 4 hours after a single dose of 185 MBq of 123 I-FP-CIT. Quantitative analysis was performed using DaTQUANT TM software providing the specific binding ratio (SBR) and z-score values of the striatum. RESULTS: We report a previously healthy 58-year-old man who developed hyposmia, generalized myoclonus, fluctuating and transient changes in level of consciousness, opsoclonus and an asymmetric hypokinetic-rigid syndrome with ocular abnormalities after a severe SARS-CoV-2 infection. DaT-SPECT confirmed a bilateral decrease in presynaptic dopamine uptake asymmetrically involving both putamina. Significant improvement in the parkinsonian symptoms was observed without any specific treatment. CONCLUSION: This case study provides clinical and functional neuroimaging evidence to support that SARS-CoV-2 can gain access to the central nervous system, affecting midbrain structures and leading to neurological signs and symptoms.
    Keywords Clinical Neurology ; covid19
    Language English
    Publisher Ovid Technologies (Wolters Kluwer Health)
    Publishing country us
    Document type Article ; Online
    ZDB-ID 207147-2
    ISSN 1526-632X ; 0028-3878
    ISSN (online) 1526-632X
    ISSN 0028-3878
    DOI 10.1212/wnl.0000000000010282
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