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=== esclerodermia ===
=== scleroderma ===
La esclerosis sistémica (esclerodermia, SSc) es una enfermedad autoinmune multisistémica rara caracterizada por autoanticuerpos, vasculopatía y fibrosis de la piel y los órganos internos, pero una revisión sistemática de la participación neurológica en la esclerosis sistémica (SSc) y la esclerodermia localizada (LS) es útil, describiendo las características clínicas, neuroimagen y tratamiento.  
Systemic sclerosis (scleroderma, SSc) is a rare multisystem autoimmune disease characterized by autoantibodies, vasculopathy, and fibrosis of the skin and internal organs, but a systematic review of the neurological involvement in systemic sclerosis (SSc) and localized scleroderma (LS) ) is useful, describing the clinical characteristics, neuroimaging and treatment.  


Un estudio de Tiago Nardi Amaral et al.l.<ref>Tiago Nardi Amaral, Fernando Augusto Peres, Aline Tamires Lapa, João Francisco Marques-Neto, Simone Appenzeller. [https://pubmed.ncbi.nlm.nih.gov/23827688/ Neurologic involvement in scleroderma: a systematic review] Semin Arthritis Rheum. 2013 Dec;43(3):335-47. doi: 10.1016/ j.semarthrit. 2013.05.002. Epub 2013 Jul 1.</ref> en una búsqueda bibliográfica en PubMed utilizando los siguientes términos MeSH, esclerodermia, esclerosis sistémica, esclerodermia localizada, esclerodermia localizada "en golpe de sable", síndrome de Parry-Romberg, deterioro cognitivo, memoria, convulsiones, epilepsia, dolor de cabeza, depresión, ansiedad, trastornos del estado de ánimo , Centro de Estudios Epidemiológicos Depresión (CES-D), SF-36, Inventario de Depresión de Beck (BDI), Inventario de Ansiedad de Beck (BAI), Cuestionario de Salud del Paciente-9 (PHQ-9), neuropsiquiatría, psicosis, afectación neurológica, neuropatía, nervios periféricos, nervios craneales, síndrome del túnel carpiano, atrapamiento cubital, síndrome del túnel tarsiano, mononeuropatía, polineuropatía, radiculopatía, mielopatía, sistema nervioso autónomo, sistema nervioso, electroencefalografía (EEG), electromiografía (EMG), resonancia magnética nuclear (RMN) y La angiorresonancia magnética (ARM) llegó a las siguientes conclusiones: Se identificaron un total de 182 informes de casos/estudios con respecto a SSc y 50 referidos a SL.<blockquote>En LS predominaron las convulsiones (41,58%) y la cefalea (18,81%). Sin embargo, se han hecho descripciones de varios nervios craneales y hemiparesia. La afectación del sistema nervioso central en el SSC se caracterizó por cefalea (23,73%), convulsiones (13,56%) y deterioro cognitivo (8,47%). Se observaron con frecuencia depresión y ansiedad (73,15% y 23,95%, respectivamente). La miopatía (51,8%), la neuropatía del trigémino (16,52%), la polineuropatía sensitivomotora periférica (14,25%) y el síndrome del túnel carpiano (6,56%) fueron la afectación del sistema nervioso periférico más frecuente en la ES. </blockquote>Pero esto no es todo porque existen algunas variantes de la esclerodermia como la Morfea.
A study by Tiago Nardi Amaral et al.<ref>Tiago Nardi Amaral, Fernando Augusto Peres, Aline Tamires Lapa, João Francisco Marques-Neto, Simone Appenzeller. [https://pubmed.ncbi.nlm.nih.gov/23827688/ Neurologic involvement in scleroderma: a systematic review] Semin Arthritis Rheum. 2013 Dec;43(3):335-47. doi: 10.1016/ j.semarthrit. 2013.05.002. Epub 2013 Jul 1.</ref> in a PubMed literature search using the following MeSH terms, scleroderma, systemic sclerosis, localized scleroderma, localized saber-stroke scleroderma, Parry-Romberg syndrome, cognitive impairment, memory, seizures, epilepsy, headache, depression, anxiety, mood disorders, Center for Epidemiological Studies Depression (CES-D), SF-36, Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Patient Health Questionnaire-9 (PHQ -9), neuropsychiatry, psychosis, neurological impairment, neuropathy, peripheral nerves, cranial nerves, carpal tunnel syndrome, ulnar entrapment, tarsal tunnel syndrome, mononeuropathy, polyneuropathy, radiculopathy, myelopathy, autonomic nervous system, nervous system, electroencephalography (EEG) ), electromyography (EMG), magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA) reached the following conclusions: A total of 182 case reports/studies regarding SSc and 50 referring to SL were identified.<blockquote>In LS, seizures (41.58%) and headache (18.81%) predominated. However, descriptions of various cranial nerves and hemiparesis have been made. Central nervous system involvement in CSS was characterized by headache (23.73%), seizures (13.56%), and cognitive impairment (8.47%). Depression and anxiety were frequently observed (73.15% and 23.95%, respectively). Myopathy (51.8%), trigeminal neuropathy (16.52%), peripheral sensorimotor polyneuropathy (14.25%) and carpal tunnel syndrome (6.56%) were the most common peripheral nervous system involvement. common in the ES. </blockquote>But this is not all because there are some variants of scleroderma such as Morphea.


==== Morfea ====
==== morphea ====
La morfea es una forma de esclerodermia que se presenta con parches aislados de piel endurecida en la cara, las manos y los pies o en cualquier otra parte del cuerpo, sin afectación de los órganos internos. La morfea se presenta con mayor frecuencia como máculas o placas de unos pocos centímetros de diámetro, pero también puede aparecer como bandas o en lesiones guttatas o nódulos. <ref>James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. Page 171. <nowiki>ISBN 0-7216-2921-0</nowiki>.</ref>La morfea es un engrosamiento y endurecimiento de la piel y los tejidos subcutáneos debido a la deposición excesiva de colágeno.  
Morphea is a form of scleroderma that presents with isolated patches of hardened skin on the face, hands and feet, or elsewhere on the body, without involvement of internal organs. Morphea most often presents as macules or plaques a few centimeters in diameter, but it can also appear as bands or in guttate lesions or nodules. <ref>James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. Page 171. <nowiki>ISBN 0-7216-2921-0</nowiki>.</ref>Morphea is a thickening and hardening of the skin and subcutaneous tissues due to excessive collagen deposition.  


La morfea abarca condiciones específicas que van desde placas muy pequeñas que involucran solo la piel hasta enfermedades generalizadas que causan deformidades funcionales y estéticas. La morfea se diferencia de la esclerosis sistémica por su presunta falta de compromiso de los órganos internos..<ref>James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. Page 171. <nowiki>ISBN 0-7216-2921-0</nowiki>.</ref><blockquote>Desafortunadamente, el camino sigue siendo difícil porque la larga serie de variantes no excluye una forma de "espasmo hemimasticatorio" de Morfea, como bien lo describen HJ Kim et al..<ref>H J Kim, B S Jeon, K W Lee. [https://pubmed.ncbi.nlm.nih.gov/10768634/ Hemimasticatory spasm associated with localized scleroderma and facial hemiatrophy].Arch Neurol. 2000 Apr;57(4):576-80. doi: 10.1001/archneur.57.4.576.
Morphea encompasses specific conditions ranging from very small plaques involving only the skin to generalized diseases causing functional and cosmetic deformities. Morphea differs from systemic sclerosis by its presumed lack of involvement of internal organs..<ref>James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. Page 171. <nowiki>ISBN 0-7216-2921-0</nowiki>.</ref><blockquote>Unfortunately, the path remains difficult because the long series of variants does not exclude a form of "hemimasticatory spasm" of Morphea, as well described by HJ Kim et al. al..<ref>H J Kim, B S Jeon, K W Lee. [https://pubmed.ncbi.nlm.nih.gov/10768634/ Hemimasticatory spasm associated with localized scleroderma and facial hemiatrophy].Arch Neurol. 2000 Apr;57(4):576-80. doi: 10.1001/archneur.57.4.576.
</ref> en el que se afirma que en base a resultados clínicos y electrofisiológicos del trigémino como el reflejo de parpadeo, el reflejo mandibular y el periodo de silencio maseterino, se sugiere como causa la desmielinización focal de las ramas motoras del nervio trigémino por alteraciones de los tejidos profundos de actividad eléctrica movimientos excitatorios anormales que resultan en movimientos masticatorios involuntarios y espasmos.</blockquote>La última afirmación indica una participación de actividades eléctricas excitatorias fisiológicas y enfáticas.
</ref> in which it is stated that based on clinical and electrophysiological results of the trigeminal, such as the blink reflex, the jaw reflex and the masseteric silent period, focal demyelination of the motor branches of the trigeminal nerve due to tissue alterations is suggested as the cause. Profound electrical activity abnormal excitatory movements that result in involuntary chewing movements and spasms.</blockquote>The latter statement indicates a involvement of physiological and emphatic excitatory electrical activities.

Revision as of 10:17, 1 May 2023

scleroderma

Systemic sclerosis (scleroderma, SSc) is a rare multisystem autoimmune disease characterized by autoantibodies, vasculopathy, and fibrosis of the skin and internal organs, but a systematic review of the neurological involvement in systemic sclerosis (SSc) and localized scleroderma (LS) ) is useful, describing the clinical characteristics, neuroimaging and treatment.

A study by Tiago Nardi Amaral et al.[1] in a PubMed literature search using the following MeSH terms, scleroderma, systemic sclerosis, localized scleroderma, localized saber-stroke scleroderma, Parry-Romberg syndrome, cognitive impairment, memory, seizures, epilepsy, headache, depression, anxiety, mood disorders, Center for Epidemiological Studies Depression (CES-D), SF-36, Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Patient Health Questionnaire-9 (PHQ -9), neuropsychiatry, psychosis, neurological impairment, neuropathy, peripheral nerves, cranial nerves, carpal tunnel syndrome, ulnar entrapment, tarsal tunnel syndrome, mononeuropathy, polyneuropathy, radiculopathy, myelopathy, autonomic nervous system, nervous system, electroencephalography (EEG) ), electromyography (EMG), magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA) reached the following conclusions: A total of 182 case reports/studies regarding SSc and 50 referring to SL were identified.

In LS, seizures (41.58%) and headache (18.81%) predominated. However, descriptions of various cranial nerves and hemiparesis have been made. Central nervous system involvement in CSS was characterized by headache (23.73%), seizures (13.56%), and cognitive impairment (8.47%). Depression and anxiety were frequently observed (73.15% and 23.95%, respectively). Myopathy (51.8%), trigeminal neuropathy (16.52%), peripheral sensorimotor polyneuropathy (14.25%) and carpal tunnel syndrome (6.56%) were the most common peripheral nervous system involvement. common in the ES.

But this is not all because there are some variants of scleroderma such as Morphea.

morphea

Morphea is a form of scleroderma that presents with isolated patches of hardened skin on the face, hands and feet, or elsewhere on the body, without involvement of internal organs. Morphea most often presents as macules or plaques a few centimeters in diameter, but it can also appear as bands or in guttate lesions or nodules. [2]Morphea is a thickening and hardening of the skin and subcutaneous tissues due to excessive collagen deposition.

Morphea encompasses specific conditions ranging from very small plaques involving only the skin to generalized diseases causing functional and cosmetic deformities. Morphea differs from systemic sclerosis by its presumed lack of involvement of internal organs..[3]

Unfortunately, the path remains difficult because the long series of variants does not exclude a form of "hemimasticatory spasm" of Morphea, as well described by HJ Kim et al. al..[4] in which it is stated that based on clinical and electrophysiological results of the trigeminal, such as the blink reflex, the jaw reflex and the masseteric silent period, focal demyelination of the motor branches of the trigeminal nerve due to tissue alterations is suggested as the cause. Profound electrical activity abnormal excitatory movements that result in involuntary chewing movements and spasms.

The latter statement indicates a involvement of physiological and emphatic excitatory electrical activities.

  1. Tiago Nardi Amaral, Fernando Augusto Peres, Aline Tamires Lapa, João Francisco Marques-Neto, Simone Appenzeller. Neurologic involvement in scleroderma: a systematic review Semin Arthritis Rheum. 2013 Dec;43(3):335-47. doi: 10.1016/ j.semarthrit. 2013.05.002. Epub 2013 Jul 1.
  2. James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 171. ISBN 0-7216-2921-0.
  3. James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 171. ISBN 0-7216-2921-0.
  4. H J Kim, B S Jeon, K W Lee. Hemimasticatory spasm associated with localized scleroderma and facial hemiatrophy.Arch Neurol. 2000 Apr;57(4):576-80. doi: 10.1001/archneur.57.4.576.