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=== | === 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 neurological involvement in systemic sclerosis (SSc) and localized scleroderma (LS) but is useful, describing the clinical features, neuroimaging and treatment. | |||
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 terms MeSH, scleroderma, systemic sclerosis, localized scleroderma, localized scleroderma "en coup de saber", 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 involvement, 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) came to the following conclusions: A total of 182 case reports / studies were identified regarding SSc and 50 referred to LS.<blockquote>In LS, seizures (41.58%) and headache (18.81%) predominated. However, descriptions of various cranial nerve and hemiparesis involvement have been made. Central nervous system involvement in the SSC 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 frequent involvement of the peripheral nervous system in the SSc. </blockquote>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 anywhere else on the body, without involvement of internal organs. Morphea most often occurs as macules or plaques a few centimeters in diameter, but 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 the excessive deposition of collagen . | |||
Morphea encompasses specific conditions ranging from very small plaques that involve only the skin to widespread diseases that cause functional and aesthetic 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 is still difficult because the long series of variants does not exclude a form of 'Hemimasticatory spasm' from Morfea as well described by H J 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. | |||
</ref> | </ref> in which it is asserted that on the basis of clinical and electrophysiological trigeminal results such as blink reflex, jaw jerk and the masseterine silent period, focal demyelination of the motor branches of the trigeminal nerve due to alterations of the deep tissues is suggested as a cause of electrical activity abnormal excitatory movements resulting in involuntary masticatory movement and spasm.</blockquote>The latter assertion indicates an involvement of physiological and emphatic excitatory electrical activities. | ||
Latest revision as of 11:45, 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 neurological involvement in systemic sclerosis (SSc) and localized scleroderma (LS) but is useful, describing the clinical features, neuroimaging and treatment.
A study by Tiago Nardi Amaral et al.[1] in a PubMed literature search using the following terms MeSH, scleroderma, systemic sclerosis, localized scleroderma, localized scleroderma "en coup de saber", 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 involvement, 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) came to the following conclusions: A total of 182 case reports / studies were identified regarding SSc and 50 referred to LS.
In LS, seizures (41.58%) and headache (18.81%) predominated. However, descriptions of various cranial nerve and hemiparesis involvement have been made. Central nervous system involvement in the SSC 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 frequent involvement of the peripheral nervous system in the SSc.
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 anywhere else on the body, without involvement of internal organs. Morphea most often occurs as macules or plaques a few centimeters in diameter, but 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 the excessive deposition of collagen .
Morphea encompasses specific conditions ranging from very small plaques that involve only the skin to widespread diseases that cause functional and aesthetic deformities. Morphea differs from systemic sclerosis by its presumed lack of involvement of internal organs.[3]
Unfortunately the path is still difficult because the long series of variants does not exclude a form of 'Hemimasticatory spasm' from Morfea as well described by H J Kim et al.[4] in which it is asserted that on the basis of clinical and electrophysiological trigeminal results such as blink reflex, jaw jerk and the masseterine silent period, focal demyelination of the motor branches of the trigeminal nerve due to alterations of the deep tissues is suggested as a cause of electrical activity abnormal excitatory movements resulting in involuntary masticatory movement and spasm.
The latter assertion indicates an involvement of physiological and emphatic excitatory electrical activities.
- ↑ 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.
- ↑ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 171. ISBN 0-7216-2921-0.
- ↑ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 171. ISBN 0-7216-2921-0.
- ↑ 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.