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.
- ↑ 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.