Modifica di Hemimasticatory spasm (sezione)

===Scleroderma ===
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> described the clinical characteristics, neuroimaging, and treatment of neurological involvement in systemic sclerosis (SSc) and localized scleroderma (LS) through a systematic review  

The authors carried out a literature search in PubMed using the following MeSH terms, scleroderma, systemic sclerosis, localized scleroderma, localized scleroderma "en coup de sabre", Parry-Romberg syndrome, cognitive impairment, memory, seizures, epilepsy, headache , depression, anxiety, mood disorders, Center for Epidemiological Studies in 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). Patients with other connective tissue diseases responsible for nervous system involvement were excluded from the analyses.

A total of 182 case reports/studies addressing SSc and 50 reporting LS were identified. The total number of patients with SSc was 9,506, while data were available on 224 patients with LS. In LS, convulsions (41.58%) and headache (18.81%) predominated. However, descriptions of various cranial nerve involvement and hemiparesis have been made. Central Nervous System involvement in 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 peripheral nervous system involvement in SSc. Autonomic neuropathy involving the cardiovascular and gastrointestinal systems has been regularly described. The treatment of nervous system involvement, however, varied from case to case. However, in more severe cases corticosteroids and cyclophosphamide were usually prescribed.<blockquote>But this is not all because there are some variants of scleroderma such as Morphea diagnosed in our poor patient Mary Poppins who among other things did not respond positively to cortisone therapy.</blockquote>

====Morphea ====
Morphea is a form of scleroderma that involves 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 presents as macules or plaques a few centimeters in diameter, but can also present 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 . Morphea encompasses specific conditions ranging from very small plaques involving only the skin to widespread disease causing functional and cosmetic deformities. Morphea is distinguished 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>

Unfortunately the path is still difficult because the long series of variants does not exclude a form of Morphea-induced hemimasticatory spasm 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> in which it is asserted that on the basis of trigeminal clinical and electrophysiological findings such as the blink reflex, the jaw jerk and the masseteric silent period, focal demyelination of the motor branches of the trigeminal nerve due to deep tissue alterations is suggested as a cause of electrical activities abnormal excitatory movements resulting in involuntary chewing movement and spasm.<blockquote>The latter assertion indicates an involvement of normal and ephaptic excitatory electrical activities.</blockquote>