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Difference between revisions of "Hemimasticatory spasm"
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'''Abstract:''' This chapter introduces the complex diagnostic pathway to identify the condition known as "Hemimasticatory Spasm" in the case of a patient referred to as "Mary Poppins." While diagnosing this neuromotor disorder may not be overly complex, differentiating it from other conditions such as Hemifacial Spasm or pathologies of the central and peripheral nervous systems is critical for guiding appropriate treatment. | |||
The chapter begins by examining involuntary movement disorders induced by trauma to cranial or peripheral nerves, referencing studies that highlight the involvement of central nervous system pathologies. Special attention is given to conditions like vestibular and trigeminal schwannomas, as well as other rare cases such as tumors affecting facial and masticatory functions. Further complexity arises when considering multiple sclerosis and pleomorphic adenoma as potential sources of trigeminal reflex anomalies, all of which require careful differentiation. | |||
Additionally, the discussion explores conditions like scleroderma, with particular emphasis on Morphea, which is identified in the patient. The possibility of Morphea-induced Hemimasticatory Spasm is discussed based on trigeminal nerve clinical and electrophysiological findings. The role of electrical excitatory activities, both normal and ephaptic, is considered a significant contributor to abnormal involuntary muscle movements. | |||
The chapter concludes by introducing "ephaptic transmission" as the key to understanding the abnormal communication phenomena in Hemimasticatory Spasm. Ephaptic transmission, a complex form of electrical signaling between neurons, is explored as part of the diagnostic process, providing a foundation for understanding the electrical dynamics underlying the condition. This crucial concept will be examined in greater detail in a subsequent chapter focused on the two forms of electrical transmission between neurons. | |||
==Introduction == | ==Introduction to the Hemimasticatory Spasm== | ||
Before getting into the heart of the discussion regarding the pathology of our patient Mary Poppins, what from the previous chapters seems to be of a neuromotry type and in particular a 'Hemimasticatory Spasm' we should focus on some points to determine the process of decryption of the signal. | Before getting into the heart of the discussion regarding the pathology of our patient Mary Poppins, what from the previous chapters seems to be of a neuromotry type and in particular a 'Hemimasticatory Spasm' we should focus on some points to determine the process of decryption of the signal. | ||
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</ref>, which can be explored in depth in the sub-chapter of Masticationpedia '[[Intermittent facial spasms as the presenting sign of a recurrent pleomorphic adenoma]]' in which the authors confirm that to date the development of facial spasms has not been reported in parotid neoplasms. The most common etiologies for hemifacial spasm are vascular compression of the facial nerve ipsilateral to the cerebellopontine angle (defined as primary or idiopathic) (62%), hereditary (2%), secondary to Bell's palsy or facial nerve injury (17 %) and imitators of hemifacial spasms (psychogenic, tics, dystonia, myoclonus, myokymia, myorrhythmia and hemimasticatory spasm) (17%). | </ref>, which can be explored in depth in the sub-chapter of Masticationpedia '[[Intermittent facial spasms as the presenting sign of a recurrent pleomorphic adenoma]]' in which the authors confirm that to date the development of facial spasms has not been reported in parotid neoplasms. The most common etiologies for hemifacial spasm are vascular compression of the facial nerve ipsilateral to the cerebellopontine angle (defined as primary or idiopathic) (62%), hereditary (2%), secondary to Bell's palsy or facial nerve injury (17 %) and imitators of hemifacial spasms (psychogenic, tics, dystonia, myoclonus, myokymia, myorrhythmia and hemimasticatory spasm) (17%). | ||
===Scleroderma=== | ===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 | 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 | ||
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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> | 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 ==== | ||
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> | 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> | ||