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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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Secondary hemifacial spasm due to vestibular schwannoma is very rare. The study by S Peker et al.<ref>S Peker, K Ozduman, T Kiliç, M N Pamir. [https://pubmed.ncbi.nlm.nih.gov/15346321/ Relief of hemifacial spasm after radiosurgery for intracanalicular vestibular schwannoma.] Minim Invasive Neurosurg. 2004 Aug;47(4):235-7. doi: 10.1055/s-2004-818485.</ref> was the first reported case of hemifacial spasm responsive to gamma knife radiosurgery in a patient with an intracanalicular vestibular schwannoma. Both spasm resolution and tumor growth control were achieved with a single session of gamma knife radiosurgery. The 49-year-old male patient with a 6-month history of right-sided hearing loss and hemifacial spasm. MRI examination revealed an intracanalicular vestibular schwannoma. The patient was treated with radiosurgery and received 13 Gy at the 50% isodose line. Control of tumor growth was achieved and there was no change in tumor volume at the latest follow-up at 22 months. The hemifacial spasm completely resolved after one year. Surgical removal of the presumably causative mass lesion has been reported to be the only treatment in secondary hemifacial spasm. <blockquote>MRI is the imaging modality of choice and is usually diagnostic in the appropriate clinical setting. The thin T2-weighted 3D CISS axial sequence is important for correct evaluation of the cisternal segment of the nerve. They are usually hypointense on T1, hyperintense on T2 with enhancement after gadolinium. But we cannot be surprised if cases like the one described by Brandon Emilio Bertot et al<ref name=":02">Brandon Emilio Bertot, Melissa Lo Presti, Katie Stormes, Jeffrey S Raskin, Andrew Jea, Daniel Chelius, Sandi Lam. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7451153/#!po=12.5000 Trigeminal schwannoma presenting with malocclusion: A case report and review of the literature.]Surg Neurol Int. 2020 Aug 8;11:230. doi: 10.25259/SNI_482_2019.eCollection 2020.</ref> occur. in which a clinical case of a 16-year-old boy with an atypical incidence of a large trigeminal schwannoma presenting with painless malocclusion and unilateral masticatory weakness was presented. This case is the first documented case, to our knowledge, in which a trigeminal schwannoma generated a true malocclusion with masseter weakness and is the 19th documented case of unilateral trigeminal motor neuropathy of various etiology. From a study by Ajay Agarwal,<ref>Ajay Agarwal. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4757116/ Intracranial trigeminal schwannoma] Ajay Agarwal. Neuroradiol J.2015 Feb;28(1):36-41. doi: 10.15274/NRJ-2014-10117.</ref> however, it is clear that intracranial trigeminal schwannomas are rare tumors. Patients usually present with symptoms of trigeminal nerve dysfunction, the most common symptom being facial pain. <gallery mode="slideshow" heights="200" caption="Trigeminal schwannoma presenting with malocclusion: A case report and review of the literature"> | Secondary hemifacial spasm due to vestibular schwannoma is very rare. The study by S Peker et al.<ref>S Peker, K Ozduman, T Kiliç, M N Pamir. [https://pubmed.ncbi.nlm.nih.gov/15346321/ Relief of hemifacial spasm after radiosurgery for intracanalicular vestibular schwannoma.] Minim Invasive Neurosurg. 2004 Aug;47(4):235-7. doi: 10.1055/s-2004-818485.</ref> was the first reported case of hemifacial spasm responsive to gamma knife radiosurgery in a patient with an intracanalicular vestibular schwannoma. Both spasm resolution and tumor growth control were achieved with a single session of gamma knife radiosurgery. The 49-year-old male patient with a 6-month history of right-sided hearing loss and hemifacial spasm. MRI examination revealed an intracanalicular vestibular schwannoma. The patient was treated with radiosurgery and received 13 Gy at the 50% isodose line. Control of tumor growth was achieved and there was no change in tumor volume at the latest follow-up at 22 months. The hemifacial spasm completely resolved after one year. Surgical removal of the presumably causative mass lesion has been reported to be the only treatment in secondary hemifacial spasm. <blockquote>MRI is the imaging modality of choice and is usually diagnostic in the appropriate clinical setting. The thin T2-weighted 3D CISS axial sequence is important for correct evaluation of the cisternal segment of the nerve. They are usually hypointense on T1, hyperintense on T2 with enhancement after gadolinium. But we cannot be surprised if cases like the one described by Brandon Emilio Bertot et al<ref name=":02">Brandon Emilio Bertot, Melissa Lo Presti, Katie Stormes, Jeffrey S Raskin, Andrew Jea, Daniel Chelius, Sandi Lam. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7451153/#!po=12.5000 Trigeminal schwannoma presenting with malocclusion: A case report and review of the literature.]Surg Neurol Int. 2020 Aug 8;11:230. doi: 10.25259/SNI_482_2019.eCollection 2020.</ref> occur. in which a clinical case of a 16-year-old boy with an atypical incidence of a large trigeminal schwannoma presenting with painless malocclusion and unilateral masticatory weakness was presented. This case is the first documented case, to our knowledge, in which a trigeminal schwannoma generated a true malocclusion with masseter weakness and is the 19th documented case of unilateral trigeminal motor neuropathy of various etiology. From a study by Ajay Agarwal,<ref>Ajay Agarwal. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4757116/ Intracranial trigeminal schwannoma] Ajay Agarwal. Neuroradiol J.2015 Feb;28(1):36-41. doi: 10.15274/NRJ-2014-10117.</ref> however, it is clear that intracranial trigeminal schwannomas are rare tumors. Patients usually present with symptoms of trigeminal nerve dysfunction, the most common symptom being facial pain. <gallery mode="slideshow" heights="200" caption="Trigeminal schwannoma presenting with malocclusion: A case report and review of the literature"> | ||
File:Scwannoma.jpeg|'''Figure 1:''' Preoperative MRI Figure Description: T1-weighted contrast MRI with axial, sagittal, and coronal views of 5.2 × 7.8 × 5.1 cm heterogeneous extra-axial enhancing mass centered in the mesial temporal region left with involvement of the skull base and left foramen ovale, teres and spinosa, encasement and moderate narrowing of the left internal carotid artery, mild obstructive hydrocephalus and marked compression of the brainstem. | File:Scwannoma.jpeg|'''Figure 1:''' Preoperative MRI Figure Description: T1-weighted contrast MRI with axial, sagittal, and coronal views of 5.2 × 7.8 × 5.1 cm heterogeneous extra-axial enhancing mass centered in the mesial temporal region left with involvement of the skull base and left foramen ovale, teres and spinosa, encasement and moderate narrowing of the left internal carotid artery, mild obstructive hydrocephalus and marked compression of the brainstem. | ||
File:Scwannoma 1.jpeg|'''Figure 2:''' | File:Scwannoma 1.jpeg|'''Figure 2:''' Postoperative magnetic resonance imaging figure description: contrasted T1-weighted magnetic resonance imaging with axial, sagittal, and coronal views demonstrates near complete resection with a thin rim of residual tumor along the lateral dural margin of the left cavernous sinus/lesser sphenoid wing and at the floor of the left middle cranial fossa extending posteriorly to the roof of the remodeled left petrous temporal bone. | ||
</gallery></blockquote> | </gallery></blockquote> | ||
===Multiple sclerosis and trigeminal reflexes=== | ===Multiple sclerosis and trigeminal reflexes=== | ||
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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> | ||