A case of parasympathetic hyperactivity and associated Parry–Romberg syndrome

This case report describes a 46-year-old female with a history of multiple endocrine neoplasia type 1 syndrome status post-parathyroidectomy, thymectomy via robotic video-assisted thoracoscopic surgery, and pituitary adenoma resection presenting with parasympathetic hyperactivity and Parry–Romberg syndrome. Parry–Romberg syndrome is a rare disorder that manifests as facial hemiatrophy. Reported symptoms include cognitive dysfunction, olfactory hallucinations, and parasympathetic hyperactivity: miosis of the right eye, anhidrosis, diarrhea, urinary incontinence, bowel incontinence, and orthostatic hypotension. Previous reports have noted associations between Parry–Romberg syndrome and sympathetic hyperactivity and dysregulation. This case report focuses on an association between Parry–Romberg syndrome and unopposed parasympathetic activity.

Neurovascular conflict with the compression of the motor portion of the trigeminal nerve: hemimasticatory spasm and Parry-Romberg syndrome. Case report

The most common cause of neurovascular conflict (NVC) is the compression of the sensory portion of the trigeminal nerve root by artery or vein directly at the brainstem. This results in local demyelination manifested as the classic trigeminal neuralgia. The motor portion of the trigeminal nerve is affected less commonly. The authors describe a case history of a 26-year-old man with a verified NVC of the motor portion of the trigeminal nerve. At the onset, the disease manifested as intense pain paroxysms in the left side of the face preceded by a masseter muscle spasm that was accompanied by lockjaw and tongue biting (hemimasticatory spasm). Shortly after disease onset, facial hemiatrophy with local scleroderma developed. The diagnosis of NVC with the compression of the motor portion of the trigeminal nerve accompanied by hemimasticatory spasm and Parry-Romberg syndrome was verified by brain FIESTA MRI and needle EMG. Later on, these symptoms spontaneously regressed. Concepts of hemimasticatory spasm and facial hemiatrophy are discussed. The rarity of this condition, lack of information on the compression of the motor portion of the trigeminal nerve in NVC, unusual disease onset, and presentations make this issue interesting for a wide range of experts. KEYWORDS: neurovascular conflict, hemimasticatory spasm, facial hemiatrophy, Parry-Romberg syndrome, trigeminal neuralgia, trigeminal nerve, local scleroderma, oromandibular dystonia. FOR CITATION: Matveeva T.V., Kazantsev A.Yu., Aleskerova A.B., Gaifutdinov R.T. Neurovascular conflict with the compression of the motor portion of the trigeminal nerve: hemimasticatory spasm and Parry-Romberg syndrome. Case report. Russian Medical Inquiry. 2021;5(10):668–673 (in Russ.). DOI: 10.32364/2587-6821-2021-5-10-668-673.

Progressive facial hemiatrophy (Parry-Romberg syndrome): short case report

Introduction: The Parry Romberg syndrome (PRS) is a mosaic disease of unknown aetiology which mostly affects women. The facial hemiatrophy generally begins during the early childhood. It has a great impact on social life, and aesthetic rehabilitation is a major challenge. Observation: A 38 years-old-female patient presented with an enucleation of the left eye, due to multiple hamartomas and progressive facial hemiatrophy. The placement of 2 extraoral implants was proposed to bear an ocular epithesis. Comments: PRS develops between the 2nd and 20th year of life, and stabilizes at the adult age. Its main characteristic is a progressive facial hemiatrophy, which involves skin, fat tissues, muscles and osteocartilaginous tissues. 20% of patients have neurological symptoms, and epilepsy is often described as a side effect of the disease. Conclusion: Diagnosis of localized scleroderma has to be eliminated, as these 2 entities are very similar. Major facial reconstructive surgery is often proposed to restore aesthetics.

Parry-Romberg syndrome in a patient with scleroderma

Parry-Romberg syndrome (PRS) is characterised by progressive but self-limiting facial hemiatrophy. We describe a 48-year-old woman with a 3-year history of gradually worsening right facial hemiatrophy on a background of scleroderma. Her initial primary concern was alopecia. Within the last year, there was greater prominence of her right zygoma and hyperpigmentation on her forearms and left neck. She also had worsening headaches and neck stiffness in the mornings. A clinical diagnosis of PRS was made and she was subsequently treated with a course of methotrexate. She is due to be followed up by dermatology, rheumatology and maxillofacial surgery with the aim of reconstructive surgery once her symptoms stabilise.