1/13. Hemimasticatory spasm associated with localized scleroderma and facial hemiatrophy.OBJECTIVES: To report a case and discuss the mechanism of hemimasticatory spasm. DESIGN: Case report. PATIENT: A 37-year-old woman had a 3-year history of involuntary spasms of the right masseter muscle in association with localized scleroderma and facial hemiatrophy. Electrophysiological studies revealed a normal blink reflex. However, the masseter reflex and silent period were absent on the affected side. Distal latency and compound muscle action potential of the masseter nerve were normal. Needle electromyography demonstrated irregular bursts of motor unit potentials similar to those described in hemifacial spasm. A magnetic resonance imaging scan of the head showed mild hypertrophy of the masseter muscle and atrophy of subcutaneous fatty tissues on the affected side. Local injection of botulinum toxin A into the masseter muscle resolved the patient's symptoms. CONCLUSION: On the basis of clinical and electrophysiological findings, focal demyelination of motor branches of the trigeminal nerve owing to deep tissue changes is suggested as the cause of abnormal excitatory electrical activities resulting in involuntary masticatory movement.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/13. Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications.BACKGROUND: Linear scleroderma "en coup de sabre" (LSCS) usually affects one side of the face and head in the frontoparietal area with band-like indurated skin lesions. The disease may be associated with facial hemiatrophy. Various ophthalmological and neurological abnormalities have been observed in patients with LSCS. We describe an unusual case of LSC. CASE PRESENTATION: A 23 year old woman presented bilateral LSCS and facial atrophy. The patient had epileptic seizures as well as oculomotor and facial nerve palsy on the left side which also had pronounced skin involvement. Clinical features of different stages of the disease are presented. CONCLUSIONS: The findings of the presented patient with bilateral LSCS and facial atrophy provide further evidence for a neurological etiology of the disease and may also indicate that classic progressive facial hemiatrophy (Parry-Romberg syndrome) and LSCS actually represent different spectra of the same disease.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
3/13. Diagnostic delay of NF1 in hemifacial hypertrophy due to plexiform neurofibromas.Benign tumors of the peripheral nerve sheath, termed neurofibromas, are the hallmark feature of neurofibromatosis type 1 (NF1). These tumors can result in hypertrophy of a limb or another anatomic region. Hemifacial hypertrophy due to an underlying neurofibroma is a typical manifestation of NF1 in young children although the overall frequency of facial involvement is low. We retrospectively studied all patients, which were referred to our outpatient clinic because of hemihypertrophy or swelling of the face for initially unknown reason with a final diagnosis of NF1. A total number of six patients were identified. Clinical and radiological characteristics of these patients were analyzed. In all patients, diagnosis of NF1 could be established based on the typical clinical criteria. However, despite other typical NF1-associated features (e.g. multiple cafe-au-lait spots) diagnosis of a plexiform neurofibroma as underlying cause for the hemifacial hypertrophy was significantly delayed in all patients. MRI scans were misinterpreted in all of the cases as lymphangioma because plexiform neurofibromas can resemble mesenchymal tumors or lymphangiomas. NF1 has to be considered in the differential diagnosis of hemifacial hypertrophy. A thorough clinical examination of affected patients should focus on typical disease-defining features. early diagnosis of NF1 can prevent unnecessary treatment at least in some patients.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
4/13. Parry-Romberg syndrome associated with borreliosis: could photochemotherapy halt the progression of the disease?Parry-Romberg syndrome (PRS) or progressive hemifacial atrophy is a rare entity characterized by unilateral atrophy of the skin, subcutaneous tissue and sometimes bone and cartilage. Although this syndrome has overlapping features of scleroderma 'en coup de sabre', it shows little or no sclerosis and may affect the entire distribution of the trigeminal nerve including the eye and tongue. As the pathogenesis is unknown, no effective therapy exists. We present here the third case of PRS associated with borreliosis and more interestingly two cases whose progressive course have been stabilized with gel puva therapy.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
5/13. Progressive hemifacial atrophy (Parry-Romberg disease).Hemifacial atrophy (Parry-Romberg syndrome) is characterized by slowly progressive atrophy of one side of the face, primarily involving the subcutaneous tissue and fat. The onset is usually in the first two decades. Ophthalmic involvement is common; the most frequent abnormality is progressive endophthalmos with subsequent changes in the palpebral fissure. Pupillary disturbances, heterochromia, uveitis, and restrictive strabismus have also been frequently reported. We describe six cases that manifest a wide spectrum of ocular and systemic findings. They are noteworthy in that all exhibit pigmentary disturbances of the ocular fundus, a finding rarely reported. Another unusual ocular manifestation in one patient was an acquired partial third nerve palsy on the unaffected side.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
6/13. Pathological changes of peripheral nerve and muscle of patients with hemiatrophy.Peripheral nerve and muscle of patients with hemiatrophy were biopsied and examined by means of light and electron microscope. The unmyelinated nerve fibers decreased in number and remaining fibers were atrophic with irregular shape and high electron density, while myelinated nerve fibers slightly decreased in number. The schwann cells enveloping nerve fibers were also atrophic with high electron density. The muscles contained numerous glycogen granules in the myofilaments and between the myofilaments. The mitochondria in the muscles showed atrophy with high electron density. Some parts of severe atrophy showed honeycomb-like structure forming large vacuoles in the muscle fibers. From the findings mentioned above, it is speculated that primary change was nerve fibers, especially unmyelinated nerve fibers.- - - - - - - - - - ranking = 5keywords = nerve (Clic here for more details about this article) |
7/13. Masticatory spasm in facial hemiatrophy.Spontaneous spasms began in the left masseter muscle six years following the onset of facial hemiatrophy in a young woman. A dental procedure had preceded the original facial wasting by four weeks. The electromyographic findings were similar to those described in hemifacial spasm and consisted of brief bursts of one to four morphologically different potentials, each firing at rates of up to 200 Hz. The findings demonstrate that spontaneous activity due to nerve injury can arise in muscles innervated by the trigeminal nerve.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
8/13. Hemimasticatory spasm in hemifacial atrophy: diagnostic and therapeutic aspects in two patients.We report two cases of hemimasticatory spasm in association with progressive hemifacial atrophy. On the basis of neurophysiological and magnetic resonance imaging assessments, a peripheral irritation of the trigeminal nerve--probably due to entrapment of the motor branches in the infratemporal fossa--is suggested as the cause of the involuntary movement. Local injections of botulinum toxin type A into the masticatory muscles proved to be a successful treatment in both patients.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
9/13. Irreversible muscle contracture after functioning free muscle transplantation using the ipsilateral facial nerve for reinnervation.Four patients who underwent functioning free muscle transplantation (FFMT) for facial reconstruction developed a progressive disfiguring muscle contracture. This complication has not been previously reported. Three of the patients had longstanding facial paralysis and were reanimated by FFMT. The fourth patient had left hemifacial atrophy but without facial paralysis. She also underwent FFMT for augmentation. All four FFMTs were innervated by the ipsilateral facial nerve. Initially, they all had a normal facial appearance at rest during the first few months after FFMT. However, they all developed a progressive severe muscle contracture from 6 to 12 months after FFMT. Continuous spontaneous electrical impulse activity, which stimulated the transferred muscle day and night, may be responsible for the progressive muscle contracture. From the patients' reported sensations and from clinical evaluation, which included a local xylocaine injection test, over-reinnervation with a synkinesis effect of the transferred muscle is hypothetically the main cause, not over-tension of the muscle itself. This complication may possibly be avoided by limiting or decreasing the number of fascicles from the ipsilateral facial nerve or better by using a cross-facial nerve graft instead of the ipsilateral facial nerve as the innervating motor nerve. The outcome with a cross-facial nerve is likely to be more predictable and reliable.- - - - - - - - - - ranking = 5keywords = nerve (Clic here for more details about this article) |
10/13. Pathophysiology of hemimasticatory spasm.Two patients aged 21 and 50 years presented with facial hemiatrophy and unilateral spasms of the masticatory muscles. Masticatory muscle biopsy showed normal findings in both patients and facial skin biopsy specimens only showed atrophy, although morphoea (localised facial scleroderma) had been diagnosed nine years previously in the second patient. The involuntary movements consisted of brief twitches and prolonged contractions clinically and electromyographically similar to those of hemifacial spasm and cramps. The jaw jerk and the silent periods were absent in the affected muscles. Direct stimulation of the muscle nerve and transcranial stimulation of the trigeminal root demonstrated slowing of conduction and after-activity due to autoexcitation. Observations in other reported cases and these two patients suggest that hemimasticatory spasm is produced by ectopic activity secondary to focal demyelination of the trigeminal motor nerve fibres. The proposed cause of the neuropathy is focal damage to the masticatory nerves caused by compression, possibly resulting from the deep tissue changes that occur in facial hemiatrophy.- - - - - - - - - - ranking = 1.5keywords = nerve (Clic here for more details about this article) |
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