Unilateral Atrophy Research Articles

Retinal Nerve Fiber Layer and Peripapillary Choroidal Thicknesses in Non-Glaucomatous Unilateral Optic Atrophy Compared with Unilateral Advanced Pseudoexfoliative Glaucoma

ABSTRACTPurpose: To investigate retinal nerve fiber layer thickness (RNFL-T) and peripapillary choroidal thickness (PC-T) in non-glaucomatous optic atrophy (OA) patients in comparison with unaffected and control eyes, furthermore, to compare thickness profiles with unilateral pseudoexfoliative advanced glaucoma.Materials and Methods: Thirty-three eyes with OA (Group A), 33 unaffected fellow eyes (Group B), 25 right eyes of 25 control subjects (Group C), and 15 eyes with advanced glaucoma (Group D) were enrolled. RNFL-T was measured in six regions by spectral-domain optical coherence tomography. Enhanced depth imaging optical coherence tomography was obtained to evaluate PC-T in corresponding regions.Results: RNFL-T was significantly lower in Group A than in Groups B and C globally and at all peripapillary regions (all p < 0.001). P-CT in Group A was significantly lower globally (p = 0.03) and in three regions (temporal, p = 0.001; temporal-superior, p = 0.01; and nasal-inferior, p = 0.037) versus Group C. However, it was significantly thinner than in Group B in all regions (temporal, p = 0.02; temporal-superior, p = 0.013; nasal-superior, p = 0.044; nasal, p = 0.02; nasal-inferior, p < 0.001; and temporal-inferior, p < 0.001) and globally (p < 0.001). In Group A RNFL-T (thicker superiorly and inferiorly; thinner temporally and nasally) and PC-T (superior > temporal > nasal > inferior) profiles were almost identical to that in unaffected fellow eyes and control eyes. However, Group D showed different patterns with less regional differences in RNFL-T, and the greatest value of PC-T in nasal quadrant.Conclusions: Besides retinal nerve fiber layer thinning, non-glaucomatous OA is associated with choroidal thinning. The RNFL-T and PC-T profiles in advanced glaucoma eyes differed from the common patterns seen among OA eyes, unaffected fellow eyes, and control eyes.

Genetics of Unilateral and Bilateral Age-Related Macular Degeneration Severity Stages.

BackgroundAge-related macular degeneration (AMD) is a common disease causing visual impairment and blindness. Various gene variants are strongly associated with late stage AMD, but little is known about the genetics of early forms of the disease. This study evaluated associations of genetic factors and different AMD stages depending on unilateral and bilateral disease severity.MethodsIn this case-control study, participants were assigned to nine AMD severity stages based on the characteristics of each eye. 18 single nucleotide polymorphisms (SNPs) were genotyped and attempted to correlate with AMD severity stages by uni- and multivariate logistic regression analyses and trend analyses. Area under the receiver operating characteristic curves (AUC) were calculated.ResultsOf 3444 individuals 1673 were controls, 379 had early AMD, 333 had intermediate AMD and 989 showed late AMD stages. With increasing severity of disease and bilateralism more SNPs with significant associations were found. Odds ratios, especially for the main risk polymorphisms in ARMS2 (rs10490924) and CFH (rs1061170), gained with increasing disease severity and bilateralism (exemplarily: rs1061170: unilateral early AMD: OR = 1.18; bilateral early AMD: OR = 1.20; unilateral intermediate AMD: OR = 1.28; bilateral intermediate AMD: OR = 1.39, unilateral geographic atrophy (GA): OR = 1.50; bilateral GA: OR = 1.71). Trend analyses showed p<0.0001 for ARMS2 (rs10490924) and for CFH (rs1061170), respectively. AUC of risk models for various AMD severity stages was lowest for unilateral early AMD (AUC = 0.629) and showed higher values in more severely and bilaterally affected individuals being highest for late AMD with GA in one eye and neovascular AMD in the other eye (AUC = 0.957).ConclusionThe association of known genetic risk factors with AMD became stronger with increasing disease severity, which also led to an increasing discriminative ability of AMD cases and controls. Genetic predisposition was also associated with the disease severity of the fellow-eye, highlighting the importance of both eyes in AMD patients.

Implicaciones anestésicas del síndrome de Parry-Romberg: reporte de un caso

IntroductionParry-Romberg syndrome is a rare degenerative disease characterized by unilateral atrophy affecting the skin, connective tissue, muscle and bone. The end result is facial asymmetry associated with other skin, dental, visual, cardiovascular, and neurological disorders. Clinical findings, diagnostic evaluation and interventionsThe case of a patient with Parry-Romberg syndrome programmed for frontonasal flap remodeling is discussed. The patient's history includes trigeminal neuralgia, epilepsy, and two previous surgical interventions. Uneventful endotracheal intubation with the Glideoscope® video laryngoscope was performed, upon adequate pre-oxygenation followed by anesthetic induction. ConclusionThe phenotypical characteristics of Parry-Romberg syndrome are severe facial hemiatrophy and craniofacial anomalies that require careful preoperative evaluation and management of a potentially difficult airway. Consequently, the use of video laryngoscopes is a first-line approach. Due to the syndrome's associated disorders, it is essential to maintain hemodynamic stability and prevent any potential seizures.

Anesthetic implications of Parry Romberg Syndrome: A case report

IntroductionParry–Romberg Syndrome is a rare degenerative disease characterized by unilateral atrophy affecting the skin, connective tissue, muscle and bone. The end result is facial asymmetry associated with other skin, dental, visual, cardiovascular, and neurological disorders. Clinical findings, diagnostic evaluation and interventionsThe case of a patient with Parry–Romberg Syndrome programmed for frontonasal flap remodeling is discussed. The patient's history includes trigeminal neuralgia, epilepsy, and two previous surgical interventions. Uneventful endotracheal intubation with the Glideoscope® video laryngoscope was performed, upon adequate pre-oxygenation followed by anesthetic induction. ConclusionThe phenotypical characteristics of Parry Romberg Syndrome are severe facial hemiatrophy and craniofacial anomalies that require careful preoperative evaluation and management of a potentially difficult airway. Consequently, the use of video laryngoscopes is a first-line approach. Due to the syndrome's associated disorders, it is essential to maintain hemodynamic stability and prevent any potential seizures.

Anti-tumor necrosis factor alpha therapy (adalimumab) in Rasmussen's encephalitis: An open pilot study.

Rasmussen's encephalitis (RE) is a severe chronic inflammatory brain disease affecting one cerebral hemisphere and leading to drug-resistant epilepsy, progressive neurologic deficit, and unilateral brain atrophy. Hemispherotomy remains the gold standard treatment but causes permanent functional impairment. No standardized medical treatment protocol currently exists for patients prior to indication of hemispherotomy, although some immunotherapies have shown partial efficacy with functional preservation but poor antiseizure effect. Some studies suggest a role for tumor necrosis factor alpha (TNF-α) in RE pathophysiology. We report an open-label study evaluating the efficacy and the safety of anti-TNF-α therapy (adalimumab) in 11 patients with RE. The primary outcome criterion was the decrease of seizure frequency. The secondary outcome criteria were neurologic and cognitive outcomes and existence of side effects. Adalimumab was introduced with a median delay of 31 months after seizure onset (range 1 month to 16 years), and follow-up was for a median period of 18 months (range 9-54 months). There was a significant seizure frequency decrease after adalimumab administration (from a median of 360 to a median of 32 seizures per quarter, p ≤ 0.01). Statistical analysis showed that adalimumab had a significant intrinsic effect (p < 0.005) independent from disease fluctuations. Five patients (45%) were found to have sustained improvement over consecutive quarters in seizure frequency (decrease of 50%) on adalimumab. Three of these five patients also had no further neurocognitive deterioration. Adalimumab was well tolerated. Our study reports efficacy of adalimumab in terms of seizure frequency control. In addition, stabilization of functional decline occurred in three patients. This efficacy might be particularly relevant for atypical slowly progressive forms of RE, in which hemispherotomy is not clearly indicated. Due to our study limitations, further studies are mandatory to confirm these preliminary results.

Changing patterns in paediatric optic atrophy aetiology: 1979 to 2015.

To evaluate the aetiology of paediatric optic atrophy in an Australian population. Retrospective review of medical records was conducted at The Children's Hospital at Westmead, Sydney, Australia. Two hundred twenty-seven subjects <16 years who were diagnosed with optic atrophy on fundoscopic examination between 1979 and 2015 were included in the study. Subjects were obtained from the hospital database, which codes diagnoses for all admissions, as well as the orthoptic department database, which codes diagnoses for ophthalmology department outpatients. The predominant cause for optic atrophy was assigned to each patient. Clinical presentation was defined as the principal reason for evaluation. Demographic data included gender, affected eye and age at diagnosis. Data on medical comorbidities (cerebral palsy, developmental delay, microcephaly and seizures) and ocular comorbidities (strabismus and nystagmus) were collected. The mean age at initial eye review was 4.7 ± 4.4 years. There was bilateral optic atrophy in 81.5% of cases. Unilateral optic atrophy was largely due to tumours. When analysing over the three time periods, (1979-1990, 1991-2003 and 2004-2015), perinatal events (3.0%, 22.7% and 22.6%) and neurodegenerative disease (3.0%, 14.9% and 15.1%) are slowly replacing tumours (39.4%, 24.8% and 15.1%) as the top causes for paediatric optic atrophy. The incidence of other causes has remained fairly stable over time, albeit an increase in idiopathic causes. There has been shift in the etiological profile of optic atrophy. Whilst tumours are still an important cause of paediatric optic atrophy for an Australian population, perinatal events and neurodegenerative disease are becoming more significant.

Determinants of brain metabolism changes in mesial temporal lobe epilepsy.

To determine the main factors influencing metabolic changes in mesial temporal lobe epilepsy (MTLE) due to hippocampal sclerosis (HS). We prospectively studied 114 patients with MTLE (62 female; 60 left HS; 15- to 56-year-olds) with (18) F-fluorodeoxyglucose-positron emission tomography and correlated the results with the side of HS, structural atrophy, electroclinical features, gender, age at onset, epilepsy duration, and seizure frequency. Imaging processing was performed using statistical parametric mapping. Ipsilateral hypometabolism involved temporal (mesial structures, pole, and lateral cortex) and extratemporal areas including the insula, frontal lobe, perisylvian regions, and thalamus, more extensively in right HS (RHS). A relative increase of metabolism (hypermetabolism) was found in the nonepileptic temporal lobe and in posterior areas bilaterally. Voxel-based morphometry detected unilateral hippocampus atrophy and gray matter concentration decrease in both frontal lobes, more extensively in left HS (LHS). Regardless of the structural alterations, the topography of hypometabolism correlated strongly with the extent of epileptic networks (mesial, anterior-mesiolateral, widespread mesiolateral, and bitemporal according to the ictal spread), which were larger in RHS. Notably, widespread perisylvian and bitemporal hypometabolism was found only in RHS. Mirror hypermetabolism was grossly proportional to the hypometabolic areas, coinciding partly with the default mode network. Gender-related effect was significant mainly in the contralateral frontal lobe, in which metabolism was higher in female patients. Epilepsy duration correlated with the contralateral temporal metabolism, positively in LHS and negatively in RHS. Opposite results were found with age at onset. High seizure frequency correlated negatively with the contralateral metabolism in LHS. Epileptic networks, as assessed by electroclinical correlations, appear to be the main determinant of hypometabolism in MTLE. Compensatory mechanisms reflected by a relative hypermetabolism in the nonepileptic temporal lobe and in extratemporal areas seem more efficient in LHS and in female patients, whereas long duration, late onset of epilepsy, and high seizure frequency may reduce these adaptive changes.

DYKE-DAVIDOFF-MASSON SYNDROME PRESENTING WITH RECURRENT SEIZURES

Dyke-Davidoff-Masson syndrome (DDMS) is cerebral hemiatrophy occurring following brain insult resulting from infarct, trauma or infection in utero or soon after birth. Clinical features of this syndrome include variable degree of contralateral hemiparesis, facial asymmetry, seizures and mental retardation. Recurrent seizures is the most debilitating and poor prognostic indicator of this disease. Neuroimaging plays pivotal role in establishing the diagnosis of DDMS syndrome. CT/MR imaging shows unilateral cerebral parenchymal atrophy, prominent sulcal spaces, ipsilateral ventriculomegaly, cerebellar atrophy, falcine and superior Sagittal sinus shift to affected side. Bone windowing in CT scan shows decreased skull volume on affected side calvarial thickening, elevation of sphenoid wing and petrous temporal bone, expanded sinuses and mastoids. Here we describe 34 years old female who had hemiparesis, seizures and facial asymmetry since childhood and imaging evaluation established diagnosis of congenital type of DDMS.

2. Follow-up of five cases with Rasmussen encephalitis with magnetoencephalography

Magnetoencephalography (MEG) recordings of interictal and/or ictal discharges can provide valuable information about the location of the epileptogenic area in the brain. Rasmussen Encephalitis (RE) is characterized by intractable epilepsy, progressive hemiparesis, and unilateral hemispheric atrophy. The progression of the symptoms to significant neurological impairment usually occurs within months to a few years. Five patients (mean age at first observation 7.6 ± 3 years) fitting Bien’s criteria for the diagnosis of RE were enrolled for the study and follow-up for at least 1.5 years (4–7 MEG measures per patient), in order to evaluate correlations between the evolution of clinical picture, neuroradiological and MEG findings. MEG data were visually inspected and interictal spikes were selected for the source localization by means of equivalent current dipoles (ECDs). Cortical thickness was evaluated from Magnetic Resonance Imaging (MRI). All patients showed ictal/interictal multifocality, reduction of background cortical magnetic activity, and progressive focal cortical atrophy on MRI of the affected hemisphere. Moreover, our preliminary data showed that dipole clusters were found mainly in the most atrophic cortical regions, suggesting a close relationship between MRI and MEG alterations.

Laryngeal Paralysis in a Loggerhead Sea Turtle (Caretta caretta)

Abstract A cold-stunned, sub-adult, female loggerhead sea turtle (Caretta caretta) was found stranded in December 2012 on Cape Cod, Massachusetts. The turtle was treated routinely; however, she did not gain weight as expected during rehabilitation. Eleven weeks after stranding, staff noticed stertorous upper airway sounds. Oral examination revealed a unilateral laryngeal paralysis-like condition resulting in partial airway obstruction. Magnetic resonance imaging and electromyography showed abnormalities of the abductor arytenoideae muscle. Muscle and hepatic biopsies were collected for histologic and toxicologic assessment to seek an underlying cause of the disorder. The turtle did not recover from anesthesia, and necropsy revealed a large amount of hemorrhage from the liver biopsy site. Unilateral myofiber atrophy of the abductor arytenoideae muscle was the predominant histological finding.

Atrophy of anterior digastric muscle detected during ultrasound of the neck - a prospective clinical study

Anatomical variants that affect both the anterior and posterior digastric muscles are well described, and include duplications and variable origin or sites of insertion. Although agenesis or atrophy of the anterior digastric occurs in up to 40% of patients with hemifacial microsomia, to our knowledge, it has been reported only rarely in those without this syndrome. To minimise observer bias and to establish the incidence of anomalies and atrophy of this muscle, a prospective 6-year study was conducted by an experienced consultant radiologist in all patients referred for ultrasound of the head and neck. Unilateral atrophy or absence of the anterior digastric muscle was seen in 3 of 1484 patients (0.2%). All 3 were female, and they had no symptoms or clinical signs related to the finding. One subsequently had a magnetic resonance scan. Non-syndromic absence or atrophy of the anterior digastric muscle is rare, and seems to be a unilateral phenomenon. Recognition of this variant could be important before operations in which it may be used as a landmark, or during procedures in which it is used, for example, for reanimation of a static lip or during reconstruction with a submental island flap.

Pathomorphology of spontaneously occurring female genital tract lesions in buffaloes of North Gujarat

The objective of the present study was to determine the prevalence of female genital tract pathology in buffaloes of North Gujarat. A total of 179 buffalo genitalia collected from the slaughter house were screened for the presence of gross lesions. Out of 179, 57 (37.84%) genitalia were found grossly affected. The lesions observed were paraovarian cyst, 18 (10.06%) followed by bilateral inactive ovaries, 14 (7.82%), uterine serosal inclusion cyst, 6 (3.35%), aneurysms, 6 (3.35%), unilateral ovarian atrophy, 5 (2.79%), unilateral follicular cyst, 4 (2.23%) and one each of unilateral cystic corpus luteum, hematoma in paraovarian tissues, unilateral hydrosalpinx and mucometra.

The Risk of Vocal Fold Atrophy after Serial Corticosteroid Injections of the Vocal Fold

The aim of this study was to illustrate the risk of vocal fold atrophy in patients who receive serial subepithelial steroid injections for vocal fold scar. This study is a retrospective case report of two patients who underwent a series of weekly subepithelial infusions of 10 mg/mL dexamethasone for benign vocal fold lesion. Shortly after the procedures, both patients developed a weak and breathy voice. The first patient was a 53-year-old man with radiation-induced vocal fold stiffness. Six injections were performed unilaterally, and 1 week later, he developed unilateral vocal fold atrophy with new glottal insufficiency. The second patient was a 67-year-old woman with severe vocal fold inflammation related to laryngitis and calcinosis, Raynaud's phenomenon, esophagean dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome. Five injections were performed bilaterally, and 1 week later, she developed bilateral vocal fold atrophy with a large midline glottal gap during phonation. In both cases, the steroid-induced vocal atrophy resolved spontaneously after 4 months. Serial subepithelial steroid infusions of the vocal folds, although safe in the majority of patients, carry the risk of causing temporary vocal fold atrophy when given at short intervals.

Aberrant topological patterns of brain structural network in temporal lobe epilepsy.

Although altered large-scale brain network organization in patients with temporal lobe epilepsy (TLE) has been shown using morphologic measurements such as cortical thickness, these studies, have not included critical subcortical structures (such as hippocampus and amygdala) and have had relatively small sample sizes. Here, we investigated differences in topological organization of the brain volumetric networks between patients with right TLE (RTLE) and left TLE (LTLE) with unilateral hippocampal atrophy. We performed a cross-sectional analysis of 86 LTLE patients, 70 RTLE patients, and 116 controls. RTLE and LTLE groups were balanced for gender (p = 0.64), seizure frequency (Mann-Whitney U test, p = 0.94), age (p = 0.39), age of seizure onset (p = 0.21), and duration of disease (p = 0.69). Brain networks were constructed by thresholding correlation matrices of volumes from 80 cortical/subcortical regions (parcellated with Freesurfer v5.3 https://surfer.nmr.mgh.harvard.edu/) that were then analyzed using graph theoretical approaches. We identified reduced cortical/subcortical connectivity including bilateral hippocampus in both TLE groups, with the most significant interregional correlation increases occurring within the limbic system in LTLE and contralateral hemisphere in RTLE. Both TLE groups demonstrated less optimal topological organization, with decreased global efficiency and increased local efficiency and clustering coefficient. LTLE also displayed a more pronounced network disruption. Contrary to controls, hub nodes in both TLE groups were not distributed across whole brain, but rather found primarily in the paralimbic/limbic and temporal association cortices. Regions with increased centrality were concentrated in occipital lobes for LTLE and contralateral limbic/temporal areas for RTLE. These findings provide first evidence of altered topological organization of the whole brain volumetric network in TLE, with disruption of the coordinated patterns of cortical/subcortical morphology.

Living donor liver transplantation using dual grafts: Experience and lessons learned from cases worldwide.

In living donor liver transplantation (LDLT), insufficient graft volume could result in small-for-size syndrome in recipients, whereas major liver donation predisposes the donor to a high risk of posthepatectomy liver failure. Dual graft LDLT is therefore introduced to obtain combined graft sufficiency. To date, 367 patients have been reported worldwide. We reviewed all the relevant literature, with a special focus on 43 case reports containing enough data to extract and analyze. A simple decision-making algorithm was developed. Dual graft LDLT is indicated when (1) a single donation is unacceptable due to graft-to-recipient size mismatch; (2) the future liver remnant is insufficient in the single donor after major resection; or (3) there is a significant underlying disorder or anatomical variation within the donor liver. The outcome of dual graft LDLT is reported to be comparable with that of single donor LDLT. Unilateral graft atrophy was found in 7 of the 43 patients, predominantly in the right-sided, heterotopic and initially smaller grafts. Technically, the heterotopic implantation and complex vascular reconstruction are the most demanding. Elaborate surgical planning and modification are needed. Ethical concerns about involving a second living donor need to be addressed. In conclusion, dual graft LDLT should be prudently performed in select cases by surgeons of proven expertise when single donation is unacceptable and a second living donor is available. The decision-making criteria need to be standardized. More surgical modification and clinical research are needed.

Long-Term Follow-Up of Fundus Autofluorescence Imaging Using Wide-Field Scanning Laser Ophthalmoscopy

Aim: To evaluate the variation of peripheral alterations in different retinal diseases over a period of >3 years by using wide-field fundus autofluorescence (FAF) scanning laser ophthalmoscopy (SLO). Methods: A total of 26 eyes from 13 patients (median age 66 years, range 19-80) with age-related macular degeneration and other retinal degenerations were examined. In 2009, the Optos P200CAF prototype and from 2012 onwards, the Optos 200Tx (Optos plc, Scotland) were used for wide-field FAF SLO (excitation 532 nm). Results: The area involvement in outer retinal pathological alterations, such as atrophy and mottling of the retinal pigment epithelium far beyond the vascular arcades, was readily and better visualized within one image frame using wide-field FAF as compared to pseudocolor SLO of the same device. Over time, progression of existing and the development of de novo peripheral lesions were recorded with a concomitant enlargement of central lesions. In two cases (unilateral paravenous pigmented choroidal atrophy and suspected phenocopy of retinal dystrophy), no longitudinal changes of the topographic distribution of peripheral FAF intensities were noted. Conclusions: Wide-field FAF SLO allows the mapping of dynamic changes at the outer retina far beyond the vascular arcades. While its ability to detect and monitor these changes appears to be better than that of pseudocolor imaging, wide-field FAF SLO may not only be helpful to assess more widespread retinal dysfunction, but may also be useful for longitudinal assessments in natural history studies and interventional clinical trials.

Focal atrophy of the masticatory muscles caused by pure trigeminal motor neuropathy: case report

Pure trigeminal motor neuropathy is rare and characterised by weakness of the mandibular motor branch with no signs of involvement of the trigeminal sensory or other cranial nerve. Its aetiology is unclear but it has been hypothesised that a viral infection may be a cause. Magnetic resonance imaging is useful in its diagnosis and typically shows loss of volume of the affected masticatory muscles and infiltration of fat. We describe the case of a 29-year-old woman who presented with a 12-year history of progressive facial asymmetry, which was later shown on imaging to be caused by unilateral atrophy of the masseter with compensatory contralateral hypertrophy.

Papillary tumor of the pineal region presenting with Foster Kennedy sign

Foster Kennedy syndrome is defined as unilateral optic nerve atrophy, contralateral disc edema and anosmia. It was originally thought to be pathognomonic of space occupying lesions in the anterior fossa causing direct compression of the optic and olfactory nerves and later intracranial hypertension and contralateral disc edema. Subsequent reports of non-tumor and non-anterior fossa causes, most commonly bilateral sequential anterior ischemic optic neuropathy, have been described. We report a 29 year old man who presented with unsteady gait and diminution of vision over 2 months. He displayed findings typical of Parinaud’s syndrome, right disc edema and left optic atrophy. MRI revealed a large pineal tumor associated with hydrocephalus and evidence of increased intracranial pressure. Papillary tumor of the pineal region (PTPR) was diagnosed on histopathology. To our knowledge, this is the first report of this very rare tumor, occurring in association with the Foster Kennedy sign.

Epilepsy surgery in adult-onset Rasmussen's encephalitis: case series and review of the literature.

Rasmussen's encephalitis (RE) is a rare immune-mediated condition characterized by drug-resistant focal epilepsy, progressive neurological, and cognitive deficits associated to unilateral hemispheric atrophy. The onset is typically reported in childhood, although adult cases (A-RE) have been described. While surgical strategies in childhood RE are well defined, little is known about usefulness of epilepsy surgery in A-RE patients. We describe clinical features, surgical approach, and outcome of five A-RE patients who underwent epilepsy surgery, and we review the literature with regard to surgical A-RE cases. We retrospectively studied five A-RE patients aged 21-38 years (mean age 22.8 years) who were followed after surgery for a period ranging from to 1 to 6 years. Demographic, electroclinical, and neuroimaging data were systematically reviewed. Four out of five subjects underwent invasive EEG monitoring to define epileptogenic zone. Epilepsy outcome was defined according to Engel's classification. Surgery consisted of frontal corticectomy in three patients, temporal lobectomy in one, combined temporal lobectomy plus insular, and frontobasal corticectomy in the remaining case. No permanent neurological deficits were observed after surgery. At the last follow-up observation, one patient was seizure-free, two subjects experienced rare disabling seizures, another had moderate seizure reduction, and one had no clinical improvement. Our experience, although limited to few cases, suggests that resective surgery in A-RE may play a role in the context of multidisciplinary therapeutical approach of this severe condition. Since the lack of specific data about surgical options, this topic seems to deserve further investigations and more targeted studies.

Oral manifestations of Parry-Romberg syndrome: A review of literature.

Parry–Romberg syndrome (PRS) or progressive facial hemiatrophy is a developmental craniofacial disorder of unknown etiology characterized by a slowly progressive unilateral facial atrophy. It is associated with different systemic manifestations particularly, maxillofacial, neurologic and ophthalmologic abnormalities. Dentists must be aware of PRS to identify this invalidating disorder. In this article, we review the etiology, clinical features (especially craniofacial and dental manifestations) and treatment of PRS. We searched in PubMed line using specific words such as PRS from 2008 to 2014 (August). We identify 14 papers have described oral manifestations of this syndrome. We excluded all the article papers that did not indicate to oral manifestations of PRS.