Unilateral Atrophy Research Articles

Unilateral Thigh Atrophy and Weakness in Hip Osteoarthritis

Osteoarthritis is a common condition that affects a significant percentage of people in the United States. The case and discussion will serve to introduce to the neurologist the issue of hip osteoarthritis causing thigh atrophy and weakness. The pathogeneses of muscular atrophy in the setting of osteoarthritis and of muscle weakness as a cause of osteoarthritis will be explored.

Distribution of regional gray matter abnormalities in a pediatric population with temporal lobe epilepsy and correlation with neuropsychological performance

Objective The goals of the work described here were to determine if hippocampal and extrahippocampal atrophy in children with temporal lobe epilepsy (TLE) follows a pattern similar to that in adult patients, and to assess the clinical and neuropsychological relevance of regional brain atrophy in pediatric TLE. Methods Children with symptomatic TLE ( n = 14: 9 with mesial TLE due to hippocampal atrophy and 5 with TLE due to neocortical lesions), healthy children ( n = 14), and 9 adults with mesial temporal lobe epilepsy (MTLE) were compared using voxel-based morphometry (VBM) of brain magnetic resonance imaging (MRI). The children underwent a comprehensive neuropsychological battery. Results Children with MTLE with unilateral hippocampal atrophy ( n = 9) exhibited a significant reduction in gray matter in the hippocampus ipsilateral to the seizure origin and significant atrophy in the ipsilateral cingulate gyrus and contralateral middle frontal lobe. Children with TLE ( n = 14) exhibited a significant reduction in the gray matter of the ipsilateral hippocampus and parahippocampal gyrus. There was a correlation between gray matter volume in children with TLE and scores on several neuropsychological tests. Atrophy in pediatric patients with MTLE was less extensive than that in adults, and involved the hippocampi and the frontal cortex. Conclusions Similar to adult MTLE, pediatric MTLE is associated with hippocampal and extrahippocampal cell loss. However, children display less intense quantifiable gray matter atrophy, which affects predominantly frontal lobe areas. There was a significant association between volume of gray matter in medial temporal and frontal regions and scores on neuropsychological tests. In childhood, TLE and the concomitant cognitive/behavior disturbances are the result of a damaged neural network.

Unilaterale pigmentierte paravenöse retinochorioidale Atrophie

We report the case of an asymptomatic unilateral pigmented paravenous retinochoroidal atrophy (PPRCA) in a 43-year-old patient. The right eye showed chorioretinal atrophy with bone-spicule-like pigmentations along the retinal veins. Visual acuity was 20 / 20 and perimetry revealed scotomas correlating to the chorioretinal atrophy. Electrophysiological examination showed decreased signals in ERG and EOG. Fundus autofluorescence and angiography findings are presented. Pathogenetically, a classification as hereditary retinal dystrophy (as in retinitis pigmentosa) as well as a post-inflammatory residuum are discussed.

Hippocampal sclerosis in association with neurocysticercosis

To discuss the possible mechanisms underlying a dual pathology combining neurocysticercosis and hippocampal atrophy, illustrated by the observation of four patients with epilepsy. The first patient presented at the age of four years with a first episode of status epilepticus, presumably due to an inflamed, calcified, parenchymal cysticercus granuloma. Thereafter, he had occasional seizures. Routine MRI undertaken several years later revealed unilateral hippocampal atrophy and sclerosis. Two other patients with initial imaging evidence of active neurocysticercosis located close to the hippocampus, and occasional seizures, developed ipsilateral hippocampal sclerosis. The seizure disorder of our fourth patient, with medically intractable epilepsy, was initially attributed to a calcified cysticercus granuloma. Clinical description, video-EEG telemetry and imaging work-up suggested a diagnosis of mesial temporal lobe epilepsy due to hippocampal sclerosis. Definitive conclusions as to the underlying mechanisms cannot be derived from the present, retrospective series of only four patients. However, the following suggestions can be made: 1) seizures due to neurocysticercosis may constitute the initial precipitating illness for the development of hippocampal sclerosis, 2) the hippocampus may be involved in host brain inflammation and gliosis in response to a nearby, degenerating cysticercus, 3) the seizure focus formed by the degenerating cysticercus, engenders epileptogenic changes in the hippocampus through kindling, and 4) the two conditions may coexist purely by chance. Systematic and prospective, serial MRI evaluations of hippocampal structures in patients with neurocysticercosis should contribute to further clarify the underlying mechanisms.

Influence of Unilateral Masseter Muscle Atrophy on Craniofacial Morphology in Growing Rabbits

The purpose of the study was to evaluate whether the induction of unilateral masticatory muscle dysfunction can alter the skeletal growth patterns. Twenty-one white male New Zealand rabbits (4 weeks old) were divided into 3 groups of 7 subjects: group 1 served as the control to study normal craniofacial growth. In groups 2 and 3, rabbits were injected with 5 units and 15 units of Botulinum toxin A (BTXA) into the right masseter muscle, respectively. The effect of a neuromuscular blockade of masseteric activity on craniofacial growth was evaluated with 3 samples of serial computed tomography (CT) scans with a slice thickness of 0.625 mm, taken at 4 weeks (base line), 8 weeks (endpoint of prepubertal craniofacial growth), and 24 weeks (after pubertal growth). The ipsilateral mandibular ramus height, zygomatic arch length, and masseteric length did not develop as much as those of the contralateral side after pubertal growth. At age 24 weeks, the masseter muscle volume asymmetry index reached -13.8% (group 2), -18.4% (group 3), and -1.6% for the control group. The ipsilateral side of the hemimandible showed less bone volume after 8 weeks but it showed partially recovered symmetry at 24 weeks. The maxillomandibular incisor midline and transverse molar discrepancies were not evident in any of the groups. The BTXA injection can be an effective method in inducing site-specific muscular hypofunctions so that masticatory muscle-craniofacial bone interaction can be investigated efficiently. The result showed that the unilateral atrophy of the masseter muscle in the growing subjects influenced the morphology of the local skeletal sites. This did not, however, result ultimately in mandibular midline asymmetry or right-left asymmetry in hemimandibular volume after growth. The results imply that alterations in specific masticatory muscle function can be compensated by the growth of other structural components.

Unilateral Lacrimal Gland Atrophy in a Patient With Hereditary Angioedema

To report a case of unilateral lacrimal gland atrophy in a patient with hereditary angioedema (HAE). Observational case report with literature review. A 32-year-old woman with HAE presented with dryness and irritation of the left eye (OS). Investigations revealed features of unilateral severe dry OS with atrophy of the ipsilateral lacrimal gland confirmed by magnetic resonance imaging (MRI). Dry eye should be included in the ocular manifestations of HAE. Recurrent mucosal and soft-tissue inflammation secondary to HAE may lead to lacrimal gland atrophy. MRI is a useful, noninvasive tool in diagnosing lacrimal gland atrophy.

Progressive Unilateral Hemispheric Atrophy in an Infant with Neurofibromatosis

Cerebrovascular diseases are rarely seen in neurofibromatosis type 1. These include vascular occlusive disease, moyamoya vessels, aneurysms, arteriovenous malformations and fistulae. We describe the case of an infant with genetically proven neurofibromatosis type 1 and progressive brain hemiatrophy over months, due to primary narrowing of intracranial carotid artery branches, as demonstrated by successive brain imaging. She presented with refractory seizures and a progressive hemiparesis associated with developmental delay. Surgical material from hemispherotomy done at 18 months showed severe abnormalities of the small vessels. Cerebrovascular changes seen in neurofibromatosis can be diffuse and progressive, with secondary hemiparesis, epilepsy and developmental delay.

Extrahippocampal gray matter atrophy and memory impairment in patients with medial temporal lobe epilepsy

Memory impairment observed in patients with medial temporal lobe epilepsy (MTLE) is classically attributed to hippocampal atrophy. The contribution of extrahippocampal structures in shaping memory impairment in patients with MTLE is not yet completely understood, even though atrophy in MTLE extends beyond the hippocampus. We aimed to evaluate the neuropsychological profile of patients with MTLE focusing on memory, and to investigate whether gray matter concentration (GMC) distribution within and outside the medial portion of the temporal lobes would be associated with their neuropsychological performance. We performed a voxel based morphometry study of 36 consecutive patients with MTLE and unilateral hippocampal atrophy. We observed a significant simple regression between general and verbal memory performance based on Wechsler Memory Scale-Revised and the GMC of medial temporal and extratemporal structures in patients with left MTLE. We also performed a "regions of interest analysis" of the medial temporal lobe, and we observed that the GMC of the hippocampus, entorhinal, and perirhinal cortices were consistently associated with general and verbal memory performance in patients with MTLE. We also observed that the GMC of the cingulate and orbito-frontal cortex are independently associated with verbal and general memory performances. Our results suggest that general and verbal memory impairments in patients with left MTLE are associated with atrophy of the hippocampus, the entorhinal, and the perirhinal cortex. We also suggest that atrophy and dysfunction of limbic and frontal structures such as the cingulate and the orbito-frontal cortex contribute to memory impairment in MTLE.

Adult‐Onset Rasmussen's Encephalitis: Anatomical‐Electrographic‐Clinical Features of 7 Italian Cases

A limited number of cases of adult-onset Rasmussen's encephalitis (A-RE) have been reported, but the features of the syndrome are still unclear. The aim of this study was to verify the clinical features of A-RE, and outline a noninvasive approach that may allow its early diagnosis and treatment. Retrospective evaluation of extensive noninvasive work-up of seven patients with A-RE, including repeat clinical, neurophysiological, and neuroimaging investigations. The study identified two distinct patterns of disease presentation, one characterized by focal motor epilepsy (the "epileptic" phenotype), and the other by focal cortical myoclonus (the "myoclonic" phenotype). Unilateral neurological deficits and brain atrophy were progressive in both phenotypes, but they were more prominent and were detected earlier in the "epileptic" phenotype. The anatomo-electroclinical features of these patients allowed a noninvasive diagnosis of A-RE and identification of two distinct disease phenotypes. Early noninvasive diagnosis can allow faster initiation of treatment.

Progressive leftsided cerebellar atrophy and intermittend CSWS-like EEG. Is it Rasmussen Encephalitis?

Objective: Is there more than coincidence of developmental delay, CSWS-like episodes in EEG and progressive unilateral cerebellar atrophy?

Unilateral Atrophy of Fungiform Papillae Associated with Lingual Nerve Injury

The lingual nerve is at risk of injury during common dental procedures because of its proximity to the inferior alveolar nerve and the roots of the third molar tooth. Lingual nerve injury has been documented following extraction of wisdom teeth, dental anesthetic injections, and other endodontic procedures. We present a case of loss of somatosensory function and taste associated with the intriguing finding of fungiform papillae atrophy as a result of lingual nerve injury.

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.

Tailored anteromedial lobectomy in the treatment of refractory epilepsy of the temporal lobe: Long term surgical outcome and predictive factors

Objective To analyze long-term results and to determine prognostic factors on seizure outcome in a series of patients with temporal lobe epilepsy (TLE) who underwent anteromedial temporal lobectomy (AMTL). Materials and methods From 1995 to 1998 forty-two patients suffering from non-lesional TLE underwent tailored AMTL at our Institution. We retrospectively reviewed surgical results and calculated predictive factors of good outcome in the long term. Results Sixty-four percent of patients were rendered seizure free (median follow up 60 months). Eleven cases (26.2%) had a significant reduction of disabling epileptic episodes. Poor seizure control was observed in four patients (9.5%). Overall surgical morbidity was 4.7%. Medial temporal sclerosis (MTS) was the most common histopathological finding (69% of cases). The presence of unilateral hippocampal abnormalities on qualitative MRI was significantly associated with excellent postoperative outcome ( p < 0.011). Qualitative preoperative MRI had a positive predictive value of 83% in detecting both MTS at pathological examination and excellent outcome. Conclusions Tailored AMTL is a safe and effective procedure in the treatment of selected patients with medically refractory TLE. Data from preoperative qualitative MRI well correlated with histopathological findings. The presence of unilateral hippocampal atrophy on qualitative MRI was predictive of excellent outcome in the long-term follow up.

Hemiconvulsion–hemiplegia–epilepsy syndrome: Early magnetic resonance imaging findings and neuroradiological follow-up

We describe a case of hemiconvulsion–hemiplegia–epilepsy (HHE) syndrome documented by longitudinal magnetic resonance imaging (MRI). A two-year and nine-month-old boy had a prolonged hemiconvulsion during fever followed by right hemiparesis. Seven days later the imaging abnormality on T2 and diffusion-weighted images (DWI) was limited to the white matter of the left hemisphere. One month later severe gliosis and unilateral brain atrophy were already evident. MRI is useful in the early stages of prolonged seizures and T2 and DWI abnormalities appear to be well correlated with parenchymal damage that results from sustained ictal activity. The neuroradiological findings in our case and in the few HHE patients reported in the literature seem to be very characteristic and, if confirmed in larger series, could permit an early diagnosis.

Treatment of 'en coup de sabre' Deformity with Porous Polyethylene Implant

Linear scleroderma "en coup de saber" (LSCS) deformity is a subgroup of scleroderma characterized by unilateral atrophy and depression above the level of the eyebrow. In rare cases where it involves the whole unilateral face it can be mistaken with Parry-Romberg syndrome. Involvement of the trunk and extremities together with facial lesions has been extremely rare in the literature. We report an unusual case of linear scleroderma "en coup de sabre" where there is concomitant involvement of the ipsilateral side of the trunk and lower extremity. The patient's facial deformity is reconstructed with a polyethylene implant.

Dynamic MR perfusion and proton MR spectroscopic imaging in sturge‐weber syndrome: Correlation with neurological symptoms

To investigate physiological alterations in Sturge-Weber syndrome (SWS) using MR perfusion imaging (PWI) and proton spectroscopic imaging (MRSI), and their association with neurological status. Six consecutive patients with a clinically established diagnosis of SWS underwent MRI using a 1.5 Tesla scanner. The protocol consisted of conventional anatomic scans, dynamic PWI, and multislice MRSI. A pediatric neurologist evaluated the neurological scores, and the imaging results were correlated with neurological scores using nonparametric correlation analysis. Two patients had classic neuroimaging findings of unilateral cerebral atrophy with corresponding leptomeningeal enhancement and hypoperfusion (prolonged mean transit time). Two patients had bilateral disease, and two had normal symmetric perfusion. Among clinical measures, the highest correlation was between hemiparesis index and hypoperfused tissue volume (Spearman's correlation coefficient, rho = 0.943, P < 0.05). There was also a trend of correlation, although not statistically significant (P = 0.06), between the hemiparesis score and the NAA/Cr ratio in the mid to posterior centrum semiovale, lateral gray matter (GM), and splenium. In SWS, PWI indicates cerebral hypoperfusion predominantly due to impaired venous drainage, with only the most severely affected regions in some patients also showing arterial perfusion deficiency. The extent and severity of the perfusion abnormality and neuronal loss/dysfunction reflect the severity of neurological symptoms and disability, and the highest correlation is found with the degree of hemiparesis. These parameters may be useful as quantitative measures of disease burden; however, further studies in larger number of patients (and with a more homogeneous age range) are required to confirm the preliminary findings reported here.

Morphologic and Volumetric Analysis of Amygdala, Hippocampus, Fornix and Mamillary Body with MRI in Patients with Temporal Lobe Epilepsy

Our aim was to demonstrate lateralization morphometrically and volumetrically measuring the main limbic structures like hippocampus, amygdala, fornix and mamillary bodies in patients with temporal lobe epilepsy (TLE) and to establish the contribution of each anatomical structure to lateralizing the pathological site. Forty-two patients with complex partial seizures originating from the temporal lobe and 42 control healthy volunteers were included in the study. T2-weighted FSE sequences in axial and oblique coronal planes and T1-weighted SE sequences in the sagittal plane were used. A high-resolution IR sequence was used for the volumetric analysis of amygdala and hippocampus and for the measurement of fornix and mamillary body thickness. Intensity changes and atrophy of limbic structures were observed qualitatively and measurement of these structures was performed quantitatively. Student's t test and Mann-Whitney U-test were used for the statistical analysis. The p values <0.05 was taken as statistically significant. Ten out of 42 patients had intracranial masses and were excluded from the study. Qualitative analysis revealed atrophy in 84% and intensity increase in 60% of cases. Quantitative measurement demonstrated that control cases had a larger hippocampus than the patients ( p<0.001). There was unilateral hippocampal volume loss in 88% and bilateral volume loss in 13% of patients. There was no difference in the volume of amygdala between the groups ( p>0.05). According to the difference in the volumes of the right and left sides, there was unilateral atrophy in 34% of patients. Bilateral atrophy was not observed. There was a significant difference in fornix and mamillary bodies of the patients and control subjects ( p<0.005). In 62.5% of cases, there were abnormalities in the fornix with bilateral involvement in 13% of cases. Mamillary bodies were abnormal in 37% of patients with bilateral involvement in 15%. Lateralization was accomplished in 65% of the patients according to the percentage difference ratios of fornix and in 38% of the patients according to the mamillary bodies. It is important to demonstrate hippocampus atrophy in patients with TLE. In the decision of lateralization of the epileptic side, evaluation of the fornix could be a good and practical solution. However, hippocampal volumetry is an indispensable criterion in demonstrating hippocampal atrophy more accurately. Atrophy of the amygdala and thickness of mamillary bodies have the least importance for lateralization.

Esclerose hipocampal unilateral com registro subdural de crises temporais contralaterais: relato de caso

INTRODUÇÃO: Pacientes com epilepsia temporal e esclerose hipocampal unilateral tem, na sua grande maioria das vezes, as suas crises e as alterações eletroencefalográficas interictais originando-se do lado da lesão. Mesmo alguns pacientes nos quais os registros de escalpo mostram as crises originando-se da região temporal contralateral à atrofia do hipocampo, registros invasivos têm mostrado que a região de início das crises é, na realidade, ipsilateral à atrofia. No entanto isso talvez não seja verdadeiro para todos pacientes. OBJETIVO: Nesse artigo é relatado uma paciente com atrofia hipocampal unilateral, mas cujas crises, tanto no escalpo quando com registros subdurais, mostram vir da região temporal contralateral. RESULTADO: A paciente tem atrofia hipocampal à direita mas cujo EEG interictal mostrava predomínio de descargas à esquerda, bem como crises vindo daí. O registro com strips subdurais mostrou origem das crises à esquerda. CONCLUSÃO: Possivelmente alguns pacientes com epilepsia temporal têm as crises vindo do hipocampo menos atrófico.

Quantification of retinal nerve fiber layer thickness reduction associated with a relative afferent pupillary defect

A relative afferent pupillary defect (RAPD) is known to develop only when more than 25% of retinal ganglion cells are ablated in monkeys' eyes. However, there was no prior study to estimate biometrically the degree of retinal nerve fiber layer (RNFL) thickness reduction leading to the development of RAPD in live human eyes. The purpose of this study was to examine the correlation between the amount of RNFL thickness reduction and the depth of a clinically detectable RAPD in patients with unilateral optic atrophy. Enrolled were 20 patients with optic atrophy of various etiologies. We quantified RAPD by performing the swinging flashlight test with log-scaled neutral density filters placed over the unaffected eye. Average RNFL thickness was measured by OCT3000 with the average RNFL thickness program. Linear regression analysis was used in assessing the relationship between RAPD and the ratio of affected to unaffected average RNFL thickness. The mean of average RNFL thickness was 95.6+/-17.3 microm in the unaffected eyes and 50.7+/-19.3 microm in the affected eyes (P<0.001). Regression analysis between RAPD and the ratio of affected to unaffected average RNFL thickness revealed a correlation coefficient R2=0.48 (P=0.0007). The regression line intersected the y-axis at 0.77. RAPD was not clinically detected until at least approximately 25% of the retinal nerve fibers were lost when compared with the unaffected eyes. Substantial retinal ganglion cell damage is required for the development of RAPD.

Memory and language impairments and their relationships to hippocampal and perirhinal cortex damage in patients with medial temporal lobe epilepsy

Chronic medial temporal lobe epilepsy (MTLE) is associated with memory loss due to damage in the hippocampal system. To investigate the relationship between volume of medial temporal lobe structures and performance on neuropsychological tests, we studied 39 consecutive patients with MTLE and unilateral hippocampal atrophy (HA) determined by volumetric magnetic resonance imaging (MRI). Structures of interest comprised hippocampus, amygdala, and entorhinal, perirhinal, parahippocampal, and temporopolar cortices. The findings indicated that (1) performance was significantly worse in the group with left HA as compared with the group with right HA on general memory, verbal memory, delayed recall, and verbal fluency tests and the Boston Naming Test (BNT), and (2) the volume of the left hippocampus and also the degree of asymmetry of perirhinal cortex volume were significant and independent predictors of performance on general memory, verbal memory, and verbal fluency tests and the BNT in patients with MTLE.