Temporal Lesions Research Articles

Cerebral Toxoplasmosis in a Previously Fit Individual – Pitfalls in Management – A Case Report.

Most people with toxoplasmosis infection are asymptomatic. Cerebral toxoplasmosis in HIV patient represents poor prognostic determinant, but treatable if early treatment is initiated. A case of delayed diagnosis of cerebral toxoplasmosis is presented. A 27-year-old man presented with one-sided facial numbness and treated as Bell’s Palsy. Later he developed slurred speech. Contrast enhanced CT brain showed left frontal temporal hypodense lesion, leptomeningeal enhancement and perilesional oedema. Steroid was started. Four days later, he presented with decreased responsiveness. Retroviral was detected and diagnosis changed to opportunistic brain infection. He died after 3 days. Toxoplasmosis IgG antibodies turned out positive. In diagnosing cerebral toxoplasmosis, clinical presentation and examination are as important as laboratory testing and radiological imaging.

Music perception in acquired prosopagnosia

BackgroundAcquired prosopagnosia is often associated with other deficits such as dyschromatopsia and topographagnosia, from damage to adjacent perceptual networks. A recent study showed that some subjects with developmental prosopagnosia also have congenital amusia, but problems with music perception have not been described with the acquired variant. ObjectiveOur goal was to determine if music perception was also impaired in subjects with acquired prosopagnosia, and if so, its anatomic correlate. MethodWe studied eight subjects with acquired prosopagnosia, all of whom had extensive neuropsychological and neuroimaging testing. They performed a battery of tests evaluating pitch and rhythm processing, including the Montréal Battery for the Evaluation of Amusia. ResultsAt the group level, subjects with anterior temporal lesions were impaired in pitch perception relative to the control group, but not those with occipitotemporal lesions. Three of eight subjects with acquired prosopagnosia had impaired musical pitch perception while rhythm perception was spared. Two of the three also showed reduced musical memory. These three reported alterations in their emotional experience of music: one reported music anhedonia and aversion, while the remaining two had changes consistent with musicophilia. The lesions of these three subjects affected the right or bilateral temporal poles as well as the right amygdala and insula. None of the three prosopagnosic subjects with lesions limited to the inferior occipitotemporal cortex exhibited impaired pitch perception or musical memory, or reported changes in music appreciation. ConclusionTogether with the results of our previous studies of voice recognition, these findings indicate an anterior ventral syndrome that can include the amnestic variant of prosopagnosia, phonagnosia, and various alterations in music perception, including acquired amusia, reduced musical memory, and subjective reports of altered emotional experience of music.

Massive jugular foramen schwanoma revealed by the hypogloss paralysis

Jugular foramen schwannomas are a rare event with an estimated 200 cases reported in the literature. These tumors represent only 2.9% to 4% of all intracranial schwannomas and less than 1% of all temporal bone lesions [1,2].

The Right Temporal Lobe and the Enhancement of Voice Recognition in Congenitally Blind Subjects

Experimental investigations and clinical observations have shown that not only faces but also voices are predominantly processed by the right hemisphere. Moreover, right brain-damaged patients show more difficulties with voice than with face recognition. Finally, healthy subjects undergoing right temporal anodal stimulation improve their voice but not their face recognition. This asymmetry between face and voice recognition in the right hemisphere could be due to the greater complexity of voice processing. To further investigate this issue, we tested voice and name recognition in twelve congenitally blind people. The results showed a complete overlap between the components of voice recognition impaired in patients with right temporal damage and those improved in congenitally blind people. Congenitally blind subjects, indeed, scored significantly better than control sighted individuals in voice discrimination and produced fewer false alarms on familiarity judgement of famous voices, corresponding to tests selectively impaired in patients with right temporal lesions. We suggest that task difficulty is a factor that impacts on the degree of its lateralization.

Pigmented Inflamed Seborrheic Keratosis of the Bulbar Conjunctiva.

A 57-year-old Black man presented with the recent onset of a pigmented temporal epibulbar lesion. As pigmentation of conjunctival epithelial lesions is correlated with complexion pigmentation, the lesion was presumed to represent a pigmented ocular surface squamous neoplasia (OSSN). Excisional biopsy, however, revealed a pigmented conjunctival seborrheic keratosis, a rare occurrence. The lesion lacked cytologic atypia. Intralesional processes of dendritic melanocytes were demonstrated by hematoxylin-eosin and Melan-A stains. Melanophages also contributed to clinical pigmentation. Subepithelial lymphocytic infiltration, elevated Ki67 proliferative rate, prominent mitotic activity, and subtle spongiosis afforded evidence of inflammation rather than malignancy in a lesion devoid of cytologic atypia.

A human T-lymphotropic virus-1 carrier who developed progressive multifocal leukoencephalopathy following immunotherapy for sarcoidosis: a case report

BackgroundProgressive multifocal leukoencephalopathy (PML) is a devastating demyelinating disorder of the central nervous system caused by opportunistic infection of the JC virus (JCV).Case presentationA 58-year-old Japanese woman was admitted to our hospital for aphasia. She had a 5-year history of untreated sarcoidosis and was a human T cell lymphotropic virus-1 (HTLV-1) carrier. Serum angiotensin-converting enzyme, soluble interleukin-2 receptor, lysozyme, and calcium levels were elevated. JCV-DNA was not detected in cerebrospinal fluid by PCR testing. Skin biopsy revealed noncaseating granuloma formation. Bilateral multiple nodular lesions were present on chest X-ray. Brain magnetic resonance imaging showed left frontal and temporal lesions without gadolinium enhancement. As we suspected that systemic sarcoidosis had developed into neurosarcoidosis, we started steroid and infliximab administration. After treatment, the chest X-ray and serum abnormalities ameliorated, but the neurological deficits remained. At 1 month after immunotherapy, she developed right hemiparesis. Cerebrospinal fluid was positive for prototype (PML-type) JCV on repeated PCR testing. Brain biopsy revealed demyelinating lesions with macrophage infiltration, atypical astrocytes, and JCV antigen-positive cells. We diagnosed her with PML and started mefloquine, leading to partial remission.ConclusionsSarcoidosis and HTLV-1 infection both affect T cell function, especially CD4+ T cells, and may developped the patient’s PML. The comorbidity of sarcoidosis, PML, and HTLV-1 infection has not been reported, and this is the world’s first report of PML associated with HTLV-1 infection and sarcoidosis.

Clinicoradiologic data of familial cerebral cavernous malformation with age-related disease burden.

Familial cerebral cavernous malformation (FCCM) is an autosomal dominant disease induced by loss-of-function mutations in three CCM genes, KRIT1, CCM2, and PDCD10. However, previous studies paid little attention to analyzing the radiologic features and age-related disease burden according to the genes. Therefore, we retrospectively reviewed the genetic tests of our center's clinical FCCM patients. This study investigated clinical FCCM patients with multiple lesions or a family history of CCMs who underwent the FCCM gene (KRTI1, CCM2, and PDCD10) panel test. The clinical, genetic, and radiologic features were analyzed. Among the patients (n=34) undergoing the FCCM gene test, twenty-seven patients had CCM confirmed by brain MRI, and twenty-one patients were considered to have FCCM (cohort 1). In cohort 1, thirteen patients had mutations in the FCCM gene, but eight did not. Cohort 2 comprised cohort 1 and four family members with the same mutation as the probands. Six novel variants in CCM genes were detected (KRIT1 c.22_26del, c.815dup, c.1094_1098del, c.1147-2A>G, c.2124dup, and PDCD10 c.150 + 1dup). Cohort 1 demonstrated that brainstem lesions were mostly associated with the mutation detection in CCM genes (brainstem, lateral temporal, and parietal lesions vs. lateral temporal and parietal lesions, AUC 0.928 vs. 0.779, P=0.0389). The radiologic severity worsened according to age in the KRIT1 group compared with the Mutation not detected group (correlation coefficient 0.75 (P < 0.001) versus 0.53 (P=0.004)). The brainstem lesion could be the radiologic marker for FCCM with the mutation detected. The age-related disease burden regarding FCCM according to genetic information was demonstrated.

Are brain MRI abnormalities associated with the semiology of functional seizures?

To investigate whether radiologically apparent brain magnetic resonance imaging (MRI) abnormalities are associated with the functional seizure (FS) semiology. All patients with a diagnosis of FS at the epilepsy centers at Shiraz University of Medical Sciences, Iran; Aichi Medical University Hospital, Japan; University of Michigan, USA; University of California, Los Angeles, USA; Emory University School of Medicine, USA; and Hospital el Cruce, Argentina, were studied. One hundred patients were included; 77 (77%) had motor functional seizures. Lobar location of brain abnormality did not have an association with the semiology (p=.83). There was no significant difference between ictal behaviors in patients with frontal or parietal lesions compared to those with temporal or occipital lesions. There were no associations between functional seizure ictal behaviors and locations of the radiologically apparent brain MRI abnormalities. Further studies are needed to evaluate the underpinnings of varying behaviors in FS.

Rapamycin Plays an Anti-Epileptic Role by Restoring Blood-Brain Barrier Dysfunction, Balancing T Cell Subsets and Inhibiting Neuronal Apoptosis.

Rapamycin (RAP), as a Mammalian Target of Rapamycin (mTOR) inhibitor, has a certain antiepileptic effect. The blood-brain barrier (BBB), neuroinflammation, lymphocyte immune cells, and neuronal apoptosis play an obligatory role in the course of a seizure. The aim of this study is to probe whether the antiepileptic mechanism of RAP involves the blood-brain barrier, neuroinflammation, lymphocytes, and neuronal apoptosis. First, we established a rat epilepsy model by injecting lithium chloride and pilocarpine into the rats (intraperitoneal injection). Then the epileptic rats were treated with different doses of RAP (1 mg/kg.d, 2 mg/kg.d, 4 mg/kg.d). Peripheral blood, brain tissue, and temporal lobe tissue were collected. The levels of blood-brain barrier-related proteins and inflammatory cytokines in the peripheral blood of rats were measured by enzyme-linked immunosorbent assay (ELISA). The effect of RAP on T cell subsets in epileptic rats was analyzed by flow cytometry. The apoptosis of neurons and glial cells in the temporal lobe of rats was analyzed by immunohistochemistry. This study found that RAP reduces the levels of BBB-interrelated proteins (matrix metallopeptidase 9 (MMP-9), MMP-2, tissue inhibitor of metalloproteinases 1 (TIMP-1), TIMP-2) and inflammatory cytokines (interleukin-2 (IL-2), interferon-γ (IFN-γ), tumor necrosis factor-α (TNF-α)) in epileptic rats compared to the model group (p < 0.05). RAP increases the level of total T cells (CD3+CD45+) and T helper cells (CD3+CD4+), decreases the level of cytotoxic T lymphocytes (CD3+CD8+), and inhibits the apoptosis of neurons and glial cells in the temporal lobe compared to the model group (p < 0.05). The antiepileptic mechanism of RAP may be to restore BBB dysfunction, reduce the inflammatory response, balance T cell subsets, and inhibit neuronal and glial cell apoptosis in temporal lobe epilepsy lesions.

PATH-40. A GIANT CELL LESION RESEMBLING REPARATIVE GIANT CELL GRANULOMA WITH FN1-TEK FUSION, IN THE SPECTRUM OF CALCIFIED CHONDROID MESENCHYMAL NEOPLASM?

Abstract A newly described entity called “calcified chondroid mesenchymal neoplasms” includes chondroid neoplasms with variable amounts of calcification and FN1 fusions [Liu et al, Mod Pathol 2021]. We report a case of a left temporal bone giant cell granuloma-like lesion that also shows an FN1-TEK fusion. Close monitoring with MRI images after surgery has showed no recurrence. Case report: a 33-year-old male developed left jaw pain and he was diagnosed with a temporomandibular joint disorder. He felt left ear fullness and 2 years later experienced severe pain and swelling in the left temporal region. Imaging showed an expansile osseous lesion with multi-cystic components involving the left sphenoid bone as well as all portions of the temporal bone including the mastoid, middle ear cavity, and glenoid fossa. Pathology showed a hemorrhagic giant cell lesion that was negative for H3G34W mutation and USP6 rearrangement, suggesting a reparative giant cell granuloma. A subsequent NGS with the Tempus 648 gene panel did not identify somatic or germline mutations, but an additional Tempus RNA fusion test found a FN1-TEK fusion suggesting the possibility of neoplasia. Close monitoring with brain MRI over past 9 months has shown no signs of recurrence. DISCUSSION A recent small series describes a novel entity called calcified chondroid mesenchymal neoplasm that has FN-1 fusions including a FN1-TEK fusion in one case. The case in that series with FIN1-TEK fusion also showed prominent giant cells. It is unclear whether giant cell granuloma-like lesions with FN1-TEK fusion belong in the calcified chondroid mesenchymal neoplasm spectrum or are a separate non-neoplasm entity that shares the same alteration. More studies are warranted.

Like a rolling stone: Psychotherapy without (episodic) memory.

People with profound amnesia still retain the capacity to learn about the emotional value of experiences, which is crucial in developing and sustaining interpersonal relationships. In a 2017 paper, we demonstrated for the first time (with patient JL) that transferential feelings develop across the therapeutic process, despite profound episodic memory impairment after medial temporal lesions. This paper reports a second case (GA) of a profoundly amnesic patient in psychotherapy, this time after lesions to the anterior fornix. The work with GA opens issues such as the differences and similarities to the previous case, counter-transference phenomena, and the effects of hyperphagia. The findings make it clear that many phenomena are common to both GA and JL, such as forgetfulness, various types of repetition, the importance of the therapeutic alliance, and the ability to make therapeutic gain. However, there were differences between the cases, for example as regards confabulation, which may relate to either pre-morbid personality or lesion site. The paper also discusses the way in which patients of this type bear the very status of psychotherapeutic work with profoundly amnesic patients. Where others have seen barriers and in principle problems in working with such patients, we see many opportunities.

Aphasic Syndromes in Cerebral Venous and Dural Sinuses Thrombosis—A Review of the Literature

Aphasia is an acquired central disorder of language that affects a person's ability to understand and/or produce spoken and written language, caused by lesions situated usually in the dominant (left) cerebral hemisphere. On one hand aphasia has a prevalence of 25-30% in acute ischemic stroke, especially in arterial infarcts. On the other hand, cerebral venous and dural sinuses thrombosis (CVT) remains a less common and underdiagnosed cause of ischemic stroke (0.5-1% of all strokes). Aphasia has been observed in almost 20% of patients who suffered CVT. The presence of aphasia is considered a negative predictive factor in patients with stroke, severe language disorders corresponding to arduous recovery. Taking into consideration data from the literature, aphasia is also considered a predictive factor for patients with CVT; its absences, together with the absence of worsening after admission, are determinants of complete recovery after CVT. This review has as the principal role of gathering current information from the literature (PubMed database 2012-2022) regarding the clinical features of aphasic syndromes and its incidence in patients with CVT. The main conclusion of this review was that aphasic syndromes are not usually the consequence of isolated thrombosis of dural sinuses or cerebral veins thrombosis. The most frequent form of CVT that determines aphasia is represented by the left transverse sinus thrombosis associated with a posterior left temporal lesion (due to left temporal cortical veins thrombosis), followed by the superior sagittal sinus thrombosis associated with a left frontal lesion (due to left frontal cortical veins thrombosis). Only a few cases are presenting isolated cortical veins thrombosis and left thalamus lesions due to deep cerebral vein thrombosis. We also concluded that the most important demographic factor was the gender of the patients, women being more affected than men, due to their postpartum condition.

Rhino-Orbital-Cerebral Mucormycosis: A Rare Complication of Uncontrolled Diabetes

Introduction Fungal infection of the central nervous system has become more common over the past two decades. It is frequently diagnosed in patients with underlying pathological conditions. We herein report a case of rhino-orbital-cerebral mucormycosis by outlining the clinical presentation, radiological images, histopathological findings, management plan, and its clinical outcome. Case Description. A 47-year-old man, known to have type 2 diabetes mellitus, presented with severe headache involving the left side of the face, numbness along the left V2 trigeminal nerve, ptosis and dryness of the left eye, short-term memory loss, and right hand numbness. He had a social history of being a bee farmer for which he was exposed to bee stings several times in the past. Neuroradiological imaging showed a left temporal ring-enhancing lesion, suggestive of abscess. The patient underwent craniotomy and resection of the lesion. The histopathological evaluation was suggestive of cerebral mucormycosis, fungal sinusitis, and invasive skull base mucormycosis. Conclusion Rhino-orbital-cerebral mucormycosis is a fulminant fungal infection commonly diagnosed in patients with uncontrolled diabetes. Early diagnosis with radiological and histopathological evaluation is required to identify patients at risk of rhino-orbital-cerebral mucormycosis.

S2789 A Rare Triad of Ulcerative Colitis, Large Vessel Vasculitis and Celiac Disease

Introduction: Inflammatory bowel disease (IBD) has been associated with large-vessel vasculitis (LVV), with the diagnosis of IBD preceding that of LVV by years. We present for the first time in known literature a triad of concurrent ulcerative colitis (UC), aortitis and celiac disease. Case Description/Methods: A 58 year old Hispanic man with a history of hypertension and gout presented with two weeks of intractable temporomandibular headaches, and two months of non-bloody diarrhea and weight loss. Physical exam was unremarkable. Labs showed hemoglobin 6.9 g/dL, erythrocyte sedimentation rate 120 mm/hr, C-reactive protein 281 mg/dL and IgA tissue transglutaminase antibody 23 U/mL. ANA, C3, C4, proteinase-3 and myeloperoxidase antibodies were within normal limits. Colonoscopy showed pancolitis from rectum to ascending colon. The terminal ileum was normal. Abdominal MRI found aortic wall hyperintensity from the renal arteries to common iliac bifurcation. CT angiogram showed wall thickening of the left carotid artery, aortic arch, descending thoracic and abdominal aorta, consistent with vasculitis. Patient was given stress dose steroids with improvement in headache and normalization of ESR and CRP. Temporal artery biopsy was unremarkable. Four months after hospitalization, repeat colonoscopy with duodenal biopsies for celiac disease revealed mild increase in intraepithelial lymphocytes with preserved villous architecture. He was started on a gluten free diet and adalimumab in combination with methotrexate for UC and LVV. Discussion: About 10 case reports of patients with both UC and either Takayasu (TAK) or giant cell arteritis (GCA) have been described, with UC typically diagnosed 15-45 years before the vasculitis. Vasculitis in the GI tract can mimic IBD, making colonoscopy and biopsy crucial for diagnosis. HLA haplotypes A24, B52, and DR2 are associated with both UC and aortitis and Interleukin-9, observed in temporal arteritis lesions, may be implicated in the pathogenesis of UC. Shared chromosomal variants between patients with UC and celiac disease may explain why IBD risk is up to 9-fold higher in patients with celiac disease. Our patient may have presented with isolated aortitis or an early form of GCA. Methotrexate is used to treat LVV and is combined with an anti-TNF agent to treat UC, as in our case. This is the first known report of co-occurring UC, celiac disease and aortitis; however, whether the three inflammatory conditions are mechanistically related warrants further research.

Assessment of lesion development in Fraxinus excelsior cultivars Altena, Atlas and Westhof's Glorie inoculated with different isolates of Hymenoscyphus fraxineus

AbstractAsh dieback caused by the invasive fungus Hymenoscyphus fraxineus leads to massive mortality among common ash (Fraxinus excelsior) in Europe. To select tolerant genotypes, inoculation experiments are frequently conducted with isolates of the fungus. The aim of this study was to improve the inoculation methodology for evaluating susceptibility of ash genotypes to ash dieback through (i) testing the virulence of different isolates of Hymenoscyphus fraxineus and (ii) investigating temporal lesion development using three widely applied cultivars: Altena, Atlas and Westhof's Glorie. First, an experiment was conducted with a repeated measure design in which virulence of five different isolates was tested. Second, a subset of two isolates that induced the longest downward lesion length in experiment one was used in combination with a slightly adjusted inoculum protocol. Significant differences were found between isolates for downward lesion length, but a significant interaction effect of isolates and cultivars was absent. Also, the inoculation position within the stem affected lesion length; the largest lesions were found on the highest inoculation position within the stem. Furthermore, we found that cryopreserved isolates can remain virulent over years. The timing of inoculation at the end of the growing season was effective as large lesions already occurred during winter dormancy. For future inoculation studies, we propose to use: (i) isolates that induce large lesions, as less virulent isolates induced not only shorter but also fewer lesions; (ii) a similar inoculation position to better compare inoculations within and between experiments; (iii) cryopreserved isolates for testing over years; (iv) mycelial suspensions for inoculum preparation to cover wood chips more evenly; (v) reference clone—like the studied cultivars—to standardize research outcomes between years and research groups.

P16.03.A Epithelioid gliobastoma requires rapid treatment and BRAF inhibitors should be made readily available for their treatment

Abstract Background Epithelioid glioblastoma is a rare subtype of Glioblastoma. We examine two cases who presented acutely with symptoms of headache, neck stiffness and an eye squint. The purpose of this case review is to look at their management, the spread of the disease and propose the availability of BRAF inhibiting agents be made readily available for this subtype. Material and Methods The clinical records including pathology and surgical reports, multi-disciplinary team meeting documents, oncology plans and inpatient notes have all been reviewed alongside the literature on epithelioid GBM and BRAF V600e mutations and inhibitors. Results Patients were females aged 25 and 32 presenting with acute onset headache and neck stiffness to emergency Department. The 25 year old had developed a right eye squint within seven days of the other symptoms, her tumour was right frontal with midline shift. The 32 year old had symptoms for 10 days prior to presentation, the tumour was right temporal. They both underwent craniotomies. The frontal tumour was totally resected, while the temporal lobe tumour was haemorrhagic in nature and minimally de-bulked. Pre-operative stealth imaging showed that there had been an increase in the size of the temporal lesion in the fourteen days since presentation. Histology proved these to be epithelioid GBM’s with BRAF V600e mutations, IDH wild-type and TERT promoter mutant. Full pathology reports with molecular markers were available within ten days. The frontal lobe patient began chemo-radiation sixteen days from her surgical date. On day two she was admitted with severe headache and nausea. She became agitated, confused, and transferred back to the neurosurgery unit for management of hydrocephalus. She was diagnosed with PRES and simultaneously treated for this and hydrocephalus. Clinically she suffered storming, passing away exactly eight weeks from presentation. Seven days after surgery the temporal lobe tumour patient suffered a seizure and admitted for symptom management and expedite oncology treatment. She passed away six days later suffering continual neurological deterioration and the tumour progression with leptomeningeal spread. This was exactly four weeks from initial presentation. Conclusion The prognosis for epithelioid Glioblastoma is limited to weeks to short months. Extent of resection in these case studies demonstrates benefit in delaying progression though it is clear that time is of the essence from presentation, to surgery, to adjuvant treatment. Neither of these tumours were methylated meaning the standard treatment for glioblastoma is likely to lack efficacy. BRAF inhibitors should be made readily available for this rare sub-type to commence treatment expediently. Both patients suffered distressing neurological symptoms in their final days which require expert management and are best managed in a neurosurgical centre.

Imaging features, staging system, and surgical management of giant cell lesions of the temporal bone

Background Giant cell tumors (GCTs) and giant cell granulomas (GCGs) are giant cell-rich lesions that occur extremely rarely in the temporal bone and have similar clinical presentations. Objectives We aimed to analyze the clinical features and introduce our staging system and surgical treatment. Methods Forty-six patients pathologically diagnosed with a giant cell lesion involving the temporal bone between October 2001 and October 2020 were reviewed retrospectively. The clinical characteristics, surgical approaches, and risk factors for recurrence were analyzed. Results GCTs and GCGs presented as masses centered on the temporomandibular joint with similar imaging features, including a thin, calcified shell and central scattered calcifications on a computed tomography scan. Differences were detected on magnetic resonance imaging in 29.6% (4/14) of GCG and 50% (16/32) of GCT cases; the remaining cases were not distinguishable. Based on our staging system and surgical strategy, 31.8% (7/22) of GCT and 10% (1/10) of GCG cases experienced recurrence, which compares to recurrence rates of 60% in GCT cases and 20% in GCG cases in previous studies. Conclusions Specific clinical and preoperative imaging features help to make a diagnosis of temporal giant cell-rich lesions. Our staging system and surgical strategy could help surgeons tailor the surgical strategy.

Personal space regulation is affected by unilateral temporal lesions beyond the amygdala.

We constantly face situations involving interactions with others that require us to automatically adjust our physical distances to avoid discomfort or anxiety. A previous case study has demonstrated that the integrity of both amygdalae is essential to regulate interpersonal distances. Despite unilateral lesion to the amygdala, as to other sectors of the medial temporal cortex, are known to also affect social behavior, their role in the regulation of interpersonal distances has never been investigated. Here, we sought to fill this gap by testing three patients with unilateral temporal lesions following surgical resections, including one patient with a lesion mainly centered on the amygdala and two with lesions to adjacent medial temporal cortex, on two versions of the stop distance paradigm (i.e. in a virtual reality environment and in a real setting). Our results showed that all three patients set shorter interpersonal distances compared to neurotypical controls. In addition, compared to controls, none of the patients adjusted such physical distances depending on facial emotional expressions, despite they preserved ability to categorize them. Finally, patients' heart rate responses differed from controls when viewing approaching faces. Our findings bring compelling evidence that unilateral lesions within the medial temporal cortex, not necessarily restricted to the amygdala, are sufficient to alter interpersonal distance, thus shedding new light on the neural circuitry regulating distance in social interactions.

Utility of multimodal imaging in the detection and follow-up of retinal astrocytic hamartomas in a young girl with tuberous sclerosis complex.

Retinal astrocytic hamartomas (RAH) are one of the major diagnostic criteria for tuberous sclerosis complex (TSC) and is conventionally detected by ophthalmoscopy. We describe the utility of multimodal imaging (MMI) with spectral domain optical coherence tomography (SDOCT) and multicolor imaging (MCI) in the detection of hamartomas which may be missed clinically, especially in pediatric patients who may not be able to cooperate for detailed fundus examination. We also present some novel imaging findings which helped to understand the evolution of these lesions. A 5-year-old girl with tuberous sclerosis complex (TSC) was subjected to retinal evaluation and MMI. Fundus examination showed subtle elevation superotemporal to the disc in the right eye (RE) and a circular greyish lesion overlying superotemporal arcade in the left eye (LE) [Fig. 1a, b]. Corresponding OCT showed dome-shaped elevation of the nerve fiber layer (NFL), confirming the lesions as RAH [Fig. 1c, d]. MCI with its component blue and green reflectance (BR, GR) well delineated these superficial lesions with uniform hyporeflectivity. MCI supported by OCT revealed additional lesions in both eyes which were not visible clinically. Right eye was found to have a well-defined superficial lesion along the inferotemporal arcade with minimal elevation of the NFL and intact outer retina on OCT [Fig. 2a]. LE had two small deep lesions, one superiorly and another temporally, with minimal or no elevation of the NFL, outer retinal collapse and ellipsoid zone loss on OCT [Fig. 2b, c]. On BR and GR, superficial lesion in the RE had hyporeflectance [Fig. 3a, b], whereas the deep lesions in the LE were hyperreflective with central hyporeflectance [Fig. 4a]. At review six months later, the temporal lesion in the LE showed an additional hyporeflective outer rim [Fig. 4b]. OCT was unchanged at follow-up.Figure 1: Colour fundus photo (CP) and optical coherence tomography (OCT) of both eyes: CP (a and b) showed retinal astrocytic hamartomas superior to the disc in the right eye (RE) and over the superotemporal arcade in the left eye (LE). OCT through the lesions showed dome-shaped elevation of the nerve fiber layer with back-shadowing in RE (c) and a hyporeflective cavitation in LE (d)Figure 2: Multicolor imaging (MCI) with (OCT) of both eyes: MCI demonstrated additional lesions in both eyes (arrow). On OCT, superficial lesion in the RE (a) showed elevation of the nerve fiber layer with intact outer retina, whereas deeper lesions in the LE (b and c) showed predominant changes in the outer retina with outer retinal collapse and ellipsoid zone loss (arrowheads)Figure 3: Blue (a) and green (b) reflectance (BR, GR) of the right eye: A well-delineated elevated lesion with uniform hyporeflectivity was noted over the temporal vessels (arrow). The confocal high-resolution BR and GR imaging with superior contrast enabled improved detection of the inner retinal lesion which was missed on ophthalmoscopy and colour fundus photographyFigure 4: Blue and green reflectance (BR and GR) of the left eye: At presentation, BR and GR of the deep lesions showed hyperreflectivity with central hyporeflectance (a, white squares). At six months follow-up, the superior lesion remained stable while the temporal lesion showed an additional hyporeflective outer rim in a characteristic Bull’s eye configuration (b, red squares)Discussion OCT features of RAH are well described and improves the detection of these lesions.[1234] A majority of RAH remain stable, but can rarely regress spontaneously.[5] MCI with its component BR and GR is an additional tool to detect and monitor these lesions. We report novel findings on MMI in RAH and correlate lesion characteristics with the evolution of the lesion. We assumed superficial RAH with elevation of NFL on OCT to be active lesions. The deep RAH with predominant changes in the outer retina and progressive changes on BR and GR may be regressing lesions and may require MMI for detection. While BR and GR showed progressive changes on follow-up, OCT features remained stable, thereby indicating that MCI may be more sensitive to monitor the course of RAH in TSC. Although RAH indicates tumor-like growth in inner retinal layers which usually remain constant, the use of BR and GR with OCT could aid in diagnosis and follow-up of RAH. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

64-Year-Old Woman With Aphasia and Troponin Elevation

64-Year-Old Woman With Aphasia and Troponin Elevation