1108 Comparing the Accuracy of Ultrasonography and Nuclear Imaging Scan in Preoperative Localisation of Parathyroid Lesions in Patients with Primary Hyperparathyroidism

Aim Parathyroidectomy remains the only method to cure Primary Hyperparathyroidism. Preoperative localisation of the lesion is vital for successful surgical management. The preferred initial preoperative imaging is an ultrasound scan (USS) of the neck. NICE recommends a second preoperative imaging modality to guide the surgical management. This study was conducted to compare the effectiveness of USS of the neck and SPECT scan in the preoperative localisation of parathyroid lesions in a single Teaching Hospital. Method A retrospective study performed included a cohort of patients between 2018 and 2020. 31 patients that underwent elective focussed parathyroidectomy were followed up. Data on preoperative investigations including USS of the neck and SPECT and final histological diagnosis of the specimen was captured using the hospital’s electronic medical records Quadramed. Results Both USS and SPECT scan correctly identified the nature and location of the lesion in only 35.4% patients. USS alone correctly identified the nature and location of the lesion in 50.0% patients whereas SPECT was 46.7%. USS incorrectly identified the nature or the location of the lesion in 33.3% patients whereas SPECT was 40.0%. Interestingly, the USS did not identify any abnormal pathology in 5 patients and SPECT in 4 patients, although all 9 showed pathological histology findings. Conclusions Data collected shows that either scan alone would not be sufficient to confirm the absence of parathyroid pathology. A second scan decreases the likelihood of missing any abnormal pathology. However, in patients with a high clinical suspicion abnormality cannot be ruled out despite having two negative scan results.

Naxitamab and GM-CSF for consolidation of high-risk neuroblastoma (HR-NB) patients in complete remission.

10021 Background: Naxitamab was recently approved in the US in combination with GM-CSF for the treatment of patients (pts) with relapse/refractory HR-NB in the bone/bone marrow who have demonstrated a partial response (PR), minor response (MR), or stable disease (SD) to prior therapy. Here we describe the use of 5 cycles of naxitamab and GM-CSF through compassionate use for consolidation of HR-NB pts in first or subsequent complete remission (CR). Methods: HR-NB pts in CR (first or subsequent) after initial multimodal induction treatment were eligible. Disease status was assessed at study entry by histology of BM biopsies/aspirates obtained from bilateral posterior and bilateral anterior iliac crests, I-MIBG SPECT scan, and whole body MRI. FDG-PET was used for MIBG non-avid cases at diagnosis. Quantitative reverse transcription-polymerase chain reaction was used to assess MRD in pooled heparinized BM aspirates. Disease response was defined according to the revised INRC. Four BM aspirates and 123I-MIBG SPECT scan or FDG-PET scans were performed after cycles 2 and 5 and every 3 months thereafter for one year in all pts to assess response. Naxitamab was administered over ≥30 min in the outpatient setting on Days 1, 3 and 5 at 3.0 mg/kg/infusion (9.0 mg/kg/cycle) in combination with GM-CSF at 250 ug/m2/day on Days -4 to 0 and at 500 ug/m2/day on days 1 to 5. Treatment cycles were repeated every 4 weeks. Survival curves were built from the time of naxitamab treatment initiation by Kaplan-Meier methods and compared using the log-rank test. Results: From June 2017 to November 30 2020, 73 pts were treated: 55 (75%) in first CR and 18 (25%) in second or more CR. Majority of pts were MYCN non-amplified (n=56, 77%), all stage 4, median age at treatment initiation 4.5 years. 61 (84%) pts had received >5 cycles of induction chemotherapy; 22 (30%) high-dose chemotherapy and autologous stem cell transplant (ASCT); and 36 (49%) radiotherapy before receiving naxitamab. 58 (79.5%) pts completed naxitamab therapy, 53 (73%) in continued CR. 10 (14%) pts relapsed during treatment and 5 (7%) had grade 4 toxicities: 2 apnea related to naxitamab; and 2 non-related: 1 opioid related chest rigidity syndrome and 1 stroke. 3-y event-free-survival (EFS) for all pts is 58% [95% CI, 43.5; 78.4], 74% for first CR and 19% for second or more CR (p=0.0029). 3-y OS for the whole group is 82% [95% CI, 66.8; 100.0], 92% for first CR and 66% for second or more CR pts (p=0.18). Univariate Cox models for CR group, MYCN status, number of chemotherapies, ASCT, radiotherapy, MRD, and age showed significant p value only for prior relapse as predictor of EFS (p=0.047). Conclusions: Naxitamab for HR-NB pts in CR provided excellent 3-y OS rates regardless of previous management. The only predictor for relapse is prior history of recurrence.

Image-Guided Right T10 Costotransverse Joint Resection for Costotransversitis: Operative Technique and Nuances: 2-Dimensional Operative Video

We describe the operative approach and management for costotransverse joint inflammation in a 49-yr-old man with worsening midthoracic pain radiating to the right paraspinal area. He underwent physical therapy with no relief of his symptoms. Thoracic spine magnetic resonance imaging (MRI) revealed severe arthritic changes involving the right T10 costotransverse joint. Scoliosis X-rays showed a dextroconvex curvature in the midthoracic spine, without any significant imbalance. Single-photon emission computed tomography (SPECT) scan revealed focal increased uptake of the right T10 costotransverse joint. T10 costotransverse joint lidocaine injection did not provide any relief. We performed a computed tomography (CT)-guided biopsy, which was negative for malignancy and also cultures were negative. MRI revealed a significant enhancement in this area and the patient's C-reactive Protein was elevated. Decision was made to perform open biopsy and costotransverse joint resection. We present a case of minimally invasive, image-guided costotransverse joint resection, which has not been described in the literature. The right T10 costotransverse joint was dissected out with the image-guided dilator, and tubular retractors were inserted. Under the microscope, using the image-guided drill, the right T10 costotransverse joint was drilled out. The lateral aspect of the right T10 process was drilled out as well as the medial-dorsal aspect of the right T10 rib. The patient recovered from surgery well with abatement of his preoperative thoracic pain, which remained abated at 6-mo follow-up. This case highlights the complex technical nuances of this procedure, and the importance of a thorough preoperative evaluation with a bone SPECT scan to help localize the pain generator. Patient consented for the procedures and for the publication of the video.

Case Report: Bupropion Reduces the [123I]FP-CIT Binding to Striatal Dopamine Transporter

The diagnosis of parkinsonian syndromes in patients with severe depression may be challenging due to overlapping clinical phenomena, especially regarding psychomotor and affective symptoms. [123I]FP-CIT-SPECT is a useful method to detect degenerative parkinsonian disorders. However, some drugs may influence the tracer binding and thus alter the result. We present a case of 56-year-old female inpatient with difficult-to-treat late-onset depression. Since the current major depressive episode (MDE) was accompanied by psychotic features including delusions and hallucinations as well as hypokinesia, stooped posture and hypomimia, underlying degenerative parkinsonism was suspected. The pathologic [123I]FP-CIT-SPECT scan under ongoing antidepressant therapy with bupropion 300 mg/die (serum level of bupropion 43 ng/ml and hydroxybupropion 2,332 ng/ml) showed reduced [123I]FP-CIT binding throughout the striatum. The scan normalized upon a wash-out phase of four half-time periods (serum level of bupropion was 0.4 ng/ml and for hydroxybupropion 80.5 ng/ml). Our report should serve as a cautionary note for use of [123I]FP-CIT in depressed patients, particularly in those treated with drugs interfering with the dopamine transporter. Furthermore, our case argues for a need of consultation of a movement disorder specialist prior to dopamine transporter imaging.

Intrinsic Alertness Is Impaired in Patients with Nigrostriatal Degeneration: A Prospective Study with Reference to [123I]FP-CIT SPECT and [18F]FDG PET

Background: Variations in alertness and attention are common in Lewy body diseases (LBD) and among the core features of dementia with Lewy bodies (DLB). Dopamine transporter SPECT is an accurate biomarker of nigrostriatal degeneration (NSD) in LBD. Objective: The present study investigated performance on a computerized alertness test as a potential measure of attention in patients with NSD compared to patients without NSD. Methods: Thirty-six patients with cognitive impairment plus at least one core feature of DLB referred for [123I]FP-CIT SPECT imaging were prospectively recruited. Performance in a computerized test of intrinsic alertness was compared between patients with and those without NSD as assessed by [123I]FP-CIT SPECT. Results: Reaction times to auditory stimuli (adjusted for age, sex, and education) were significantly longer in patients with NSD compared to those with a normal [123I]FP-CIT SPECT scan (p < 0.05). Statistical analyses revealed no significant differences comparing reaction times to visual stimuli or dispersion of reaction times between groups. Exploratory analysis in a subgroup of patients with available [18F]FDG PET revealed that longer reaction times were associated with decreased glucose metabolism in the prefrontal cortex (statistical parametric mapping, adjusted for age and sex; p < 0.005, cluster extent > 50 voxels). Conclusion: Computerized assessment of auditory reaction times is able to detect alertness deficits in patients with NSD and might help to measure alertness deficits in patients with LBD and NSD. Future studies in larger samples are needed to evaluate the diagnostic utility of computerized alertness assessment for the differential diagnosis of LBD.

PEMERIKSAAN PET DAN SPECT SCAN PADA BEDAH EPILEPSI

Epilepsi merupakan penyakit sistem saraf pusat yang ditandai dengan bangkitan berulang karena terganggunya aktivitas sel di otak. Sekitar 1-25 populasi didunia menderita epilepsi. Penderita epilepsi yang masih mengalami suatu bangkitan, meski sudah mendapatkan 2 jenis obat anti epilepsi, perlu dipertimbangkan untuk dilakukan suatu tindakan bedah epilepsi. Bedah epilepsi merupakan suatu tindakan invasif yang mereseksi fokal epilepsi, dengan harapan penderita bebas kejang. PET Scan merupakan salah satu pencitraan yang perlu dilakukan untuk mengevaluasi pasien sebelum dilakukan tindakan bedah epilepsi.

Hyposmia as a marker of (non-)motor disease severity in Parkinson’s disease

The aim of this study was to evaluate the relationship of hyposmia in Parkinson’s disease (PD) with other motor and non-motor symptoms and with the degree of nigrostriatal dopaminergic cell loss. A total of 295 patients with a diagnosis of PD were included. Olfactory function was measured using the University of Pennsylvania Smell Identification Test (UPSIT). Motor symptoms were rated using the Unified Parkinson’s Disease Rating Scale motor subscale (UPDRS III). To evaluate other non-motor symptoms, we used the Mini-Mental State Examination (MMSE) as a measure of global cognitive function and validated questionnaires to assess sleep disturbances, psychiatric symptoms, and autonomic dysfunction. A linear regression model was used to calculate correlation coefficients between UPSIT score and motor and non-motor variables [for psychiatric symptoms a Poisson regression was performed]. In a subgroup of patients (n = 155) with a dopamine transporter (DaT) SPECT scan, a similar statistical analysis was performed, now including striatal DaT binding. In the regression models with correction for age, sex, disease duration, and multiple testing, all motor and non-motor symptoms were associated with UPSIT scores. In the subgroup of patients with a DaT-SPECT scan, there was a strong association between olfactory test scores and DaT binding in both putamen and caudate nucleus. Hyposmia in PD is associated with various motor and non-motor symptoms, like cognition, depression, anxiety, autonomic dysfunction and sleep disturbances, and with the degree of nigrostriatal dopaminergic cell loss. This finding adds further confirmation that hyposmia holds significant promise as a marker of disease progression.

Functional Neurological Symptom Disorder: A Diagnostic Algorithm

Functional neurological symptom disorder (FNSD) is a neuropsychiatric disorder characterized by the presence of neurological symptoms in the absence of any neurological abnormality that can be linked to a known pathology. Few studies have taken interest in this subject probably because of the heterogeneity of results. It is most often a diagnosis of exclusion which often means that patients undergo many tests and find themselves erring for a diagnosis with very little satisfaction of the outcomes. A reliable imagery pattern would therefore provide some relief and confirmation for both patients and clinicians. It could also facilitate acceptation of the diagnosis and reduce the societal cost associated with FNSD for the patient. The aim of this present study was to describe a clinicoradiological correspondence algorithm of FNSD using the PET scan and SPECT scan (PoSPs) and grant the clinician with a reliable tool to facilitate the diagnosis of FNSD. A systematic review according to the 2009 PRISMA criteria statement was used to guide the review. Our study included 3 of our own consenting patients who met the Diagnostic and Statistical Manual of Mental Disorders 5th edition criteria as well as 25 other patients from 7 different studies. Our results showed a hypoactivation with poor clinicoradiological correspondence and poor stability in time. This hypoactivation was mostly in the frontal lobe, which could explain some behavioral alterations. These findings oppose the ones found in organic pathologies and therefore should orient towards FNSD. In the light of these findings, we recommend the clinicians to perform two PoSPs, searching for clinicoradiological lack of correspondence and time stability using our algorithm.

REM behavior disorder predicts motor progression and cognitive decline in Parkinson disease

ObjectiveTo investigate whether REM sleep behavior disorder (RBD) is associated with worse motor and cognitive decline in Parkinson disease (PD)MethodsFour-hundred twenty-one drug-naive patients with early-stage PD and 196 controls without PD were included in this study. All participants underwent a [123I]FP-CIT SPECT scan, CSF assessment, 3-tesla MRI, and thorough clinical assessments.ResultsAt cross-sectional analyses, patients with PD and probable RBD (PD-RBD) had lower CSF β-amyloid 1–42 (Aβ42) levels and higher total tau to Aβ42 CSF ratio, higher nonmotor symptoms burden, and worse scores on neuropsychological tests of processing speed, visuospatial functioning, and delayed recognition memory compared to patients with PD without RBD. At longitudinal analyses, the presence of RBD was associated with faster motor progression (hazard ratio [HR] = 1.368, 95% confidence Interval [CI] = 1.036–1.806; p = 0.027) and cognitive decline (HR = 1.794, 95% CI = 1.163–2.768; p = 0.008) over 60-month follow-up. The presence of RBD was a predictor for motor progression only in patients with PD who had both low α-synuclein levels and low [123I]FP-CIT uptake in the striatum (HR = 2.091, 95% CI = 1.116–3.918; p = 0.021) and a predictor for cognitive decline only in patients with PD who had both low Aβ42 and low α-synuclein levels (HR = 2.810, 95% CI = 1.462–5.400; p = 0.002). In the population of controls without PD, the presence of RBD was not associated with cognitive decline or any baseline pathologic changes.ConclusionThe presence of RBD in PD is associated with faster motor progression in patients with greater synuclein and dopaminergic pathology, and with higher risk of cognitive decline in patients with greater synuclein and amyloid pathology. Our findings provide an important direction toward understanding phenotypes and their prognosis in PD.