Patient with Corticobasal Syndrome Showing Disease-Associated Biomarkers of Dementia with Lewy Bodies: A Treviso Dementia (TREDEM) Registry Case Report.

Background: An 82-year-old right-handed man, a retired teacher, reported the occurrence, three years earlier, of difficulties in moving his left arm and foot, tremor in his left hand, and gestures of the left upper limb that appeared to be independent of the patient's will. Objective: We describe an unusual case of corticobasal syndrome (CBS) showing disease-associated biomarkers of dementia with Lewy bodies (DLB). Methods: Clinical, neuropsychological, imaging, and biomarker evaluations were conducted, including tau and amyloid-ß levels in the cerebrospinal fluid (CSF) and a RT-QuIC assay for α-synuclein both in the CSF and olfactory mucosa (OM), as well as a QEEG assessment. Results: The patient presented resting tremor, mild extrapyramidal hypertonus, mild bradykinesia on the left side, and severe apraxia on the left upper limb. Brain MRI showed a diffuse right hemisphere atrophy which was prominent in the posterior parietal and temporal cortices, and moderate in the frontal cortex and the precuneus area. 18F-FDG PET imaging showed reduced glucose metabolism in the right lateral parietal, temporal, and frontal cortices with involvement of the right precuneus. The putamen did not appear to be pathological at DaTQUANT. Neuropsychological tests showed memory and visual-perceptual deficits. CSF tau and amyloid measurements did not show clear pathological values. RT-QuIC for α-synuclein in CSF and OM samples were positive. The QEEG analysis showed a pre-alpha dominant frequency in posterior derivations, typical of early stages of DLB. Conclusion: Although in the present patient the clinical diagnosis was of probable CBS, unexpectedly positive biomarkers for DLB suggested the co-presence of multiple pathologies.

"Occipital Pole" Sign on 18 F-FDG PET for Dementia With Lewy Bodies and Posterior Cortical Atrophy : Evidence From the Treviso Dementia (TREDEM) Registry.

ABSTRACT: The 18 F-FDG PET images of dementia with Lewy bodies and posterior cortical atrophy, a visual-cognitive phenotype described in patients with Alzheimer disease, show occipital lobe hypometabolism with relative sparing of the primary visual cortex (PVC) generating the "occipital tunnel" sign proposed by Sawyer and Kuo in 2017, which is viewable on the medial sagittal projection. We believe that the saving of PVC compared with the lateral occipital cortex can be better appreciated by capturing the posterior projection of the PVC in a 3D stereotactic surface projection map, and we propose the name of "occipital pole" sign for this evidence.

More Atypical than Atypical Alzheimer's Disease Phenotypes: A Treviso Dementia (TREDEM) Registry Case Report.

BACKGROUND: A 57-year-old right-handed man was admitted to the Treviso Memory Clinic due to the presence of memory forgetfulness, repetition of the same questions, episodes of confusion, initial difficulties in performing complex tasks and easy distraction over the past two years, as well as recurrent and never-happened-before car accidents. OBJECTIVE: We report a peculiar case of an early onset Alzheimer's disease (AD) with an unusual symptomatology, apparently not fitting in any of the categorized atypical forms of AD nor being representative of a typical amnestic AD. METHODS: The patient underwent a neuropsychological, structural, and metabolic cerebral evaluation by MRI and 18F-FDG PET, together with the search for cerebral amyloid (amyloid PET), a genetic testing for dementia related genes and the dosage of CSF protein biomarkers of neurodegenerative conditions. RESULTS: We observed a convergence of predominant frontal (dysexecutive, verbal disinhibition) and posterior (visuospatial) features of cognitive impairment. Structural MRI sequences showed subarachnoid spaces of the vault enlarged in the fronto-parietal region with anterior and posterior cortical atrophy. The hippocampus appeared preserved. The 18F-FDG PET scans showed hypometabolism in the prefrontal, lateral temporal, posterior parietal, and occipital regions bilaterally. The 18F-Flutemetamol scan showed a diffused uptake of the amyloid tracer at the cerebral cortex. CSF biomarkers were compatible with Alzheimer's disease (AD). CONCLUSION: This case report presented with clinical phenotypic aspects atypical of AD, both frontal and posterior, never described as concomitant in the most accredited criteria for atypical AD, and appeared therefore more atypical than each of the atypical AD phenotypes already reported.

[CME/Answers: Musculoskeletal Manifestations of Lyme Disease]. / CME/Antworten: Borrelien-Manifestationen am Bewegungsapparat.

Lyme disease is the most common vector-borne illness in Switzerland and Lyme arthritis the most prevalent manifestation of late-stage Lyme disease. It presents as a monoarthitis or oligoarthritis in large joints, often involving the knee. Such a clinical presentation, together with positive Lyme serologies or polymerase chain reactions from synovial fluid/tissue, is considered diagnostic. If there is no tick bite or erythema migrans in the patient's history the diagnosis can be challenging due to the many differential diagnoses. The initial treatment is a prolonged course of oral antibiotics.

[CME: Musculoskeletal Manifestations of Lyme Disease]. / CME: Borrelien-Manifestationen am Bewegungsapparat.

CME: Musculoskeletal Manifestations of Lyme Disease Abstract. Lyme disease is the most common vector-borne illness in Switzerland and Lyme arthritis the most prevalent manifestation of late-stage Lyme disease. It presents as a monoarthitis or oligoarthritis in large joints, often involving the knee. Such a clinical presentation, together with positive Lyme serologies or polymerase chain reactions from synovial fluid/tissue, is considered diagnostic. If there is no tick bite or erythema migrans in the patient's history the diagnosis can be challenging due to the many differential diagnoses. The initial treatment is a prolonged course of oral antibiotics.

Anti-Cholinergic Derangement of Cortical Metabolism on 18F-FDG PET in a Patient with Frontotemporal Lobar Degeneration Dementia: A Case of the TREDEM Registry.

We present the case of a patient with an atypical course of frontotemporal lobar degeneration (FTLD) complicated by the use of an anticholinergic drug. A 70-year-old patient, followed by psychiatrists for depression and behavioral disorders, received a diagnosis of dementia with Lewy bodies (DLB) at another Center due to auditory hallucinations, gait impairment, and tendency to fall. He was then admitted to our Memory Clinic Unit for behavioral disturbances, such as delusional thinking, auditory hallucinations, and memory complaints. At that time, the patient's therapy included Lorazepam, Quetiapine, Promazine, and Biperiden. The latter was immediately suspended for the absence of extrapyramidal signs and to avoid the anticholinergic cognitive side effects. A 18F-FDG PET showed a derangement of cortical metabolism with diffusely reduced activity, and limited areas of hyperactivity involving lateral frontal and lateral temporal inferior regions bilaterally. The patient underwent a series of exams, including neuropsychological tests, 123I-MIBG scintigraphy, cerebrospinal fluid examination, and genetic analysis. A second 18F-FDG PET showed an extensive remodulation of metabolic activity: relative higher concentration of the tracer in the prefrontal and inferior temporal cortex was no more detectable. Similarly, the diffuse reduced metabolic activity could not be traced anymore. Nonetheless, the metabolic activity still appeared reduced in the frontal lobe, in the anterior cingulate bilaterally, and in the anterior part of the hemispheric fissure. Taken together, clinical and neuroimaging features would point to a FTLD-like form. Furthermore, the diagnostic work-up was likely confounded by the anticholinergic drug on 18F-FDG PET, highlighting the importance of carefully checking the patient's pharmacology during the diagnostic process.

A Case with Early Onset Alzheimer's Disease, Frontotemporal Hypometabolism, ApoE Genotype ɛ4/ɛ4 and C9ORF72 Intermediate Expansion: A Treviso Dementia (TREDEM) Registry Case Report.

We report the case of a woman firstly referred to our Memory Clinic at the age of 61, following the development of cognitive complaints and difficulties in sustained attention. The investigation that was performed showed: predominant executive dysfunctions at the neuropsychological evaluation, with mild, partial and stable involvement of the memory domain; cortical and subcortical atrophy with well-preserved hippocampal structures at MRI; marked fronto-temporal and moderate parietal hypometabolism from 18F-FDG PET study with a sparing of the posterior cingulate and precuneus; positivity of amyloid-ß at 18F-Flutemetamol PET; an hexanucleotide intermediate repeats expansion of C9ORF72 gene (12//38 repeats) and ApoE genotype ɛ4/ɛ4. The patient was diagnosed with probable early onset frontal variant of Alzheimer's disease (AD), presenting with a major executive function impairment. The lack of specific areas of brain atrophy, as well as the failure to meet the clinical criteria for any frontotemporal dementia, drove us to perform the aforementioned investigations, which yielded our final diagnosis. The present case highlights the need to take into consideration a diagnosis of frontal variant of AD when the metabolic and the clinical picture are somehow dissonant.

Clinical usefulness of 18-fluorodeoxyglucose positron emission tomography in the management of patients with nonpancreatic periampullary neoplasms.

BACKGROUND: 18-Fluorodeoxyglucose positron emission tomography (18-FDG PET) has been investigated for the diagnosis and staging of gastrointestinal malignancies including pancreatic adenocarcinoma. The aim of this study was to examine the clinical usefulness of 18-FDG PET in the diagnosis and follow-up evaluation of patients with periampullary neoplasms. METHODS: Twenty-five patients underwent whole-body 18-FDG PET and abdominal computed tomography (CT). Pathologic confirmation was obtained in all patients by surgical resection or biopsy examination. The 18-FDG PET was analyzed visually and semiquantitatively using the standard uptake value (SUV). Positivity was assumed when a focal uptake occurred with an SUV of 2.5 or greater. RESULTS: Between January 1998 and December 2003, 14 ampullary, 7 bile duct, and 4 duodenal tumors were included in the study. PET showed increased focal uptake in 22 patients (88%): 11 of 14 (79%) ampullary tumors, and 100% of bile duct and duodenal tumors. PET showed a focal uptake in 11 of 12 patients without detectable mass at CT scan, and lymph node metastases in 6 patients. An SUV value of 2.7 discriminated adenomas or noninvasive cancers (n = 6) from invasive malignancies (n = 14). Follow-up evaluation including CT scan and PET was performed in 12 patients: PET showed recurrent disease not seen by CT in 4 patients, confirmed CT findings in 6 patients, and showed an unsuspected primary lung cancer in 1 patient and colon cancer in another patient. CONCLUSIONS: 18-FDG PET is very sensitive for detecting periampullary neoplasms. It may be useful to differentiate benign or borderline lesions from invasive tumors when no mass has been identified by traditional imaging. Finally, it is very useful in the follow-up evaluation of resected patients to identify recurrent disease or other malignancies.

Tc-99m exametazime-labeled leukocyte scans in the study of infections in skull neurosurgery.

Diagnosis and follow-up of skull infections are usually performed by neurologic examination, laboratory tests and instrumental diagnostic methods such as computed tomography (CT) and magnetic resonance imaging (MRI). These have, however, shown some limitations for specificity. The aim of the current study was to evaluate the overall contribution of Tc-99m exametazime-labeled leukocyte imaging scan Tc-99m hexamethylpropyleneamine (HMPAO) labeled white blood cells (WBC) in the diagnosis and management of infections in skull neurosurgery. Thirty-four patients were subdivided into 4 groups on the basis of the suspected pathology: intracerebral lesions on CT or MRI (group A, n = 20), suspected postsurgical infections (group B, n = 6), suspected deep infection of the surgical wound (group C, n = 4), and suspected infection of the ventriculoperitoneal shunt (group D, n = 4). All patients underwent CT, MRI, and Tc-99m HMPAO WBC imaging. Patients in group C also underwent bacteriologic and culture examinations of wound secretions if present. In positive cases in group A, Tc-99m HMPAO WBC imaging was repeated. The scintigraphic results were compared with histologic findings in patients who underwent surgery and with the results of a 12-month clinical follow-up in the remaining patients. Tc-99m HMPAO WBC scans correctly detected the infections in all groups. Furthermore, such imaging proved to be able to document recovery from the disease in all of the assessed cerebral abscesses. This study may have an important role both in the diagnosis and in the management of infections in skull neurosurgery, which, it is hoped, will be confirmed in the future.