Nondegenerative Dementias and Encephalopathies

Nondegenerative dementias are a diverse but important group of cognitive disorders because they may be reversible with treatment. Thus, evaluation is important when a nondegenerative dementia is suspected. Many causes of nondegenerative dementia result in what is known as subcortical dementia, which is thought to be primarily due to damage to the frontal subcortical connections. Typical clinical features include inattention, bradyphrenia (slowed thought process), executive dysfunction (difficulties planning and sequencing tasks), apathy, psychomotor slowing, and mood disorders. Gait apraxia and urinary difficulties may coexist. Cortical features such as agnosia, seizures, aphasia, and ideomotor apraxia are typically absent.

Vertebrobasilar Dolichoectasia, Hypoplastic Third Ventricle, and Related Biventricular Hydrocephalus: Case Report and Review of the Literature

Background Vertebrobasilar dolichoectasia (VBDE) is defined as a symptomatic dilatation and tortuosity of the vertebrobasilar arteries. The risk of hydrocephalus development is due to direct compression of the third ventricle outflow or brainstem compression and related aqueduct stenosis. We present an uncommon case of a patient with symptomatic VBDE with the uniqueness of a hypoplastic third ventricle associated with biventricular hydrocephalus. A literature review concerning diagnosis and management of patients affected by biventricular hydrocephalus caused by VBDE was also performed. Case Illustration We report a case of a 54-year-old man who presented with headache, ideomotor apraxia, and gait disorder. A head computed tomography (CT) scan showed a biventricular hydrocephalus and a subsequent CT angiography documented the presence of a VBDE compressing the anterior part of the third ventricle that also appeared hypoplastic. The patient also presented a clinical history of arterial hypertension for which he was given a proper pharmacologic treatment with symptom relief. A surgical treatment of ventriculoperitoneal shunt along with endoscopic septostomy was proposed, but the patient refused, probably due to the slightly positive response to medical treatment. Conclusions The natural clinical history of patients affected by VBDE is unfavorable with 7.8 years of median survival. The therapeutic strategy is usually conservative and the role of antiplatelets or oral anticoagulants is still debated. In selected patients, ventriculoperitoneal shunt to resolve intracranial hypertension caused by biventricular hydrocephalus is the most effective treatment. In our opinion, chronic third ventricle compression could lead to anatomic–pathologic alterations like the third ventricle hypoplasia documented in our report.

CORTICOBASAL SYNDROME DUE TO ALZHEIMER’S DISEASE

Background: Corticobasal Syndrome (CBS) is a neurodegenerative syndrome that combines cortical and cognitive deficits secondary to different underlying pathological entities. Objectives: to report an early onset dementia case fulfilling criteria of probable CBS due to Alzheimer’s Disease (AD) based on biomarkers and neuroimaging. Methods: case report. Results: a 57-yearsold woman with college-level education and 18 months of cognitive decline. The first symptom was progressive inability to change gears in her car, followed by difficulties to get dressed, cognitive and motor complaints. Neurological examination revealed marked limb bilateral ideomotor apraxia and mild asymetric parkinsonism. Cognitive tests showed mild visuospatial and language impairments, scoring 18/30 in the MoCA. Brain MRI and FDG PET showed bilateral posterior atrophy and hypometabolism worse to the left. CSF biomarkers revealed decreased amyloid and increased tau and p-tau levels, a pattern suggestive of CBS due to AD. Conclusions: this case illustrates recent evidence that suggests when AD presents as CBS (CBS-AD), limb apraxia and language impairment are more prevalent. CBS patients with underlying AD pathology and tauopathies correctly diagnosed in the future may benefit from symptomatic therapies and future disease-modifying agents.

Thyroid Hormones and Antibodies: An Economic Risk Factor to Remember

BackgroundThyroid metabolites play an important role in cognitive functions. It is absolutely recommended to evaluate thyroid function tests (TFT) in the etiologic examination of cognitive disorders in dementia. In this study, we aimed to compare laboratory and cognitive data of patients aged under 65 years who presented to the other clinics with memory loss and who had been diagnosed with Alzheimer disease (AD).MethodsWe retrospectively reviewed the findings of patients aged under 65 years meeting the inclusion criteria of the study. Before admitting to our clinic, the patients who had memory problems were diagnosed with AD without screening TFT and started medications in different centers were screened. After performing thyroid screening for differential diagnosis in our first evaluation, patients who had diagnosed with disorders of thyroid hormones or/and antibodies and started to treat with thyroid medical therapy, included in our study.There were three groups. GroupI patients had elevated values of fT3 and anti-TPO, normal anti-TG levels. GroupII patients had decreased fT3 and fT4 levels and normal values of antibodies. GroupIII patients had normal fT3-fT4 and elevated values of anti-TPO and anti-TG. The patients of medications for AD (donepezil/rivastigmine/memantine/irregular medications) were recorded. The patients were followed-up for at 1st and 24th weeks after starting thyroid medical treatment. Follow-up of the patients was performed by the standardized mini mental state examination (MMSE), Verbal memory process, Boston naming test (BNT), Frontal assessment battery (FAB), Barthel activities of daily life index (BI) and Ideomotor apraxia test (IAT).ResultsWe identified 55 (30 female-25 male) patients who could be included. The mean age of patients was 51.8±7.6 (35-65). There were 40 (72.7%) patients in GroupI, 11 (20.2%) in GroupII, and 4 (7.1%) in GroupIII.MMSE, BNT, FAB (similarities go-no-go and lexical fluency), IAT were found statistically significant differences in all groups. Cognitive tests were found to be improved in patients. Although BI was found significant improvement in GroupII at 24th week. ConclusionIf there is a disorder of thyroid hormones and antibodies in patients with memory loss, the treatment of thyroid dysfunction should be planned before onset of anti-dementia medications.