Summary Multiple sclerosis (MS) is a progressive disease of the CNS that is characterised by widespread lesions in the brain and spinal cord. MS results in motor, cognitive, and neuropsychiatric symptoms, all of which can occur independently of one another. The common cognitive symptoms include deficits in complex attention, efficiency of information processing, executive functioning, processing speed, and long-term memory. These deficits detrimentally affect many aspects of daily life, such as the ability to run a household, participate fully in society, and maintain employment—factors that can all affect the overall quality of life of the patient. The increased use of neuroimaging techniques in patients with MS has advanced our understanding of structural and functional changes in the brain that are characteristic of this disease, although much remains to be learned. Moreover, examination of efforts to treat the cognitive deficits in MS is still in the early stages.

https://www.thelancet.com/pdfs/journals/laneur/PIIS1474-4422(08)70259-X.pdf

Cognitive impairment in multiple sclerosis

Evidence for topographic organization in the cerebellum of motor control versus cognitive and affective processing.

The cerebellum and cognition.

Wernicke's encephalopathy is an acute neuropsychiatric syndrome resulting from thiamine deficiency, which is associated with significant morbidity and mortality. According to autopsy-based studies, the disorder is still greatly underdiagnosed in both adults and children. In this review, we provide an update on the factors and clinical settings that predispose to Wernicke's encephalopathy, and discuss the most recent insights into epidemiology, pathophysiology, genetics, diagnosis, and treatment. To facilitate the diagnosis, we classify the common and rare symptoms at presentation and the late-stage symptoms. We emphasise the optimum dose of parenteral thiamine required for prophylaxis and treatment of Wernicke's encephalopathy and prevention of Korsakoff's syndrome associated with alcohol misuse. A systematic approach helps to ensure that patients receive a prompt diagnosis and adequate treatment.

http://unmhospitalist.pbworks.com/f/Wernickes+encephalopathy.pdf

Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management

A central aspect of the cerebellar cognitive affective syndrome is the dysregulation of affect that occurs when lesions involve the ‘limbic cerebellum’ (vermis and fastigial nucleus). In this case series we describe neuropsychiatric disturbances in adults and children with congenital lesions including cerebellar agenesis, dysplasia, and hypoplasia, and acquired conditions including cerebellar stroke, tumor, cerebellitis, trauma, and neurodegenerative disorders. The behaviors that we witnessed and that were described by patients and families included distractibility and hyperactivity, impulsiveness, disinhibition, anxiety, ritualistic and stereotypical behaviors, illogical thought and lack of empathy, as well as aggression and irritability. Ruminative and obsessive behaviors, dysphoria and depression, tactile defensiveness and sensory overload, apathy, childlike behavior, and inability to appreciate social boundaries and assign ulterior motives were also evident. We grouped these disparate neurobehavioral profiles into five major domains, characterized broadly as disorders of attentional control, emotional control, and social skill set as well as autism spectrum disorders, and psychosis spectrum disorders. Drawing on our dysmetria of thought hypothesis, we conceptualized the symptom complexes within each putative domain as reflecting either exaggeration (overshoot, hypermetria) or diminution (hypotonia, or hypometria) of responses to the internal or external environment. Some patients fluctuated between these two states. We consider the implications of these neurobehavioral observations for the care of patients with ataxia, discuss the broader role of the cerebellum in the pathogenesis of these neuropsychiatric symptoms, and revisit the possibility of using cerebellar stimulation to treat psychiatric disorders by enhancing cerebellar modulation of cognition and emotion.

The neuropsychiatry of the cerebellum - insights from the clinic.

Objective: (1) To assess cognitive function and cerebral magnetic resonance imaging (MRI) involvement in relapsing-remitting multiple sclerosis; (2) to monitor disease evolution, cognitive dysfunction, and cerebral lesion burden over time (mean 8.5 year follow up period); (3) to study the relation between clinical, neuropsychological, and MRI data. On follow up assessment, visual and auditory oddball event related potentials (ERPs) were recorded as psychophysiological evaluation of cognitive status. Correlations between neuropsychological, MRI, and ERP data were also analysed. Methods: Neuropsychological study assessed verbal and non-verbal IQ, deterioration index (DI) from WAIS subtests, conceptual reasoning, attention, verbal and visuospatial short-term and long term memory. MRI assessment detected presence of demyelinating lesions by using a semiquantitative method as well as cortical and subcortical atrophy over time. Results: Attention, short-term and long term visuospatial memory were mildly impaired at baseline and remained unaltered longitudinally. At retesting a significant worsening of verbal long term memory (p=0.023), DI presence (p=0.041) and the increase of supratentorial and subtentorial MRI lesions load (p=0.001) emerged. Expanded disability status scale score correlated significantly with total lesion burden at both evaluations (p=0.043 and p=0.024 respectively). Temporal, occipital, and frontal horn lesions as well as cortical atrophy correlated significantly with attention and memory tests at baseline. Follow up assessment revealed significant correlation between cortical atrophy and attention as well as visuospatial short-term memory; spatial long term memory correlated significantly with lesions in body of lateral ventricle and frontal lobe. ERP study showed P300 latency abnormalities in 75% of patients, involving specifically more visual P300 (58.4 % of cases) than auditory wave (41.6 %). Visual P300 latency and amplitude correlated significantly with DI and auditory P300 latency with frontal horn and brain stem lesions. Conclusions: These findings revealed mild cognitive impairment in MS patients particularly consistent with slowing information processing over time. Increased MRI lesions do not correlate with the clinical course of the disease and cognitive deficit evolution. Thus, cognitive dysfunction could be related to disease peculiarity and not to the time course. Correlations between P300, neuropsychological, and MRI findings provide further information about ERP application to examine cognitive impairment in MS and probably to investigate their neural origin.

https://jnnp.bmj.com/content/jnnp/74/7/878.full.pdf

Longitudinal study of cognitive dysfunction in multiple sclerosis: neuropsychological, neuroradiological, and neurophysiological findings

Cerebellar pathology commonly shows important
motor signs and less evident cognitive dysfunction. The
’cerebellar cognitive affective syndrome’ is characterised by
impairment on executive function, spatial cognition, language
and behaviour. We report the case of a man with acute
onset of transitory motor features and severe mental disorders.
Cranial CT and brain MRI revealed extended cerebellar
lesions. Neuropsychological assessment disclosed
deficits of attention, executive function and memory.
Auditory event–related potentials showed abnormal P300.
These data suggest a pure “cerebellar cognitive affective syndrome”
and strengthen the hypothesis of cerebellar cognitive
function modulation.

Pure post-stroke cerebellar cognitive affective syndrome: a case report

Study Design. Three cases of idiopathic epidural lipomatosis are reported.

Objectives. Description of the relationship between spinal pathologic overgrowth of fat tissue and neurologic symptoms.

Summary of Background Data. Idiopathic epidural lipomatosis is a very rare condition; it is usually secondary to chronic steroid therapy or endocrinopathic diseases.

Methods. Three men with a mean age of 58.5 years, who experienced intermittent claudication, bilateral radicular pain in both legs, and urinary dysfunction with hypoesthesia in the perineal region, were evaluated by plain radiography and magnetic resonance imaging, the results of which demonstrated a pathologic overgrowth of fat tissue in the spinal canal with a marked impingement of the dural sac. Obesity, endocrinopathic diseases, and chronic steroid therapy were excluded for all patients. Surgical treatment was performed by wide multilevel laminectomies, fat debulking, and instrumented posterolateral fusion.

Results. After surgery there was a gradual improvement in symptoms and signs so that 2 years later the patients returned to daily activities and were neurologically normal.

Conclusions. Spinal epidural lipomatosis can be a cause of back pain but rarely radicular impingement. Magnetic resonance imaging is the procedure of choice. The treatment must be performed early by wide surgical decompression.

Cauda equina syndrome secondary to idiopathic spinal epidural lipomatosis.

Introduction. Over the last decade, the field of medical imaging experienced an exponential growth, leading to the development of radiomics, with which innumerable quantitative features are obtained from digital medical images, providing a comprehensive characterization of the tumor. This review aims to assess the role of this emerging diagnostic tool in breast cancer, focusing on the ability of radiomics to predict malignancy, response to neoadjuvant chemotherapy, prognostic factors, molecular subtypes, and risk of recurrence. Evidence Acquisition. A literature search on PubMed and on Cochrane database websites to retrieve English-written systematic reviews, review articles, meta-analyses, and randomized clinical trials published from August 2013 up to July 2018 was carried out. Results. Twenty papers (19 retrospective and 1 prospective studies) conducted with different conventional imaging modalities were included. Discussion. The integration of quantitative information with clinical, histological, and genomic data could enable clinicians to provide personalized treatments for breast cancer patients. Current limitations of a routinely application of radiomics are represented by the limited knowledge of its basics concepts among radiologists and by the lack of efficient and standardized systems of feature extraction and data sharing.

/pdf/a-new-challenge-for-radiologists-radiomics-in-breast-cancer-qo1hljfizj.pdf

Maurizio Conti

Papers

Longitudinal study of cognitive dysfunction in multiple sclerosis: neuropsychological, neuroradiological, and neurophysiological findings

Pure post-stroke cerebellar cognitive affective syndrome: a case report

Cauda equina syndrome secondary to idiopathic spinal epidural lipomatosis.

A New Challenge for Radiologists: Radiomics in Breast Cancer

Restless legs syndrome and periodic limb movements after ischemic stroke in the right lenticulostriate region