Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder belonging to the group of transmi... more Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder belonging to the group of transmissible spongiform encephalopathies. The transition of physiological, soluble, neuroprotective prion protein PrPc into its insoluble, misfolded isoform PrPSc remains its central pathogenic event. The progressive accumulation of isoform PrPSc within the brain tissue results in spongiform degeneration and a plethora of clinical symptoms. Typically, CJD manifests as progressive dementia with myoclonus, visual or cerebellar dysfunction, pyramidal/ extrapyramidal signs or akinetic mutism. However, a growing number of studies indicate that CJD may present with prodromal psychiatric manifestations including anhedonia, anxiety, irritability, depression, insomnia, psy-chosis and catatonic behavior. We present a case of CJD with a history of prodromal psychiatric symptoms and catatonic behavior diagnosed by brain biopsy. Creutzfeld-Jakob disease/brain biopsy/psychiatric symptoms
The microbiome co-evolved with its human host over a long time and became essential for many proc... more The microbiome co-evolved with its human host over a long time and became essential for many processes. Bacteria play a role in maintaining human health as they digest food, produce vitamins and participate in the regulation of metabolism. By influencing the cytokine balance along with the composition and activity of leu-kocytes, they constantly interact with the immune system, affecting innate and adaptive immune homeostasis. A growing number of studies indicate that the microbiome in the human intestine may have an impact on the functions of the central nervous system (CNS), through identified pathways called the gut–brain axis. Recent data show that the human microbiome ecosystem interferes with the brain's development, central signaling systems and behavior. It has been proposed that disruption in the human microbiome may affect the course of psychiatric disorders. The aim of this review is to summarize the recognized pathways of the gut–brain axis that have been thoroughly studied in animal models and to evaluate the role of the dialogue between the mi-crobiota and the central nervous system in schizophrenia, bipolar disorder and depression.
A b s t r a c t Angiocentric glioma (AG) is a newly-classified, very rare, WHO grade I central ne... more A b s t r a c t Angiocentric glioma (AG) is a newly-classified, very rare, WHO grade I central nervous system (CNS) lesion, occurring usually in children and young adults. Only 52 patients with AG have been reported so far, making it one of the rarest neuropathological entities. Hereby we present two new cases of AG in young subjects with detailed neuropatholog-ical investigations and a neuroradiological picture along with a brief summary of all already published literature reports of this tumor. Histopathological examination of the resected tissue from both cases revealed similar changes characteristic of AG. The tumors were composed of spindle-like, elongated cells, forming characteristic pseudorosettes around vessels and diffusively infiltrating surrounding tissue, trapping neurons between tumor cells. Noticeably, some neoplastic cells encrusting vessels extended far beyond the main tumor mass. Hypothetically, this may be responsible for the recurrence of the tumor even in the case of apparently total excision. In immunohistochemistry, AG cells were glial fibril-lary acidic protein (GFAP) and vimentin positive, also exhibiting a strikingly significant epithelial membrane antigen (EMA) dot-like staining pattern. In one of the cases, electron microscopy revealed ependymal differentiation features such as microvilli and cilia. Taken together, all these data strongly confirm a dual astroglial-ependymal nature of the tumor. Follow up corroborates benign character of this neoplasm. Both AGs reported here were immunonegative for the product of the mutated IDH-1 gene what, according to our best knowledge, has never been reported so far. It may suggest that in their pathogenesis AGs differ from grade II astrocytomas, which in most cases harbor a mutation of IDH-1. Noteworthy, neuroimaging in our cases was relatively characteristic but not conclusive, therefore biopsy (at least) is mandatory. A newly proposed so called " A-B-C " classification of long-term epilepsy-associated tumors (LEATs) places AG in a category named ANET. The authors shortly review the A-B-C classification of LEATs.
Bergmann glia (BG), a specific type of radial astro-cytes in the cerebellum, play a variety of vi... more Bergmann glia (BG), a specific type of radial astro-cytes in the cerebellum, play a variety of vital functions in the development of this structure. However, the possible role of BG in the development of abnormalities observed in individuals with autism spectrum disorder (ASD) seems to be underestimated. One of the most consistent findings observed in ASD patients is loss of Purkinje cells (PCs). Such a defect may be caused by dysregulation of glutamate homeostasis, which is maintained mainly by BG. Moreover, these glial cells are involved in long-term depression (LTD), a form of plasticity which can additionally subserve neuroprotective functions. The aim of presented review is to summarize the current knowledge about interactions which occur between PC and BG, with special emphasis on those which are relevant to the survival and proper functioning of cerebellar neurons.
Procedural learning involves the acquisition of new skills or knowledge on how to perform specifi... more Procedural learning involves the acquisition of new skills or knowledge on how to perform specific tasks. Evaluation of procedural learning is part of neuropsy- chological and neuropsychiatric diagnosis. Deficits observed in such diagnosis can be the basis for the inference of structural and functional brain disorders. The classic division of skills acquired during proce- dural learning includes four types: cognitive, percep- tual, motor and visuo-motor skills. Most of the tests examine cognitive and visuo-motor skills. This study focuses on the two latter types of skills that the acqui- sition of which is generally known as motor learning. In the natural conditions motor learning occurs both as implicit and explicit process, depending on its phase. However, paradigms used for diagnostic purposes as- sume that motor learning is an implicit process. The work provides an overview of the most popular tools used for the assessment of implicit motor learning as part of the neuropsycholog...
Lithium salts are the most commonly used drug in the treatment of bipolar disorder, with proven e... more Lithium salts are the most commonly used drug in the treatment of bipolar disorder, with proven effectiveness in re- ducing the risk of relapses. However, the narrow therapeutic range of concentrations and high risk of side effects require a closer look at their mechanisms of action. Lithium exhibits a neuroprotective effect in hippocampus and amygdala as well as the neurotoxic properties which are most evident in the cerebellum. The purpose of this paper is to review the literature on the neuroprotective and neurotoxic properties of lithium salts. Neuroprotective properties of prolonged lithium treatment result from the inhibition of neuronal excitotoxicity by blocking the NMDA receptors, and anti-apoptotic action in the particular areas of the brain. In vitro studies have also found a positive effect on neurogenesis, which coincides with the morphometric studies in humans, where increased volume of grey matter in the prefrontal cortex and the limbic system was reported. A lot of r...
The article provides an overview of the latest studies concerning the role of the cerebellum in c... more The article provides an overview of the latest studies concerning the role of the cerebellum in certain psychiatric diseases. We analyzed in detail the following disorders: schizophrenia, depression, bipolar disorder, autism and anxiety disorders with a main focus on PTSD. Thanks to its numerous connections with cortical and subcortical structures, the cerebellum participates in psychiatric disorders in various ways, both in the terms of structure: changes in cerebellar structures were noted in all analyzed diseases; and function: numerous neuroimaging data indicates a abnormal activation patterns in analyzed disorders. Still the results of the studies are hardly conclusive and the clear description of the relationship between the cerebellum and psychiatric disorders is at this moment impossible. More research is needed to provide unequivocal conclusions concerning this subject.
The study describes an abnormal convergence symptom, i.e. unilateral exophoria at near, in patien... more The study describes an abnormal convergence symptom, i.e. unilateral exophoria at near, in patients with schizophrenia (SZ) and bipolar disorder (BD). The aim of this paper is to present the symptom and discuss its possible explanations. 29 patients with SZ, 15 patients with BD and 20 healthy controls (HC) took part in the study. The neurological assessment was done with International Co-operative Ataxia Rating Scale (ICARS) and Neurological Evaluation Scale (NES). The abnormal vergence pattern was observed in 12 patients with SZ, 1 patient with BD and 0 HC. Symptom appeared statistically more often in SZ patients than in BD patients and HC. SZ patients with vergence symptom performed significantly worst in oculomotor and dysarthia subscores of ICARS. The symptom can be linked to disruptions in cortico-ponto-cerebellar network and midbrain. It was the only neurological symptom that differed SZ and BD groups, thus it might be used in differential diagnosis. Further research is needed to obtain a full clinical description of the symptom.
Emotional disorders in patients with cerebellar damage – case studies
"Aim. Growing number of research shows the role of the cerebellum in the regulation of affect. Le... more "Aim. Growing number of research shows the role of the cerebellum in the regulation of affect. Lesions of the cerebellum can lead to emotional disregulation, a significant part of the Cerebellar Cognitive Affective Syndrome. The aim of this article is to analyze the most recent studies concerning the cerebellar participation in emotional reactions and to present three cases: two female and one male who suffered from cerebellar damage and presented post-traumatic affective and personality change.
Method. The patients’ neuropsychological examination was performed with Raven’s Progressive Matrices Test – standard version, Trial Making Test, Wisconsin Card Sorting Test, Auditory Verbal Learning Test by Łuria, Benton Visual Retention Test, Verbal Fluency Test, Stroop Interference Test, Attention and Perceptivity Test (Test Uwagi i Spostrzegawczości TUS), Frontal Behavioral Inventory (FBI).
Results. The review of the literature suggest cerebellar participation, especially teh vermis and paravermial regions, in the detection, integration and filtration of emotional information and in regulation of autonomic emotional responses. In the described patients we observed: oversensitivity, irritability, impulsivity and self-neglect. The man and the woman with right-sided lesions presented similar symptoms: rigidity of thought, stubbornness, lack of criticism, jocular and inappropriate behavior. The woman with left-sided cerebellar lesion was adynamic, apathic and passive, she presented emotional blunting, social isolation, lack of interests and motivation, general cognitive slowdown.
Conclusions. Both the analyzed research and the described cases indicate the connection between the cerebellum and emotion regulation. The symptoms presented by the described patients were most probably a consequence of damaged cerebellar projections to subcortical structures (the limbic system) and frontal areas. The diversification of symptoms depending on the localization of lesions had not been described yet and seems to indicate an interesting direction for future research."
Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder belonging to the group of transmi... more Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder belonging to the group of transmissible spongiform encephalopathies. The transition of physiological, soluble, neuroprotective prion protein PrPc into its insoluble, misfolded isoform PrPSc remains its central pathogenic event. The progressive accumulation of isoform PrPSc within the brain tissue results in spongiform degeneration and a plethora of clinical symptoms. Typically, CJD manifests as progressive dementia with myoclonus, visual or cerebellar dysfunction, pyramidal/ extrapyramidal signs or akinetic mutism. However, a growing number of studies indicate that CJD may present with prodromal psychiatric manifestations including anhedonia, anxiety, irritability, depression, insomnia, psy-chosis and catatonic behavior. We present a case of CJD with a history of prodromal psychiatric symptoms and catatonic behavior diagnosed by brain biopsy. Creutzfeld-Jakob disease/brain biopsy/psychiatric symptoms
The microbiome co-evolved with its human host over a long time and became essential for many proc... more The microbiome co-evolved with its human host over a long time and became essential for many processes. Bacteria play a role in maintaining human health as they digest food, produce vitamins and participate in the regulation of metabolism. By influencing the cytokine balance along with the composition and activity of leu-kocytes, they constantly interact with the immune system, affecting innate and adaptive immune homeostasis. A growing number of studies indicate that the microbiome in the human intestine may have an impact on the functions of the central nervous system (CNS), through identified pathways called the gut–brain axis. Recent data show that the human microbiome ecosystem interferes with the brain's development, central signaling systems and behavior. It has been proposed that disruption in the human microbiome may affect the course of psychiatric disorders. The aim of this review is to summarize the recognized pathways of the gut–brain axis that have been thoroughly studied in animal models and to evaluate the role of the dialogue between the mi-crobiota and the central nervous system in schizophrenia, bipolar disorder and depression.
A b s t r a c t Angiocentric glioma (AG) is a newly-classified, very rare, WHO grade I central ne... more A b s t r a c t Angiocentric glioma (AG) is a newly-classified, very rare, WHO grade I central nervous system (CNS) lesion, occurring usually in children and young adults. Only 52 patients with AG have been reported so far, making it one of the rarest neuropathological entities. Hereby we present two new cases of AG in young subjects with detailed neuropatholog-ical investigations and a neuroradiological picture along with a brief summary of all already published literature reports of this tumor. Histopathological examination of the resected tissue from both cases revealed similar changes characteristic of AG. The tumors were composed of spindle-like, elongated cells, forming characteristic pseudorosettes around vessels and diffusively infiltrating surrounding tissue, trapping neurons between tumor cells. Noticeably, some neoplastic cells encrusting vessels extended far beyond the main tumor mass. Hypothetically, this may be responsible for the recurrence of the tumor even in the case of apparently total excision. In immunohistochemistry, AG cells were glial fibril-lary acidic protein (GFAP) and vimentin positive, also exhibiting a strikingly significant epithelial membrane antigen (EMA) dot-like staining pattern. In one of the cases, electron microscopy revealed ependymal differentiation features such as microvilli and cilia. Taken together, all these data strongly confirm a dual astroglial-ependymal nature of the tumor. Follow up corroborates benign character of this neoplasm. Both AGs reported here were immunonegative for the product of the mutated IDH-1 gene what, according to our best knowledge, has never been reported so far. It may suggest that in their pathogenesis AGs differ from grade II astrocytomas, which in most cases harbor a mutation of IDH-1. Noteworthy, neuroimaging in our cases was relatively characteristic but not conclusive, therefore biopsy (at least) is mandatory. A newly proposed so called " A-B-C " classification of long-term epilepsy-associated tumors (LEATs) places AG in a category named ANET. The authors shortly review the A-B-C classification of LEATs.
Bergmann glia (BG), a specific type of radial astro-cytes in the cerebellum, play a variety of vi... more Bergmann glia (BG), a specific type of radial astro-cytes in the cerebellum, play a variety of vital functions in the development of this structure. However, the possible role of BG in the development of abnormalities observed in individuals with autism spectrum disorder (ASD) seems to be underestimated. One of the most consistent findings observed in ASD patients is loss of Purkinje cells (PCs). Such a defect may be caused by dysregulation of glutamate homeostasis, which is maintained mainly by BG. Moreover, these glial cells are involved in long-term depression (LTD), a form of plasticity which can additionally subserve neuroprotective functions. The aim of presented review is to summarize the current knowledge about interactions which occur between PC and BG, with special emphasis on those which are relevant to the survival and proper functioning of cerebellar neurons.
Procedural learning involves the acquisition of new skills or knowledge on how to perform specifi... more Procedural learning involves the acquisition of new skills or knowledge on how to perform specific tasks. Evaluation of procedural learning is part of neuropsy- chological and neuropsychiatric diagnosis. Deficits observed in such diagnosis can be the basis for the inference of structural and functional brain disorders. The classic division of skills acquired during proce- dural learning includes four types: cognitive, percep- tual, motor and visuo-motor skills. Most of the tests examine cognitive and visuo-motor skills. This study focuses on the two latter types of skills that the acqui- sition of which is generally known as motor learning. In the natural conditions motor learning occurs both as implicit and explicit process, depending on its phase. However, paradigms used for diagnostic purposes as- sume that motor learning is an implicit process. The work provides an overview of the most popular tools used for the assessment of implicit motor learning as part of the neuropsycholog...
Lithium salts are the most commonly used drug in the treatment of bipolar disorder, with proven e... more Lithium salts are the most commonly used drug in the treatment of bipolar disorder, with proven effectiveness in re- ducing the risk of relapses. However, the narrow therapeutic range of concentrations and high risk of side effects require a closer look at their mechanisms of action. Lithium exhibits a neuroprotective effect in hippocampus and amygdala as well as the neurotoxic properties which are most evident in the cerebellum. The purpose of this paper is to review the literature on the neuroprotective and neurotoxic properties of lithium salts. Neuroprotective properties of prolonged lithium treatment result from the inhibition of neuronal excitotoxicity by blocking the NMDA receptors, and anti-apoptotic action in the particular areas of the brain. In vitro studies have also found a positive effect on neurogenesis, which coincides with the morphometric studies in humans, where increased volume of grey matter in the prefrontal cortex and the limbic system was reported. A lot of r...
The article provides an overview of the latest studies concerning the role of the cerebellum in c... more The article provides an overview of the latest studies concerning the role of the cerebellum in certain psychiatric diseases. We analyzed in detail the following disorders: schizophrenia, depression, bipolar disorder, autism and anxiety disorders with a main focus on PTSD. Thanks to its numerous connections with cortical and subcortical structures, the cerebellum participates in psychiatric disorders in various ways, both in the terms of structure: changes in cerebellar structures were noted in all analyzed diseases; and function: numerous neuroimaging data indicates a abnormal activation patterns in analyzed disorders. Still the results of the studies are hardly conclusive and the clear description of the relationship between the cerebellum and psychiatric disorders is at this moment impossible. More research is needed to provide unequivocal conclusions concerning this subject.
The study describes an abnormal convergence symptom, i.e. unilateral exophoria at near, in patien... more The study describes an abnormal convergence symptom, i.e. unilateral exophoria at near, in patients with schizophrenia (SZ) and bipolar disorder (BD). The aim of this paper is to present the symptom and discuss its possible explanations. 29 patients with SZ, 15 patients with BD and 20 healthy controls (HC) took part in the study. The neurological assessment was done with International Co-operative Ataxia Rating Scale (ICARS) and Neurological Evaluation Scale (NES). The abnormal vergence pattern was observed in 12 patients with SZ, 1 patient with BD and 0 HC. Symptom appeared statistically more often in SZ patients than in BD patients and HC. SZ patients with vergence symptom performed significantly worst in oculomotor and dysarthia subscores of ICARS. The symptom can be linked to disruptions in cortico-ponto-cerebellar network and midbrain. It was the only neurological symptom that differed SZ and BD groups, thus it might be used in differential diagnosis. Further research is needed to obtain a full clinical description of the symptom.
Emotional disorders in patients with cerebellar damage – case studies
"Aim. Growing number of research shows the role of the cerebellum in the regulation of affect. Le... more "Aim. Growing number of research shows the role of the cerebellum in the regulation of affect. Lesions of the cerebellum can lead to emotional disregulation, a significant part of the Cerebellar Cognitive Affective Syndrome. The aim of this article is to analyze the most recent studies concerning the cerebellar participation in emotional reactions and to present three cases: two female and one male who suffered from cerebellar damage and presented post-traumatic affective and personality change.
Method. The patients’ neuropsychological examination was performed with Raven’s Progressive Matrices Test – standard version, Trial Making Test, Wisconsin Card Sorting Test, Auditory Verbal Learning Test by Łuria, Benton Visual Retention Test, Verbal Fluency Test, Stroop Interference Test, Attention and Perceptivity Test (Test Uwagi i Spostrzegawczości TUS), Frontal Behavioral Inventory (FBI).
Results. The review of the literature suggest cerebellar participation, especially teh vermis and paravermial regions, in the detection, integration and filtration of emotional information and in regulation of autonomic emotional responses. In the described patients we observed: oversensitivity, irritability, impulsivity and self-neglect. The man and the woman with right-sided lesions presented similar symptoms: rigidity of thought, stubbornness, lack of criticism, jocular and inappropriate behavior. The woman with left-sided cerebellar lesion was adynamic, apathic and passive, she presented emotional blunting, social isolation, lack of interests and motivation, general cognitive slowdown.
Conclusions. Both the analyzed research and the described cases indicate the connection between the cerebellum and emotion regulation. The symptoms presented by the described patients were most probably a consequence of damaged cerebellar projections to subcortical structures (the limbic system) and frontal areas. The diversification of symptoms depending on the localization of lesions had not been described yet and seems to indicate an interesting direction for future research."
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Papers by Adrian A Chrobak
Method. The patients’ neuropsychological examination was performed with Raven’s Progressive Matrices Test – standard version, Trial Making Test, Wisconsin Card Sorting Test, Auditory Verbal Learning Test by Łuria, Benton Visual Retention Test, Verbal Fluency Test, Stroop Interference Test, Attention and Perceptivity Test (Test Uwagi i Spostrzegawczości TUS), Frontal Behavioral Inventory (FBI).
Results. The review of the literature suggest cerebellar participation, especially teh vermis and paravermial regions, in the detection, integration and filtration of emotional information and in regulation of autonomic emotional responses. In the described patients we observed: oversensitivity, irritability, impulsivity and self-neglect. The man and the woman with right-sided lesions presented similar symptoms: rigidity of thought, stubbornness, lack of criticism, jocular and inappropriate behavior. The woman with left-sided cerebellar lesion was adynamic, apathic and passive, she presented emotional blunting, social isolation, lack of interests and motivation, general cognitive slowdown.
Conclusions. Both the analyzed research and the described cases indicate the connection between the cerebellum and emotion regulation. The symptoms presented by the described patients were most probably a consequence of damaged cerebellar projections to subcortical structures (the limbic system) and frontal areas. The diversification of symptoms depending on the localization of lesions had not been described yet and seems to indicate an interesting direction for future research."
Method. The patients’ neuropsychological examination was performed with Raven’s Progressive Matrices Test – standard version, Trial Making Test, Wisconsin Card Sorting Test, Auditory Verbal Learning Test by Łuria, Benton Visual Retention Test, Verbal Fluency Test, Stroop Interference Test, Attention and Perceptivity Test (Test Uwagi i Spostrzegawczości TUS), Frontal Behavioral Inventory (FBI).
Results. The review of the literature suggest cerebellar participation, especially teh vermis and paravermial regions, in the detection, integration and filtration of emotional information and in regulation of autonomic emotional responses. In the described patients we observed: oversensitivity, irritability, impulsivity and self-neglect. The man and the woman with right-sided lesions presented similar symptoms: rigidity of thought, stubbornness, lack of criticism, jocular and inappropriate behavior. The woman with left-sided cerebellar lesion was adynamic, apathic and passive, she presented emotional blunting, social isolation, lack of interests and motivation, general cognitive slowdown.
Conclusions. Both the analyzed research and the described cases indicate the connection between the cerebellum and emotion regulation. The symptoms presented by the described patients were most probably a consequence of damaged cerebellar projections to subcortical structures (the limbic system) and frontal areas. The diversification of symptoms depending on the localization of lesions had not been described yet and seems to indicate an interesting direction for future research."