RESUMO
Rapidly progressive dementia (RPD) is a heterogeneous group of diseases characterized by cognitive impairment and other neurological disorders developed in a short span of fewer than 2 years. Currently viewed as new and infrequent entities, most medical personnel have little understanding of it. Nevertheless, they significantly compromise many patients' quality of life. Here, we drive 3 clinical cases that evolve as RPD with different etiologies. Case 1: 70-year-old woman presented to the emergency with neuropsychiatric syndrome for 18 days. The researchers identified inflammatory cerebrospinal fluid (CSF), protein 14-3-3-positive T-tau protein, MRI: T2 and FLAIR hyperintensities in bilateral caudate nuclei with diffusion restriction, EEG shows a generalized periodic pattern with triphasic wave morphology. Case 2: 29-year-old man with cognitive impairment and faciobrachial dystonia seizure. The diagnosis was confirmed by achieving elevated antibodies against voltage-gated potassium channels. Case 3: A 49-year-old woman with encephalopathy and myoclonic seizures; EEG and MRI showed subtle changes. The patient also had a normal CSF but a positive CBA serologic NMDA-R antibody test. We described fundamental aspects of RPD to allow made differential diagnoses in patients with cognitive impairment and encephalopathy. Establishing an early and accurate diagnosis can benefit patients with RPD etiologies that are treatable and even reversible, decreasing in morbidity and mortality.
RESUMO
Frontotemporal dementia (FTD) is a neuropsychiatric pathology characterized by dysfunctions in the frontal lobe of the brain, especially in planning, execution, and inhibition tasks, with an inability to make decisions and handle different sequences, also affecting the temporal lobe. The patient presents alterations to store, consolidate, and recall information. These neurocognitive deficits are accompanied by neurobehavioral disorders such as depression, anxiety, and apathy that contribute to the worsening of the quality of life, with a high impact on the individual, social, and family level. To identify the neurobehavioral disorders and neurocognitive deterioration that present a patient diagnosed with FTD: clinical case of low incidence and prevalence disease in Colombia. A 40-year-old man, with progressive deterioration of his immediate verbal memory, low verbal fluency, aberrant motor behavior, sexual impulsivity, alterations in his executive functions, especially in planning tasks, decision-making, and inhibition was found to have a lesser degree of affectation in his visuospatial functioning and visuoconstructive abilities. It was found that the patient presents a severe dysexecutive syndrome associated with a clinical picture of FTD, correlated with an inability to process and recall information, accompanied by disorders such as depression, anxiety, and apathy. It is necessary to generate a functional neurorehabilitation plan that aims to improve the quality of life in these patients. In the same way, it is necessary to create new lines of research and intervention that have the purpose to create a greater field of heuristics or new questions in this type of neurodegenerative pathologies.
RESUMO
Corticobasal degeneration (CBD) is a pathology of low incidence and prevalence worldwide; it is accompanied by symptoms such as dystonia, rigid akinetic syndrome (bradykinesia), gait disturbances, neurological deterioration associated with severe cortical subcortical atrophy, and progressive to moderate to severe neurocognitive deficits, especially in immediate verbal memory and dorsolateral or dysexecutive syndrome. We identified neurocognitive impairment and neuropsychiatric symptoms in a patient diagnosed with CBD. Participant was a 70-year-old female patient, single; she presented progressive memory loss of an immediate verbal nature. Initially, she was diagnosed with Alzheimer's disease and Lewy body dementia, finding that she had no characteristic signs and symptoms of these pathologies. The patient presented conciliation insomnia, gait disturbances, and severe neurocognitive deficit, especially in executive functions, immediate verbal memory, and visuospatial functioning. It was found that the patient presented neurocognitive alterations of the executive type (frontal lobe) such as decision making, planning, inhibition and operative memory, correlated with a severe alteration in her basic, instrumental and advanced activities of daily life, with a high risk factor for developing dementia. It is necessary to diagnose in an assertive and timely manner in order to generate functional neurorehabilitation plans in people diagnosed with CBD, with the main objective of positively impacting quality of life, at the individual, family, and social level.
RESUMO
This study was addressed to determine the presence of Parkinson disease (PD) manifestations, their distribution according to motor subtypes, and the relationships with health-related quality of life (QoL) using the recently validated European Parkinson's Disease Association sponsored Parkinson's Disease Composite Scale (PDCS). Frequency of symptoms was determined by the scores of items (present if >0). Using ROC analysis and Youden method, MDS-UPDRS motor subtypes were projected on the PDCS to achieve a comparable classification based on the PDCS scores. The same method was used to estimate severity levels from other measures in the study. The association between the PDCS and QoL (PDQ-39) was analyzed by correlation and multiple linear regression. The sample consisted of 776 PD patients. We found that the frequency of PD manifestations with PDCS and MDS-UPDRS were overlapping, the average difference between scales being 5.5% only. Using the MDS-UPDRS subtyping, 215 patients (27.7%) were assigned as Tremor Dominant (TD), 60 (7.7%) Indeterminate, and 501 (64.6%) Postural Instability and Gait Difficulty (PIGD) in this cohort. With this classification as criterion, the analogous PDCS-based ratio provided these cut-off values: TD subtype, ≥1.06; Indeterminate, <1.06 but >0.65; and PIGD, <0.65. The agreement between the two scales on this classification was substantial (87.6%; kappa = 0.69). PDCS total score cut-offs for PD severity were: 23/24 for mild/moderate and 41/42 for moderate/severe. Moderate to high correlations (r = 0.35-0.80) between PDCS and PDQ-39 were obtained, and the four PDCS domains showed a significant independent influence on QoL. The conclusions are: (1) the PDCS assessed the frequency of PD symptoms analogous to the MDS-UPDRS; (2) motor subtypes and severity levels can be determined with the PDCS; (3) a significant association between PDCS and QoL scores exists.