Subject(s)
Akinetic Mutism/diagnosis , Delirium/diagnosis , Epilepsy, Frontal Lobe/diagnosis , Akinetic Mutism/drug therapy , Akinetic Mutism/epidemiology , Anticonvulsants/therapeutic use , Comorbidity , Delirium/epidemiology , Electroencephalography , Epilepsy, Frontal Lobe/drug therapy , Epilepsy, Frontal Lobe/epidemiology , Female , Humans , Middle AgedABSTRACT
BACKGROUND AND PURPOSE: Akinetic mutism is thought to be an appropriate therapeutic end-point in patients with sporadic Creutzfeldt-Jakob disease (sCJD). However, prognostic factors for akinetic mutism are unclear and clinical signs or symptoms that precede this condition have not been defined. The goal of this study was to identify prognostic factors for akinetic mutism and to clarify the order of clinical sign and symptom development prior to its onset. METHODS: The cumulative incidence of akinetic mutism and other clinical signs and symptoms was estimated based on Japanese CJD surveillance data (455 cases) collected from 2003 to 2008. A proportional hazards model was used to identify prognostic factors for the time to onset of akinetic mutism and other clinical signs and symptoms. RESULTS: Periodic synchronous discharges on electroencephalography were present in the majority of cases (93.5%). The presence of psychiatric symptoms or cerebellar disturbance at sCJD diagnosis was associated with the development of akinetic mutism [hazard ratio (HR) 1.50, 95% confidence interval (CI) 1.14-1.99, and HR 2.15, 95% CI1.61-2.87, respectively]. The clinical course from cerebellar disturbance to myoclonus or akinetic mutism was classified into three types: (i) direct path, (ii) path via pyramidal or extrapyramidal dysfunction and (iii) path via psychiatric symptoms or visual disturbance. CONCLUSIONS: The presence of psychiatric symptoms or cerebellar disturbance increased the risk of akinetic mutism of sCJD cases with probable MM/MV subtypes. Also, there appear to be sequential associations in the development of certain clinical signs and symptoms of this disease.
Subject(s)
Creutzfeldt-Jakob Syndrome/diagnosis , Adult , Aged , Aged, 80 and over , Akinetic Mutism/epidemiology , Akinetic Mutism/etiology , Cerebellar Diseases/complications , Cerebellar Diseases/epidemiology , Creutzfeldt-Jakob Syndrome/epidemiology , Creutzfeldt-Jakob Syndrome/physiopathology , Disease Progression , Electroencephalography , Female , Humans , Incidence , Magnetic Resonance Imaging , Male , Mental Disorders/complications , Mental Disorders/epidemiology , Middle Aged , Myoclonus/epidemiology , Myoclonus/etiology , Predictive Value of Tests , PrognosisABSTRACT
Delirium is sometimes defined as acute onset of either overactivity or underactivity. This article reviews the nature and clinico-anatomical locations of lesions in patients with reduced activity. The term abulia is used to describe global underactivity. Abulia is customarily explained by interruptions in frontal-subcortical circuitry. These interruptions can occur with lesions in the frontal lobes, caudate nuclei, midbrain, and thalamus. The article describes the anatomy of frontal and subcortical circuits and reviews in detail individual cases and series of patients with reduced initiative and activity who have had localized central nervous system lesions.
Subject(s)
Akinetic Mutism/physiopathology , Motor Activity/physiology , Nervous System Diseases/physiopathology , Akinetic Mutism/diagnosis , Akinetic Mutism/epidemiology , Animals , Brain/physiopathology , Humans , Nerve Net/physiopathology , Nervous System Diseases/diagnosis , Nervous System Diseases/epidemiologyABSTRACT
BACKGROUND: Individual variations in the course of Lewy body Parkinson disease (PD) are well known. Patients have been classified into different clinical subtypes to identify differences in the course among the subgroups. Several studies indicate that the outcome is more favorable in tremor dominant (TD) cases but others report no difference. A majority of progression studies are based on cross-sectional single point data or short-term clinical observations. The lack of longitudinally followed autopsy-confirmed PD cohort remains a major weakness in the literature. Biochemical studies of brain indicate most pronounced abnormalities in akinetic/rigid (AR) and the least in TD cases. We postulate that PD course in these subtypes is concordant with the biochemical findings. OBJECTIVE: To compare the course in TD, mixed (MX), and AR subtypes of PD. METHODS: Longitudinal clinical follow-up and autopsy studies were performed on 166 patients with PD over 39 years (1968-2006). Patients were classified into TD, AR, and MX based on the entire clinical course. Only the pathologically confirmed PD cases were included. RESULTS: Sixty-six percent of cases had MX, 26% AR, and 8% TD profile. The age at onset was younger (p < 0.001) and progression to Hoehn & Yahr stage 4 was slower (p = 0.016) in the TD cases. Dementia was most common in AR (p = 0.039) and the least common in TD. In general, the course was most favorable in TD, followed by MX and AR subgroups. CONCLUSION: The three subtypes of Parkinson disease have different courses which are concordant with the differences in brain biochemical abnormalities.
