Subject(s)
Neuropeptides , POEMS Syndrome/complications , POEMS Syndrome/physiopathology , Paroxysmal Hemicrania/etiology , Vascular Endothelial Growth Factor A/blood , Adult , Angiogenesis Inhibitors/therapeutic use , Anti-Inflammatory Agents/therapeutic use , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Antibodies, Monoclonal/therapeutic use , Antibodies, Monoclonal, Humanized , Bevacizumab , Diarrhea/complications , Fatal Outcome , Fatigue/complications , Female , Humans , Hyperpigmentation/etiology , Indomethacin/therapeutic use , POEMS Syndrome/drug therapy , Paroxysmal Hemicrania/drug therapy , Paroxysmal Hemicrania/physiopathology , Prednisolone/therapeutic use , Vision Disorders/etiologySubject(s)
Arnold-Chiari Malformation/complications , Neck Pain/etiology , Nerve Compression Syndromes/etiology , Paresthesia/etiology , Tongue/innervation , Adolescent , Arnold-Chiari Malformation/pathology , Atlanto-Axial Joint/pathology , Female , Glossopharyngeal Nerve/physiopathology , Head Movements , Humans , Hypoglossal Nerve/physiopathology , Joint Dislocations/etiology , Joint Dislocations/pathology , Lingual Nerve/physiopathology , Magnetic Resonance Imaging , Spinal Nerve Roots/physiopathology , SyndromeABSTRACT
The frontal assessment battery (FAB) is a bedside test of executive function. It takes less than 10 minutes to administer and a low score indicates executive dysfunction. To determine whether the FAB could detect the more severe subcortical dementia that is a feature of PSP and differentiate it from other bradykinetic rigid syndromes, we studied 17 patients with progressive supranuclear palsy (PSP); 11 with multiple system atrophy (MSA) and 12 with Parkinson's disease (PD). We compared FAB scores with the results of more detailed tests of executive and general cognitive function.FAB scores were significantly lower in PSP than in MSA or PD (p=0.02 and p<0.001) and were also found to be significantly lower in MSA than in PD (p=0.047). We divided the study group into those with an FAB score <15 and those with an FAB score>/=5, regardless of the clinical diagnosis. While 82% of the PSP group had FAB scores of <15, such scores were recorded in only 36% of the MSA and 8% of the PD groups. The lexical fluency and motor series subscores of the FAB discriminated 70% of the PSP, MSA and PD patients. The FAB scores correlated with tests of executive function, as well as with scores on the Mattis Dementia Rating Scale, the Mini Mental State Examination and other tests of general cognitive function. A stepwise regression analysis revealed that across the groups, among the variables that correlated with FAB scores, alternating semantic fluency accounted for 80% of FAB variance.These results suggest that the FAB is a valid and easily applicable bedside test to discriminate executive dysfunction in these three frequently confused bradykinetic rigid syndromes.
Subject(s)
Cognition Disorders/diagnosis , Frontal Lobe/physiopathology , Muscle Rigidity/diagnosis , Neuropsychological Tests/statistics & numerical data , Problem Solving/physiology , Aged , Cognition Disorders/etiology , Diagnosis, Differential , Female , Humans , Male , Mental Status Schedule/statistics & numerical data , Middle Aged , Multiple System Atrophy/complications , Muscle Rigidity/complications , Neurologic Examination/methods , Parkinson Disease/complications , Prospective Studies , Reproducibility of Results , Retrospective Studies , Supranuclear Palsy, Progressive/complicationsABSTRACT
BACKGROUND: Postmortem studies have shown atrophy of the superior cerebellar peduncle (SCP) to distinguish progressive supranuclear palsy (PSP) from other neurodegenerative diseases. It is not clear whether MRI-based measurements can differentiate this relative atrophy of the SCP during life. METHODS: Volumetric MRI was acquired prospectively in 53 subjects: 19 with PSP, 10 with multiple system atrophy (MSA), 12 with Parkinson disease (PD), and 12 healthy controls. SCP volume was assessed by detailed quantitative volumetric measurement and independently by blinded visual rating of SCP atrophy. RESULTS: The mean SCP volume, corrected for total intracranial volume, was lower in patients with PSP than controls (p < 0.001), patients with MSA (p = 0.001), and patients with PD (p = 0.003). There was an overlap between individual SCP volume measurements in the PSP subjects and the other groups. Neuroradiologic rating correctly identified PSP cases based on the presence of SCP atrophy with a sensitivity of 74% and a specificity of 94%. CONCLUSIONS: The authors propose that together with other radiologic features of progressive supranuclear palsy (PSP) such as midbrain atrophy, a visual assessment of the superior cerebellar peduncle may help increase the clinical diagnostic accuracy in PSP.
Subject(s)
Cerebellum/pathology , Magnetic Resonance Imaging , Supranuclear Palsy, Progressive/pathology , Aged , Atrophy , Cerebellum/diagnostic imaging , Diagnosis, Differential , False Negative Reactions , False Positive Reactions , Female , Humans , Male , Middle Aged , Multiple System Atrophy/diagnosis , Multiple System Atrophy/diagnostic imaging , Multiple System Atrophy/pathology , Organ Size , Parkinson Disease/diagnosis , Parkinson Disease/diagnostic imaging , Parkinson Disease/pathology , Prospective Studies , Radiography , Sensitivity and Specificity , Supranuclear Palsy, Progressive/diagnosis , Supranuclear Palsy, Progressive/diagnostic imagingABSTRACT
BACKGROUND: Most magnetic resonance imaging (MRI) studies of progressive supranuclear palsy (PSP) are cross-sectional and lack post mortem confirmation of the diagnosis. MRI features described previously in PSP correspond to regions of pathological involvement demonstrated in separate studies, but serial MRI with pathological follow up has not been undertaken. OBJECTIVE: To investigate whether regions of increased atrophy rates demonstrated in PSP during life using fluid registered serial MRI correspond with pathological findings in confirmed PSP. METHODS: A 59 year old male presented with a six month history of balance problems and dysarthria. He had a symmetrical, levodopa unresponsive akinetic-rigid syndrome with a vertical supranuclear gaze palsy. A clinical diagnosis of probable PSP was made. His disease progressed relentlessly and he died five years after onset. Two serial MRI scans undertaken during life were reviewed and fluid (non-linear) registration of the images carried out. Post mortem histopathological analysis of the brain was undertaken to definitively confirm the diagnosis and compare regional pathology with the serial imaging. RESULTS: Fluid registration demonstrated greatest rates of atrophy in the brainstem and frontal cortex, in keeping with the distribution of pathology seen at autopsy. CONCLUSION: Fluid registration of serial MRI allows the topography and rates of regional atrophy in PSP to be delineated in life. Atrophy patterns correlated well with regional pathological load. These observations suggest that serial MRI with registration may help differentiate PSP from clinically similar conditions and supports its use as a surrogate marker of disease progression.
Subject(s)
Brain/pathology , Supranuclear Palsy, Progressive/complications , Supranuclear Palsy, Progressive/pathology , Disease Progression , Follow-Up Studies , Humans , Magnetic Resonance Imaging , Male , Middle AgedABSTRACT
The frontotemporal lobar degenerations (FTLDs) are a group of disorders in which the clinical picture is not necessarily predictive of the underlying neuropathology. The FTLD with ubiquitin-only-immunoreactive neuronal changes (FTLD-U) subtype is pathologically characterized by ubiquitin-positive, tau and alpha-synuclein-negative neuronal cytoplasmic inclusions in the frontotemporal cortex and hippocampal dentate fascia. When similar pathological changes are accompanied by histological features of motor neuron disease (MND), the term FTLD-MND is used. The latter pathological changes may be found in patients with or without clinical evidence of MND. We retrospectively reviewed the clinical details of three patients with a rapidly progressive, levodopa-unresponsive bradykinetic-rigid syndrome and frontal cognitive impairment. A diagnosis of progressive supranuclear palsy (PSP) had been considered in all three cases at initial presentation. Two of the cases fulfilled clinical diagnostic criteria for PSP, which was the final clinical diagnosis during life. Pathological analysis showed typical histological appearances of FTLD-MND in two cases and of FTLD-U in one case. Semi-quantitative analysis of pathological load seemed to correlate with the clinical phenotype. FTLD-U or FTLD-MND should be considered in the differential diagnosis of progressive frontal dementia with an akinetic rigid syndrome and supranuclear gaze palsy or Steele-Richardson-Olszewski disease.
