ABSTRACT
Pseudobulbar affect (PBA) is characterized by uncontrollable emotional episodes disconnected or disproportionate with mood, in association with an array of neurologic conditions. PBA is associated with disruption of descending control of brainstem motor circuitry and dysregulation of serotonergic and glutamatergic function. PBA has been historically under recognized, though advances resulting in more specific diagnostic criteria, validated rating scales, and an approved pharmacotherapy offer opportunities for improved treatment outcomes.
Subject(s)
Affective Symptoms/drug therapy , Affective Symptoms/physiopathology , Mood Disorders/drug therapy , Mood Disorders/physiopathology , Pseudobulbar Palsy/drug therapy , Pseudobulbar Palsy/physiopathology , Affective Symptoms/psychology , Brain Stem/drug effects , Brain Stem/physiopathology , Clinical Trials as Topic/methods , Humans , Mood Disorders/psychology , Motor Cortex/drug effects , Motor Cortex/physiopathology , Pseudobulbar Palsy/psychology , Psychopharmacology , Psychotropic Drugs/pharmacology , Psychotropic Drugs/therapeutic useABSTRACT
The objective of this study was to identify key features differentiating multiple system atrophy cerebellar type (MSA-C) from idiopathic late-onset cerebellar ataxia (ILOCA). We reviewed records of patients seen in the Massachusetts General Hospital Ataxia Unit between 1992 and 2013 with consensus criteria diagnoses of MSA-C or ILOCA. Twelve patients had definite MSA-C, 53 had possible/probable MSA-C, and 12 had ILOCA. Autonomic features, specifically urinary urgency, frequency, and incontinence with erectile dysfunction in males, differentiated MSA-C from ILOCA throughout the disease course (p = 0.005). Orthostatic hypotension developed later and differentiated MSA-C from ILOCA (p < 0.01). REM sleep behavior disorder (RBD) occurred early in possible/probable MSA-C (p < 0.01). Late MSA-C included pathologic laughing and crying (PLC, p < 0.01), bradykinesia (p = 0.01), and corticospinal findings (p = 0.01). MRI distinguished MSA-C from ILOCA by atrophy of the brainstem (p < 0.01) and middle cerebellar peduncles (MCP, p = 0.02). MSA-C progressed faster than ILOCA: by 6 years, MSA-C walker dependency was 100 % and ILOCA 33 %. MSA-C survival was 8.4 ± 2.5 years. Mean length of ILOCA illness to date is 15.9 ± 6.4 years. A sporadic onset, insidiously developing cerebellar syndrome in midlife, with autonomic features of otherwise unexplained bladder dysfunction with or without erectile dysfunction in males, and atrophy of the cerebellum, brainstem, and MCP points strongly to MSA-C. RBD and postural hypotension confirm the diagnosis. Extrapyramidal findings, corticospinal tract signs, and PLC are helpful but not necessary for diagnosis. Clarity in early MSA-C diagnosis can prevent unnecessary investigations and facilitate therapeutic trials.
Subject(s)
Multiple System Atrophy/diagnosis , Multiple System Atrophy/physiopathology , Adult , Age of Onset , Aged , Diagnosis, Differential , Disease Progression , Female , Follow-Up Studies , Humans , Kaplan-Meier Estimate , Male , Middle Aged , Neurologic Examination , Prospective Studies , Retrospective Studies , Spinocerebellar Degenerations/diagnosis , Spinocerebellar Degenerations/physiopathology , Young AdultABSTRACT
No abstract available.
Subject(s)
Frontotemporal Dementia , Postural Orthostatic Tachycardia Syndrome , Supranuclear Palsy, ProgressiveABSTRACT
No abstract available.
Subject(s)
Brain , Frontotemporal Dementia , Supranuclear Palsy, Progressive , Tomography, Emission-Computed, Single-PhotonABSTRACT
No abstract available.
