Cortical‐basal ganglionic degeneration

Abstract

Article abstract-We report our experience with 15 patients believed to have cortical-basal ganglionic degeneration. The clinical picture is distinctive, comprising features referable to both cortical and basal ganglionic dysfunction. Characteristic manifestations include cortical sensory loss, focal reflex myoclonus, "alien limb" phenomena, apraxia, rigidity and akinesia, a postural-action tremor, limb dystonia, hyperreflexia, and postural instability. The asymmetry of symptoms and signs is often striking. Brain imaging may demonstrate greater abnormalities contralateral to the more affected side. Postmortem studies in 2 patients revealed the characteristic pathologic features of swollen, poorly staining (achromatic) neurons and degeneration of cerebral cortex and substantia nigra. Biochemical analysis of 1 brain showed a severe, diffuse loss of dopamine in the striatum. This condition is more frequent than previously believed, and the diagnosis can be predicted during life on the basis of clinical findings. However, as with other "degenerative" diseases of the nervous system, a definitive diagnosis of cortical-basal ganglionic degeneration requires confirmation by autopsy. NEUROLOGY 1990;40:1203-1212 In Patient 1. A 52-year-old housewife noted a tremor of her left hand aggravated by action or anxiety beginning in 1976. Four years later she developed stiffness and slowness of the left arm and leg and slurring of her speech. She began having difficulty arising from a chair, and her left hand became progressively more difficult to use, especially for dressing. Examination disclosed an action tremor of both upper limbs, more prominent on the left, left-sided rigidity and akinesia, and generalized hyperreflexia, also greater on the left. She was given trials of levodopa/carbidopa, propranolol, methysergide, and amantadine without effect. Procyclidine produced only a dry mouth and orolingual dyskinesias, and bromocriptine was abandoned due to dyskinesias and intermittent confusion.Hypertension was noted and treated with methyldopa.In 1982, her right hand began shaking and became awkward at handwriting. Examination then showed apraxias for both eyelid opening (levator inhibition) and closure as well as apraxias for orolingual and upper limb actions, the left arm being more affected. There was severe reflex blepharospasm. Ocular movements showed impaired pursuit and saccades in all directions, in part due to easy distractibility and spasms of fixation (inability to break gaze). When requested to look in a specific direction, she often stared ahead and stated, "I can"." At other times, she spontaneously managed a full range of ocular motion. Optokinetic nystagmus was normal. There were prominent tongue dyskinesias. Her motor examination now showed pronounced rigidity in the left arm, athetosis in

Natural History of Progressive Supranuclear Palsy: A Clinicopathologic Study from a Population of Brain Donors

“…Symmetric onset of motor symptoms is characteristic, in contrast to PD and corticobasal degeneration [30, 31] where the signs are typically asymmetric and even unilateral. Although symmetry is not invariant, unilateral or predominantly unilateral motor deficits suggest a diagnosis other than PSP [13].…”

“…Down-gaze palsy is considered specific for PSP (although it occurs in corticobasal degeneration [30, 31] and, uncommonly, in other conditions [5]). Supranuclear gaze palsy was present in all cases in our study.…”

Magnetic resonance imaging findings in corticobasal degeneration

“…1,2 It was initially reported as "corticodentatonigral degeneration with neuronal achromasia." 3 Patients typically present a slowly progressive asymmetric akinetic-rigid syndrome commonly affecting the upper extremities.…”

Could a structural damage mimic a Parkinson plus syndrome?

“…Although the existing diagnostic criteria for CBD are not formally validated and definitive diagnosis is made only after neuropathological confirmation, Watts et al suggested akinesia, rigidity, postural or gait disturbance, postural and action tremor, alien limb phenomenon, dystonia, myoclonus as the major criteria for the disease diagnosis and choreoathetosis, dementia, blepharospasm, and cerebellar signs (like supranuclear gaze abnormalities) as the minor ones [4,5]. According to Riley et al, the clinical manifestations can be separated into those that appear as a result of basal ganglia impairment (eg akinesia, rigidity, dystonia, athetosis, postural instability), to those that have a cerebral cortex origin (eg alien limb phenomenon caused by cortical sensory loss, cognitive decline) and other features such as a postural-action tremor that often evolves to myoclonus, hyperreflexia, impaired ocular and eyelid motility, dysarthria and dysphagia [6]. The L-dopa response is poor.…”

“…No abnormalities appear on conventional EEG, at least at the disease onset, although some patients may present a unilateral slowing [22][23][24]. J. Vion-Dury et al found significant modification of brain electrical activity in CBD.…”