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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

A retrospective clinical and pathological review of 905 consecutive brain tumor cases (excluding pituitary adenoma and recurrent tumor) was conducted to identify cases in which intratumoral hemorrhage was confirmed grossly and/or pathologically. There were 132 cases so identified, for an overall tumor hemorrhage rate of 14.6%; of these, 5.4% were classified as macroscopic and 9.2% as microscopic. The presence of hemorrhage was correlated with the neurological presentation. The highest hemorrhage rate (70.0%) was found in patients with prior neurological history who experienced apoplectic deterioration (acute-on-chronic presentation). Only 57.1% of patients with acute deterioration in the absence of prior neurological symptoms had hemorrhages. The highest hemorrhage rate for primary brain tumors was 29.2% for mixed oligodendroglioma/astrocytoma, while the highest hemorrhage rate for any tumor type was 50% for metastatic melanoma. The clinical relevance of tumor hemorrhage is discussed.

All four MR imaging methods had significant linear correlations with iron and could potentially be used to determine iron status of subcortical gray matter structures in MS, with R2* mapping being preferred. A reliable method of determining iron status within MS lesions was not established.

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