They viewed with skepticism the Republican proposal to apply private-sector market cures to the public Medicare and Medicaid, fearing that the proposals would accelerate cost-shifting to private business payers. The second patient presented with a psychosis and fever, developing severe dyskinesias involving the mouth, trunk and limbs, together with respiratory irregularities and presumed hypothalamic disturbance and disturbance of consciousness.

Big business objections have been muted, however, by the organizational weakness so vividly illustrated during the national health reform debate. Medicaid is the major national program promoting access to care for low-income populations, but the program also is a federal-state partnership. Yet Medicaid is a complex social insurance system that over time has been used to finance a variety of needs, often using cross-subsidies.

With costs rising and universal access still a remote objective, many states have turned to market-based strategies involving managed care, with the goals of generating savings for the state, improving access for Medicaid beneficiaries, and sometimes expanding coverage to those who were previously uninsured.

In addition, states vary in both the scope of their Medicaid programs and the sophistication of the skills and resources they can bring to bear in shaping them. This suggests that the neck deviations in this patient were due to a disinhibited vestibulo-collic reflex and a disturbed head position resetting mechanism.

Understanding how these influence the ability to implement market-based strategies in Medicaid and what the effects of these strategies appear to be is of crucial importance because most states now include some features of this approach in their programs.

A patient with the clinical picture of Steele-Richardson-Olszewski syndrome and an unusual intermittent neck twisting is reported. However, in response to optokinetic or vestibular stimulation, he developed full eye deviations in the direction of the slow phase of the expected nystagmus.

He had virtually no voluntary ocular movements and only very slow, low-amplitude voluntary head movements. Computed tomography (CT) brain scan was normal but in both cases EEG revealed diffuse slow wave activity. Two patients with presumed encephalitis lethargica are presented with clinical features suggestive of two forms of the disease described by Von Economo: One patient had a psychosis and a mute-akinetic syndrome associated with myoclonus.

No quick phases were observed, and the deviation outlasted the duration of the vestibular stimuli because of defective saccades. The head also turned fully during these stimuli, quicker than on attempted voluntary movements, and remained deviated similarly to the eyes.

Neck electromyographic responses in response to whole-body rotation indicated that the vestibulocollic reflex responsible for the torticollis in this patient had a short latency of approximately 30 ms. We report the case of a 44-year-old woman with a partial 6-pyruvoyl tetrahydropterin synthase (6-PTS) deficiency, whose predominant clinical symptom was generalized dystonia with marked diurnal fluctuation.

In both cases, initial cerebrospinal fluid (CSF) examination revealed an elevated white cell count (predominantly lymphocytes), elevated protein in case 2, and oligoclonal bands in both cases. The autosomal dominant ataxias (ADA) are a diverse group of multisystem, neurodegenerative disorders characterized by mutations at several chromosomal loci (SCA types 1-5, SCA type 7, DRPLA).

A 18F-Dopa positron emission tomography (PET) scan in case 2 was normal. In case 1, L-Dopa improved the akinesia, while the myoclonus responded to clonazepam. Both patients responded rapidly and dramatically to intravenous methylprednisolone. A marked and sustained positive response to levodopa was observed. A molecular genetic study revealed a homozygous mutation (I114V) in the 6-PTS gene. This study indicates that genetic abnormality in the 6-PTS gene may be a hereditary dystonia disorder.

In case 2, the severe dyskinesias failed to respond to a number of drugs, and she ultimately required paralysis to relieve her almost continuous movements. We suggest that steroid treatment should be considered in the acute phase of patients with features suggestive of encephalitis lethargica. Dystonia was present in the eyelids, oromandibular region, trunk, and extremities (Meige syndrome plus double hemiplegia-like dystonia).

We speculate that our patient has residual 6-PTS activity in the central nervous system, such as in the liver, and we suggest that residual, but insufficient production of tetrahydrobiopterin may play an important role in causing diurnal fluctuation of symptoms.