Persistent vegetative and minimally conscious states.

The diagnosis and management of patients with persistent vegetative (PVS) and minimally conscious (MCS) states entail powerful medical, ethical and legal debates. The recent description of the MCS highlights the crucial role of unexpected and well-documented recoveries of cognitive functions. Functional neuroimaging has provided new insights for assessing neuropathology and cerebral activity in these patients, providing information on the presence, degree, and location of any residual brain function in patients with PVS or MCS.

Irreversibility: cardiac death versus brain death.

The acceptance of brain death (BD) as death of the human being has been progressively accepted beginning in the early 1960s. The issue of irreversibility is directly related to the diagnosis of human death, and it is closely associated with the concept of potentiality, i.e.

Brain anatomy, cerebral blood flow, and connectivity in the transition from PVS to MCS.

Background: We report a 15 year-old girl with sickle cell disease, who developed important cognitive impairment due to multiple strokes, and who had been diagnosed with PVS. Nonetheless, when she was later admitted to our Institute, according to the presence of inconsistent but clearly demonstrable behavioral evidence of consciousness awareness, we changed our diagnosis to MCS.

Methods: This patient was studied by T1 MRI images, co-registration of fractional anisotropy (FA), and SPECT with MRI.

The Declaration of Sydney on human death.

On 5 August 1968, publication of the Harvard Committee's report on the subject of "irreversible coma" established a standard for diagnosing death on neurological grounds. On the same day, the 22nd World Medical Assembly met in Sydney, Australia, and announced the Declaration of Sydney, a pronouncement on death, which is less often quoted because it was overshadowed by the impact of the Harvard Report. To put those events into present-day perspective, the authors reviewed all papers published on this subject and the World Medical Association web page and documents, and corresponded with Dr A G Romualdez, the son of Dr A Z Romualdez.

Recognizing a mother's voice in the persistent vegetative state.

We studied an 8-year-old boy after a near-drowning left him in a vegetative state (VS) for 4 years before the study. Findings fulfilled all clinical criteria for the diagnosis of VS. The purpose of this study was to investigate whether there was significant differential activation of the brain in response to hearing his mother's voice compared with the voices of unknown women.

Maple syrup urine disease: clinical, EEG, and plasma amino acid correlations with a theoretical mechanism of acute neurotoxicity.

Classical Maple Syrup Urine Disease (MSUD) is a disease of infancy which is an inherited disorder of metabolism of branched-chain amino acids (BCAA). The BCAA are normally transaminated to branched-chain keto acids (BCKA). However, the enzyme required to metabolize the BCKA is deficient, resulting in elevation of both, the BCAA and the BCKA.

Neuropathological findings in the brain of Karen Ann Quinlan. The role of the thalamus in the persistent vegetative state.

Background: Karen Ann Quinlan had a cardiopulmonary arrest in 1975 and died 10 years later, having never regained consciousness. Her story prompted a national debate about the appropriateness of life-sustaining treatment in patients who are in a persistent vegetative state and led to the development of medicolegal guidelines for the care of such patients. This report describes the neuropathologic features of Quinlan's brain.

Electroencephalogram and treatment of hospitalized aggressive children with haloperidol or lithium.

Electroencephalograms (EEGs) of 48 children ages 5.2 to 12.9 years were examined on baseline placebo and on optimal dosages of haloperidol, lithium, or placebo.

Human leukocyte anitgen in torticollis and other idiopathic dystonic syndromes.

Two groups totaling 67 patients with idiopathic focal, segmental, and generalized dystonia, including torticollis, were compared with normal controls to determine whether there was a difference in the frequency of A, B, and C locus human leukocyte (HLA) antigens. The results indicated no statistically significant deviations in HLA antigen frequencies between the patients and the normal controls. Thirteen of the patients with idiopathic torsion dystonia were compared with normal controls for DR locus antigens.

Effect of L-glutamine and isoniazid on torticollis and segmental dystonia.

Fourteen patients with spasmodic torticollis and other segmental dystonic syndromes, who were refractory to previous forms of therapy, were selected for treatment with drugs intended to elevate brain gamma-aminobutyric acid (GABA) levels. These patients were simultaneously given diazepam, isoniazid, pyridoxine, and large doses of L-glutamine. Involuntary spasmodic activity improved to varying degrees in 7 patients; in 2 the dyskinesia became worse.

Iris pigmentation (melanin) in idiopathic dystonic syndromes including torticollis.

Iris pigmentation was evaluated in 153 Caucasian patients with torticollis and other focal, segmental, or generalized dystonias of unknown cause. Since these disorders are rare in non-Caucasians, it was hypothesized that a relationship might exist between decreased melanin metabolism, reflected by iris pigmentation, and a genetic predisposition to these disorders of voluntary movement. Patients were separated into two groups on the basis of iris pigmentation.

Evaluation of Parkinson's disease.

An examination was developed for patients with PD. The four major signs--rigidity, tremor, bradykinesia, and gait disorder--were assessed through a series of specific maneuvers. Each sign was allocated a number of points reflecting its relative value.

Dementia in Parkinson Disease.

In 520 patients with parkinsonism seen over eight years, 168 (32%) had moderate to marked dementia. Although the demented patients were older than the nondemented patients (70.4 versus 65.

Helping hemiparetics to help themselves. Sensory feedback therapy.

Despite the presence of some voluntary movement, the loss of discrete control impairs functioning of the arm and hand in most hemiparetics. Seventy hemiparetic patients, aged 12 to 78 years, were treated and followed up for six months to three years. Electromyographic activity monitored from dysfunctional primary movers during attempted movement was displayed to the patients as a continuous oscilloscopic trace, reflecting generated muscle activity and allowing its quantification.

A controlled crossover study of triiodothyronine in autistic children.

A placebo-controlled crossover study of behavioral effects of triiodothyronine (T3) was conducted in 30 young clinically euthyroid autistic children. Multiple independent raters and multiple rating scales were used. Except for a few symptoms that were reduced on T3, the drug did not differ from placebo.