Vasomotor hyporeactivity in the anterior cerebral artery during motor activation in Huntington's disease patients.

This experiment used functional transcranial Doppler ultrasonography to demonstrate that blood flow velocity in the anterior cerebral artery is hyporeactive in Huntington's disease (HD) patients during maze testing (P<0.05). These vascular changes are due to tracing, as opposed to problem solving, components of the maze task. Using logistic regression, the reactivity of the ACA during activation is able to categorize patients as being either gene negative, or positive, for HD (P=0.0007). The possible role that nitric oxide/peroxynitrite may play in this phenomena is discussed.

Nonvasculitic autoimmune inflammatory meningoencephalitis (NAIM): a reversible form of encephalopathy.

Five patients, age 54 to 80 years, presented between 3 weeks and 18 months after symptomatic onset of progressive cognitive decline, psychosis, and unsteady gait that proved to be due to a steroid-responsive nonvasculitic autoimmune inflammatory meningoencephalitic syndrome. CSF examination showed elevated immunoglobulin (Ig)G index and IgG synthesis rate in all three patients in whom it was checked, and brain biopsy revealed perivascular lymphocytic infiltrates without vessel wall invasion.

A study of the cutaneous manifestations of Behçet's disease in patients from the United States.

BACKGROUND: The type and frequency of different manifestations of Behçet's disease (BD) vary in different geographic areas. This variability could affect the ability to diagnose the disease in certain areas by using standardized criteria. The frequency of cutaneous lesions in patients from the United States, where the disease is less prevalent, is not known. OBJECTIVE: We sought to determine the frequency and type of skin lesions in a series of patients with BD from the United States and to identify methods of confirmation of these lesions as part of the disease process. RESULTS: Cutaneous manifestations were present in 64% of patients with BD. Clinicians most often relied on their clinical diagnosis to identify lesions as part of the spectrum of BD. Skin biopsy specimens were generally nonspecific. CONCLUSION: Cutaneous manifestations were common in patients with BD from the United States and usually were necessary to fulfill the diagnostic criteria of the disease in most cases.

Whipple's arthritis: direct detection of Tropheryma whippelii in synovial fluid and tissue.

We describe 2 patients presenting with polyarthritis in whom the synovial fluid (1 patient) or synovial tissue (1 patient) was positive for Tropheryma whippelii, the Whipple's disease-associated bacillus, when examined by polymerase chain reaction (PCR) and DNA sequencing. Histopathologic findings were consistent with articular Whipple's disease in the synovial fluid of 1 patient and the synovial tissue of the other. In both patients, bowel mucosal specimens were negative for Whipple's disease features by histologic and PCR methods. One patient was positive for T whippelii in the peripheral blood. Control synovial fluid specimens from 40 patients with other arthritides, including Lyme arthritis, were negative. Sequencing of a 284-basepair region of the 16S ribosomal RNA gene confirmed that the sequence is closely related to the known T whippelii sequence. Both patients responded to treatment with antibiotics.

Improvement of myelopathy in Sjögren's syndrome with chlorambucil and prednisone therapy.

The authors present a patient with Sjögren's syndrome with a fluctuating and then progressive myelopathic syndrome, and optic nerve involvement. Treatment with chlorambucil and prednisone improved the patient's function from being wheelchair bound to walking unaided. Spinal MRI showed multiple, extensive intraparenchymal areas of abnormal T2-weighted signal intensity, gadolinium enhancement, and cord swelling, which also improved during the period of treatment.

Interferon-alpha treatment of Behçet's disease.

OBJECTIVE: To determine if interferon-alpha (IFN-alpha) is effective in Behçet's disease (BD). METHODS: Ten patients having active BD were entered into a 6 month open label trial of IFN-alpha therapy given by self-administered dose of 3 million units subcutaneously daily. Provision was made for downward or upward dosing depending on predetermined responses. Four study centers followed the same protocol. Patients having ocular or neurologic inflammation at onset of the study were excluded. RESULTS: Seven patients completed the trial, while 3 were removed at 3 and 4 months for side effects or lack of efficacy. There was a substantial reduction in the number of oral and genital ulcers, cutaneous lesions, and articular signs and symptoms by the end of the study. Pain scores, global assessments, and prednisone dose also declined. The IFN-alpha dose at end of study averaged 1.5 million units. Significant side effects, besides the anticipated flu-like syndrome experienced by all the patients, included single instances of seizure, psychosis, psoriasis, and hyperthyroidism. CONCLUSION: The results suggest that IFN-alpha is effective therapy for some manifestations of Behçet's disease.

Polyarticular blastomycotic arthritis.

Blastomycotic arthritis usually presents as acute monoarticular septic arthritis resembling a bacterial process. Only 2 cases in the literature have subsequently developed arthritis in a second joint. We describe the case of an elderly woman with polyarticular blastomycotic arthritis associated with lung, skin, and bone involvement. The organism was identified in the synovial fluid and skin biopsies. Our patient underwent open drainage and aspiration of the involved joints and received antimycotic therapy.

A prospective study of the safety of joint and soft tissue aspirations and injections in patients taking warfarin sodium.

OBJECTIVE: To determine the safety of joint or soft tissue aspirations and injections in patients taking warfarin sodium. METHODS: The outcome of 32 joint or soft tissue aspirations or injections in patients receiving stable doses of warfarin sodium was assessed through a standardized interview 4 weeks after the procedure. The primary outcome measure was significant joint or soft tissue hemorrhage, ascertained by patient-reported increases in swelling or warmth at the procedure site. RESULTS: None of 32 procedures was complicated by joint or soft tissue hemorrhage reported by the patients, yielding, by the "rule of threes," a risk of significant hemorrhage of < 10% (with 95% certainty). Diagnostic information was obtained for 53% of aspirated sites (8 of 15) and therapeutic benefit was noted in 74% of corticosteroid-injected sites (17 of 23). CONCLUSION: Joint or soft tissue injections and aspirations in selected patients taking warfarin sodium are associated with a low risk of hemorrhage and are often of diagnostic or therapeutic value.

The clinician-scientist in neuropsychiatry: a position statement from the Committee on Research of the American Neuropsychiatric Association.

Neuropsychiatric research seeks to improve the lives of patients with brain-based behavioral disturbances. There has been dramatic progress in diagnosis and treatment of neuropsychiatric disorders, and progress in neuroscience and biotechnology promises further success. Paradoxically, recent trends threaten to erode this progress. In this environment, neuropsychiatric clinician-scientists must advocate for the importance of research. This position statement defines neuropsychiatric research, describes current challenges to the neuropsychiatric clinician-scientist, summarizes research opportunities, describes how future neuropsychiatric clinician-investigators should be trained, and makes recommendations for promoting neuropsychiatric research.

Prevalence of apathy following head injury.

Although several studies have examined the demographics of mood disorders and personality changes following closed head injury (CHI), there are no studies that address the prevalence of apathy after CHI. Utilizing standardized evaluation tools, this study examines the prevalence of apathy in 83 consecutive patients seen in a neuropsychiatric clinic. A total of 10.84% had apathy without depression while an equal number were depressed without apathy; another 60% of patients exhibited both apathy and depression. Younger patients were more likely to be apathetic than older patients who were more likely to be depressed and apathetic. Patients with severe injury were more likely to exhibit apathy alone. Family members rated the patients higher on apathy scale. These findings suggest that apathy is a frequent symptom after head injury and may occur either alone or in association with depression.

Evaluation of glucocorticosteroid injection for the treatment of trochanteric bursitis.

OBJECTIVE: We conducted an open observational study to assess the short and longterm effect of single local glucocorticosteroid injection for trochanteric bursitis. METHODS: 75 patient diagnosed with trochanteric bursitis based on clinical criteria were injected; 20, 32, and 22 patients each received 6, 12, and 24 mg betamethasone, respectively, mixed with 4 cm3 of 1% lidocaine. A standardized baseline questionnaire was administered to assess the severity and functional limitation due to trochanteric pain, including the visual analog scale for pain. Patients were followed at Weeks 1, 6, and 26 to determine their response to treatment. RESULTS: 77.1, 68.8, 61.3% of responding patients reported improvement in pain at Week 1, 6, and 26, respectively. Patients receiving higher doses of betamethasone were more likely to experience pain relief (p < 0.0123). CONCLUSION: Corticosteroid and lidocaine injection for trochanteric bursitis is an effective therapy with prolonged benefit.

Neuropsychiatric side effects related to treatment with felbamate.

Seven patients with refractory seizure disorders and neuropsychiatric symptoms believed secondary to felbamate are presented. Five were on concomitant valproic acid (and other agents). Anergia, apathy, bradyphrenia, and increased irritability were prominent. One patient on felbamate monotherapy had a new-onset psychosis. Felbamate's NMDA receptor antagonism and GABA potentiation (perhaps enhanced by valproic acid use) are discussed as possible mechanisms of these side effects.

Obsessive-compulsive disorder after closed head injury: review of literature and report of four cases.

An increasing number of recent reports has pointed to the underlying neuropathological substrate for obsessive-compulsive disorder (OCD). Neuropsychological testing may suggest underlying organicity even though the neuroimaging studies and the neurological examination may be normal. Earlier reports are not in agreement about the laterality of deficits. Abnormalities in frontal regions, limbic areas and basal ganglia are noted in functional neuroimaging and neuropsychological studies. In closed head injury the damage tends to be diffuse, and it is not easy to clearly localize deficits or to determine their laterality. In this paper we review the various theories and literature on OCD and organicity, and report on four individuals who developed OCD symptoms after closed head injury. We also discuss their neuroimaging and neuropsychological testing results.

Clinical utility of clozapine in 16 patients with neurological disease.

Patients who develop psychosis or agitated behavior secondary to neurological disorders present a therapeutic dilemma. The authors review clinical efficacy and side effect profiles of clozapine in a cohort of 16 patients with various neurobehavioral disorders. One-third showed a marked decrease in symptoms while on clozapine. However, one-quarter developed an acute confusional state; of these, all had diffuse slowing on their baseline EEG prior to starting the drug.