Persistent genital arousal disorder: a special sense neuropathy.

INTRODUCTION: Persistent genital arousal (PGAD) is a syndrome of unprovoked sexual arousal/orgasm of uncertain cause primarily reported in female patients. Most patients are referred for mental-health treatment, but as research suggests associations with neurological symptoms and conditions, there is need to analyze cases comprehensively evaluated by neurologists. METHODS: The IRB waived consent requirements for this retrospective university-hospital study. We extracted and analyzed neurological symptoms, test, and treatment results from all qualifying participants' records and recontacted some for details. RESULTS: All 10 participants were female; their PGAD symptoms began between ages 11 to 70 years. Two patterns emerged: 80% reported daily out-of-context sexual arousal episodes (≤30/day) that usually included orgasm and 40% reported lesser, often longer-lasting, nonorgasmic arousals. Most also had symptoms consistent with sacral neuropathy-70% had urologic complaints and 60% had neuropathic perineal or buttock pain. In 90% of patients, diagnostic testing identified anatomically appropriate and plausibly causal neurological lesions. Sacral dorsal-root Tarlov cysts were most common (in 4), then sensory polyneuropathy (2). One had spina bifida occulta and another drug-withdrawal effect as apparently causal; lumbosacral disc herniation was suspected in another. Neurological treatments cured or significantly improved PGAD symptoms in 4/5 patients, including 2 cures. CONCLUSIONS: Although limited by small size and referral bias to neurologists, this series strengthens associations with Tarlov cysts and sensory polyneuropathy and suggests new ones. We hypothesize that many cases of PGAD are caused by unprovoked firing of C-fibers in the regional special sensory neurons that subserve sexual arousal. Some PGAD symptoms may share pathophysiologic mechanisms with neuropathic pain and itch.

The clinical significance of bilateral basal ganglia calcification presenting with mania and delusions.

The authors present the case of a 37-year-old man who developed a psychotic manic episode and was found to have bilateral basal ganglia calcification (BGC). The authors present this case report along with a discussion of the literature on the neuropsychiatry of BGC.

The use of palliative sedation for existential distress: a psychiatric perspective.

This article introduces a structure for standardization in the ongoing debate about the application of palliative sedation for psychological and existential suffering at the end of life. We differentiate the phenomenon of existential distress from the more general one of existential suffering, defining existential distress as a special case of existential suffering that applies to persons with terminal illness. We introduce both a clinical classification system of existential distress based on proximity to expected death and a decision-making process for considering palliative sedation (represented by the mnemonic, TIRED). Neuropsychiatric clinical cases will be used to demonstrate some of the concepts and ethical arguments.

Prospective assessment of stereotactic ablative surgery for intractable major depression.

BACKGROUND: Despite therapeutic advances for major depression, a subset of patients with this disorder does not respond to conventional treatment. Stereotactic ablative procedures such as anterior cingulotomy have been performed in severely affected, treatment-resistant patients, but the long-term results of such procedures are not fully understood. METHODS: Findings are reported for 33 patients with severe treatment-resistant major depression who underwent ablative stereotactic procedures (dorsal anterior cingulotomy followed if necessary by subcaudate tractotomy). Preoperative and long-term postoperative Beck Depression Inventory scores were obtained along with postoperative Clinical Global Improvement values. Both were analyzed to evaluate patients' responses to the surgical procedure(s). RESULTS: At mean follow-up of 30 months after one or more stereotactic ablative procedures, 11 patients (33.3%) were classified as responders, 14 (42.4%) were partial responders, and 8 (24.2%) did not respond to the surgical procedure(s). Among those (17) who underwent only one procedure, seven (41.2%) responded, whereas six (35.3%) and four (23.5%) showed partial or no response, respectively. Among patients who required multiple surgical procedures, four patients (25%) responded, whereas eight (50%) and four (25%) patients demonstrated partial or no responses, respectively, at long-term follow-up evaluations. CONCLUSIONS: Approximately 75% of depression patients previously resistant to antidepressant therapies received partial or substantial benefit from stereotactic ablative procedures. Those requiring only a single anterior cingulotomy tended to demonstrate more pronounced responses than patients who underwent multiple surgical procedures.

Should electroconvulsive therapy be given to patients with intracranial vascular lesions?

The risk of rupture and hemorrhage of intracranial vascular lesions during electroconvulsive therapy (ECT) is currently unknown. We describe 2 cases in which ECT was discontinued because of perceptual disturbances, confusion, and the subsequent discovery of intracranial angiomas. ECT has been associated with nonconvulsive status epilepticus and prolonged altered mental status following treatment, but there has been scant documentation of side effects associated with intracranial vascular malformations. We review the literature on ECT in patients with such lesions and present 2 cases in which lesions were found in the context of perceptual disturbances and altered states of consciousness following ECT.

Memory dysfunction in clinical practice.

Extract: Complaints of impaired memory are amongst the most common symptoms voiced by patients to physicians in the fields of neurology, psychiatry, medicine, and surgery. Impairment of memory is one of the most disabling aspects of many neurological disorders, including neurodegenerative diseases, strokes, tumors, head trauma, hypoxia (reduced exposure of tissue to oxygen), cardiac surgery, malnutrition, attention deficit disorder, depression, anxiety, medication side-effects, and normal aging. This memory loss often impairs the patient's normal daily activities, profoundly affecting both the patients and their families. Research in memory began with neuropsychological studies of patients with focal brain lesions and now includes new methods such as PET (positron emission tomography, where the decay of an injected radioactive element or drug creates an image) and functional MRI (magnetic resonance imaging, where hydrogen atoms are polarized by a magnet and the summation of their spinning energy creates an image). Event-related methodologies have provided us with more refined and improved classification systems. Rather than conceptualizing memory as "short-term" and "long-term," we now think of memory as a collection of mental abilities that use different systems within the brain. In the present article we will summarize the four memory systems that are of clinical relevance: episodic memory, semantic memory, procedural memory, and working memory.

Cerebral metabolic correlates as potential predictors of response to anterior cingulotomy for treatment of major depression.

OBJECT: Neurosurgical procedures are a viable intervention for severe, treatment-refractory major depression, although they have been associated with only modest rates of efficacy. The purpose of this study was to identify possible neuroimaging predictors of treatment response to anterior cingulotomy in patients with major depression. METHODS: Thirteen patients underwent stereotactic anterior cingulotomy for treatment-refractory major depression. Symptom severity was measured using the Beck Depression Inventory (BDI) both before and approximately 12 months after surgery. The authors performed [18F]fluorodeoxyglucose-positron emission tomography (PET) studies in all patients preoperatively. Statistical parametric mapping methods were used to test for loci of significant correlation between preoperative regional cerebral metabolism and postoperative reduction in BDI scores. The mean (+/- standard deviation) change in the BDI score from the preoperative period (43.7 +/- 7.8) to the postoperative period (30.5 +/- 21.3) was 33.1 +/- 45.4%. Two loci--the left subgenual prefrontal cortex and left thalamus--were identified as sites at which preoperative metabolism was significantly correlated with subsequent improvement in depressive symptom severity following cingulotomy. Specifically, higher preoperative rates of metabolism at these loci were associated with better postoperative results. CONCLUSIONS: Possible PET scanning predictors of treatment response were identified in patients with major depression who had undergone anterior cingulotomy. Further research will be necessary to determine the reproducibility of this finding. If confirmed, the availability of an index for noninvasively predicting a patient's response to cingulotomy for the treatment of major depression would be of great clinical value.

Magnetic resonance imaging-guided stereotactic limbic leukotomy for treatment of intractable psychiatric disease.

OBJECTIVE: To assess the efficacy and complication rates of magnetic resonance imaging-guided stereotactic limbic leukotomy for the treatment of intractable major depressive disorder (MDD) and obsessive-compulsive disorder (OCD). METHODS: We conducted preoperative evaluations and postoperative follow-up assessments of efficacy and complications for 21 patients who underwent limbic leukotomy. Efficacy was based on physician- and patient-rated global assessments of functioning, as well as evaluations using disease-specific rating scales commonly used in studies of MDD and OCD. RESULTS: The mean time from limbic leukotomy to follow-up assessment was 26 months. On the basis of standard outcome measures, 36 to 50% of patients were considered to be treatment responders. Although permanent surgical morbidity was rare, there were reports of postoperative sequelae, including apathy, urinary incontinence, and memory complaints, which occurred in a substantial minority of cases. CONCLUSION: For this cohort of 21 patients with chronic severe MDD or OCD, who had experienced failure with an exhaustive array of previous treatments, limbic leukotomy was associated with substantial benefit for 36 to 50%. This rate is comparable to those of previous studies of limbic system surgery and indicates that limbic leukotomy is a feasible treatment option for severe, treatment-refractory MDD or OCD. Adverse consequences associated with the procedure included affective, cognitive, and visceromotor sequelae, which were generally transient.