A case of kleptomania?

We report on a case of kleptomania with manifestation as deceptive ordering by catalogue. The patient is additionally suffering from severe agoraphobia.     

A case of neuro-Beh?et's disease presenting as chronic progressive cerebellar ataxia]

A case of neuro-Beh?et's disease manifested as chronic progressive cerebellar ataxia is reported. A 56-year-old woman had suffered from recurrent oral aphthous ulcers, genital ulcers and polyarthritis since her late twenties. At age 53, she noticed small stepped-gait; at age 55, she developed scanning speech, a wide-based gait and memory disturbance. On admission she had oral aphthous ulcers, scarring of genital ulcers and polyarthralgia. A neurological examination revealed memory disturbance, saccadic eye movement, scanning speech, a slow tongue wiggle, moderate limb and truncal ataxia and moderate hyperreflexia in four limbs without pathological reflexes. Relevant laboratory examination data showed a positive HLA-B51. The cerebrospinal fluid (CSF) had a mild elevation of the cell counts and the amounts of protein. Brain magnetic resonance imaging showed mild atrophy of the cerebellum and brainstem. Fluoro-2-deoxyglucose-positron emission tomography showed a decreased glucose metabolism in the cerebellum and brainstem. Methylprednisolone pulse therapy (1 g x 3 days) followed by oral corticosteroids (50 mg/day) with gradual tapering markedly alleviated the cerebellar ataxia. The presence of oral and genital ulcers and CSF pleocytosis as well as effectiveness of corticosteroids in relieving the neurologic symptoms suggested neuro-Beh?et's disease. We propose the existence of a new subtype of neuro-Beh?et's disease characterized by chronic progressive cerebellar involvement possibly due to microvasculitis for which corticosteroids may be effective.     

Deep brain stimulation for Parkinson's disease and camptocormia? A case report

Camptocormia is recognised as a severe postural movement disorder complicating neurodegenerative diseases like Parkinson's disease (PD) and multisystem atrophy. Pathophysiologically two main hypotheses are discussed: (i) a disorder of the basal ganglia resulting in axial dystonia and rigidity on the one hand and (ii) an extensor truncal myopathy on the other hand. Therapeutic efforts often result in limited success. Therefore, reports on improvements by deep brain stimulation (DBS) are of great interest. However, the role of DBS in the treatment of camptocormia remains unclear. Here, we report a female PD patient who responded well to DBS of the subthalamic nucleus for severe dyskinesias and fluctuations. However, after 6 months she started to develop a rapidly progressing camptocormia which did not respond to DBS. The clinical and electrophysiological examination suggested a truncal erector myopathy. The inconsistent reports on the effects of DBS on camptocormia in PD patients suggest heterogeneous pathogenetic pathways. A pathophysiological subtype with predominant basal ganglia dysfunction and responsivity to DBS, however, seems to be rather rare. A myopathy, in contrast, seems to be more frequent and DBS is not effective in this condition. Therefore, camptocormia in PD patients is not an established indication for DBS.     

Suicidal Ideation as an Articulation of Intent: A Focus for Suicide Prevention?

Suicidal ideation is the most common of all suicidal behavior, but only a minority of ideators ever engages in overt self-harm. If ideation is to prove useful in the assessment of suicide intent and risk, factors creating continuity between suicidal ideation and action need to be carefully examined. The relationship between ideation and intent may resolve this dilemma, as intent is assessed by examining thoughts of self-harm in the distressed person, yet there is debate as to whether ideation must involve intent. Applying ideation as a risk factor is complicated by the failure to agree upon its definition within the nomenclature of suicidology (Leenaars et al., 1997; Shneidman, 1995). Suicide ideators are an important group because most suicides and parasuicides have engaged in suicidal thoughts prior to their acts (Shneidman, 1996). Identification of those ideators most likely to attempt or commit suicide is therefore a clinical priority (Bagley, 1975).     

Enuresis as the initial manifestation of neuro-Beh?et's disease. A case report]

A 20-year-old man noticed enuresis since the age of 18 years. He was also pointed out gait unsteadiness by his friends. Neurologic examination revealed spastic tetraparesis with mild cerebellar ataxia. The cerebrospinal fluid had mild pleocytosis containing neutrophils and brain magnetic resonance imaging showed mild pontocerebellar atrophy. Urodynamic study revealed uninhibited bladder and detrusor-sphincter dyssynergia. The diagnosis of neuro-Beh?et's disease was made by the past history of recurrent genital and aphthous ulcers and positive HLA-B51. The frequency of enuresis was reduced by the oral administration of corticosteroid. We speculated the patient's enuresis was caused by the central nervous disinhibition at night together with uninhibited bladder.     

Kynurenic acid leads,dopamine follows: A new case of volume transmission in the brain?

Summary. Intrastriatal infusion of nanomolar concentrations of kynurenic acid (KYNA), an astrocyte-derived neuroinhibitory tryptophan metabolite, reduces basal extracellular dopamine (DA) levels in the rat striatum. This effect is initiated by the inhibition of α7 nicotinic acetylcholine receptors (α7nAChRs) on glutamatergic afferents. The present study was designed to further investigate this functional link between KYNA and DA using striatal microdialysis in awake animals. In rats, increases in KYNA, caused by intrastriatal infusions of KYNA itself (100 nM) or of KYNA’s bioprecursor L-kynurenine (2 μM), were associated with substantial reductions in DA. Co-infusion of KYNA with the α7nAChR agonist galantamine (5 μM), but not with the NMDA receptor agonist D-serine (100 nM), prevented this effect. Moreover, KYNA also reduced DA levels in the NMDA-lesioned striatum. Conversely, extracellular DA levels were enhanced when KYNA formation was compromised, either by astrocyte poisoning with fluorocitrate or by perfusion with aminooxyacetic acid (AOAA; 5 mM), a non-specific inhibitor of KYNA synthesis. Notably, this effect of AOAA was prevented by co-perfusion with 100 nM KYNA. In the striatum of 21 day-old mice with a targeted deletion of kynurenine aminotransferase II, extracellular KYNA levels were reduced by 67 ± 6%, while extracellular DA levels were simultaneously increased by 170 ± 14%. Taken together, a picture emerges where fluctuations in the astrocytic production of KYNA, possibly through volume transmission, inversely regulate dopaminergic tone. This newly uncovered mechanism may profoundly influence DA function under physiological and pathological conditions.     

The brain behaves as a muscle?

It is known that lactate accumulates in the skeletal muscle during intense anaerobic exercise but is rapidly cleared from the muscles when they resume aerobic metabolism; however, at least some lactate reaches the blood stream. It has been observed that during maximal exercise, blood lactate increases with transport of the blood to the brain. This could be interpreted as a mechanism to protect against possible “central fatigue” in times of maximal activity during the course of which the lactate would preserve the functionality of the primary cortical motor and sensory areas, even at the expense of the efficiency of the other structures. In this way, the role of lactate, both at the muscular level and in the CNS, appears to shift from being responsible for fatigue to protector from fatigue, which represents a real transition similar to that of how the image of Mr. Hyde changes to that of Dr. Jekyll.     

Diagnosis and treatment of an obsessive–compulsive disorder following traumatic brain injury: A single case and review of the literature

A 27-year-old patient with traumatic brain injury and neuropsychiatric symptoms fitting the obsessive–compulsive disorder was investigated. Brain CT-scan revealed left temporal and bilateral fronto-basal parenchymal contusions. Main Outcome Measure was the Yale–Brown Obsessive Compulsive Scale at pre- and post-treatment and at 6 months follow-up. The combination of pharmacotherapy and psychotherapy resulted in lower intensity and frequency of symptoms. Our case illustrates the importance of a detailed diagnostic procedure in order to provide appropriate therapeutic interventions. Further studies are needed to guide the clinician in determining which patients are likely to benefit from a psychotherapeutic intervention in combination with pharmacotherapy.     

Metastatic melanoma: Surgical treatment of brain metastases – Analysis of 110 patients

New Zealand has one of the highest rates of melanoma in the world. In up to 10% of cases, the disease is metastatic at diagnosis. Cerebral metastatic involvement carries a particularly poor prognosis. 110 patients were included in the analysis. A retrospective consecutive series of patients treated surgically at Auckland City Hospital were studied, with parameters of demographics, tumour characteristics, surgery, pathology, systemic therapy and survival analysed. Mean age was 59.9 years (range 22–81 years). Median survival from date of surgery was 8.1 months (95% CI 6.9–9.4 months). Of the 58 patients tested for BRAF mutation, 28 were positive, similar to previously published data. This conferred a better prognosis with median overall survival of 12.3 months (95% CI 7.2–17.3 months) compared to 7.8 months (95% CI 5.6–10 months) for those who were negative (p < 0.05). Survival correlated positively with extent of surgical resection. Both BRAF positive status and targeted and/or immunotherapy were significant predictors of improved survival. In this cohort, radiation therapy did not show a statistically significant improvement in overall survival.Survival from resection of cerebral metastases from melanoma is improving. Survival benefit is conferred by BRAF mutation, solitary metastasis and gross total resection of lesion.     

A Japanese case of Poland-M?bius syndrome]

We herein report a Japanese case of Poland-M?bius syndrome. The patient was a 19-year-old female. She was the product of a full-term forceps delivery. Birth weight was 2500 g. She had a defect of the right pectoral muscle, and syndactyly of the right hand. When she was 10 days old, facial diplegia, bilateral abducens nerve palsy, and bilateral ptosis were also noted. She was admitted to our hospital at 19 years of age. On physical examination, she had microsyndactyly of the right hand, and her right pectoralis major muscle was absent. Neurological examination revealed bilateral abducens nerve paresis, mild impairement of the upward and adducting movement of both eyes and bilateral facial weakness and atrophy of the left side of her tongue. Her karyotype was normal. Neither R 1 nor R 2 response was evoked in the blink reflex on either side. Brain MRI disclosed thin facial nerves and atrophy of the pons and medulla. Therefore, she was diagnosed as a case of Poland-M?bius syndrome. In this case, the facial nerves were considered to be hypoplastic.