Tako-tsubo cardiomyopathy in a patient with bilateral lesions in the dorsal medulla.

Tako-tsubo-like cardiomyopathy (TTC) is much more common than originally thought. The exact pathophysiology of TTC is unclear. The most accepted theory proposes myocardial stunning of neurogenic origin, supported by the frequent antecedent of emotional or physical stress, suggesting a catecholamine-mediated mechanism. We present a patient with this syndrome and bilateral damage of the dorsal medulla oblongata likely affecting both solitary tract nuclei. Our case points to a link between baroreflex failure and TTC, highlighting the important role of sympathetic discharge in the pathophysiology of TTC.

Bilateral akinetic seizures: a clinical and electroencephalographic description.

PURPOSE: The main feature of akinetic seizures is the inhibition of voluntary movements without impairment of awareness. Most clinical information about akinetic seizures has been obtained from cortical electrical stimulation studies, whereas clinical and video-electroen-cephalography (EEG) features have not been described thoroughly. We aimed to analyze clinical and EEG characteristics of bilateral akinetic seizures (BAS). METHODS: Patients with BAS were retrospectively identified from 1,858 consecutive video-EEG studies. All patients had ictal video-EEG, comprehensive clinical evaluation, neuropsychological testing, and brain magnetic resonance imaging (MRI). RESULTS: Ten patients (nine men) were identified; mean age was 22.5 years (range 0.3-71 years) at the time of epilepsy onset and 34.9 years (range 5-73 years) at the time of evaluation. BAS was the only seizure type in four patients. BAS consisted of sudden speech and motor arrest in eight patients, whereas in two patients seizures were characterized by abrupt freezing precipitated by gait initiation. Startle precipitated BAS in four patients. Magnetic resonance imaging (MRI) showed mesial frontal lobe lesions in six patients. Epileptiform activity was restricted to the frontal midline electrodes in all patients, with variable extension to frontal regions. In five patients, BAS were initially misdiagnosed as generalized seizures or nonepileptic events. DISCUSSION: BAS should be considered in the differential diagnosis of patients reporting paroxysmal inability to move with preservation of awareness, bearing in mind that these seizures can occur spontaneously or be precipitated by startle. The diagnosis can be achieved with video-EEG monitoring, showing stereotyped semiology and distinctive EEG abnormalities, and is often supported by the presence of lesions involving the frontal lobes.

Lrrk2-associated parkinsonism is a major cause of disease in Northern Spain.

Herein we describe a comparative clinical and genetic study of Lrrk2-associated parkinsonism in Northern Spain. In our sample from the Basque region, Lrrk2 R1441G and G2019S account for 15 out of 50 kindreds (30%) with familial Parkinson's disease. We observe common founder haplotypes for both R1441G and G2019S carriers. Our findings highlight the importance of Lrrk2 parkinsonism in this population and may have important consequences for its extended Diaspora in North, Central and South Americas.

Use of ziprasidone in parkinsonian patients with psychosis.

Twelve patients with Parkinson disease and psychosis were included in an open-label 12-week trial of ziprasidone. Two patients withdrew from the treatment because of adverse effects. The remaining 10 patients reported a significant improvement in psychiatric symptoms. Altogether, there was no deterioration of motor symptoms (UPDRS III score: basal 40.4 +/- 11.1, first month 41.1 +/- 10.8; final visit, 37.7 +/- 13.3). Two patients (20%) suffered a slight deterioration in motor symptoms and another patient suffered deterioration of gait. No analytic alterations or serious adverse effects that could limit the use of ziprasidone were observed. Although controlled trials are needed, the findings suggest that ziprasidone may be effective in parkinsonian patients with psychosis.

High-dose methotrexate for intraocular lymphoma.

PURPOSE: Intraocular lymphoma (IOL) frequently coexists with primary central nervous system lymphoma(PCNSL). We sought to determine the efficacy of high-dose methotrexate (MTX) alone in patients with IOL. We also sought to determine whether micromolar levels of MTX could be achieved in the aqueous and vitreous humor of the eye after i.v. administration of the drug. EXPERIMENTAL DESIGN: Nine patients with concurrent PCNSL and IOL or isolated IOL were treated with MTX alone. All patients were treated with i.v. 8 g/m(2) MTX. MTX concentrations in serum, aqueous humor, and vitreous humor were obtained in seven of nine patients with IOL and in one additional patient with PCNSL but no evidence of IOL. RESULTS: Micromolar concentrations of MTX were present in both ocular chambers 4 h after completion of the infusion in eight of eight patients. Levels of MTX were lower in the vitreous humor compared with the aqueous humor in five of six patients in whom both chambers were assayed. Initial response of IOL to MTX was demonstrated by seven of nine patients (six complete responses and one partial response), whereas two patients had persistent IOL despite achievement of micromolar concentrations of MTX. In the patients with concurrent PCNSL and IOL, seven of seven had complete responses in the brain after treatment with MTX. Three of seven patients with initial response of IOL experienced relapse in the eye requiring orbital radiation, and four of nine patients had sustained response of IOL to MTX. CONCLUSIONS: A subset of patients with IOL may experience sustained remission when treated with high-dose i.v. MTX alone. Although micromolar MTX concentrations are present in the eye 4 h after infusion, the lower concentration achieved in vitreous humor may contribute to persistence of IOL.

Nocturnal hypertension and dysautonomia in patients with Parkinson's disease: are they related?

Orthostatic hypotension and supine hypertension frequently coexist in Parkinson's disease (PD) patients, leading to visceral damage and increased mortality rates. The aim of this paper is to analyze the frequency and association of both conditions in a sample of outpatients with PD. A total of 111 patients, diagnosed with PD, were studied. Disease duration, treatment, cardiovascular risk factors, UPDRS I-IV and Scopa Aut scale scores were reported. Subjects underwent 24-h ambulatory blood pressure (BP) monitoring and were assessed for orthostatic hypotension. We compared our results with those published in 17,219 patients using the same protocol and the same type of device. Overall, 71.1 % had no proper circadian rhythm. This frequency was significantly higher than that of the control population (48 %). The prevalence of the nondipper or riser patterns was higher in patients with orthostatic hypotension (77.8 vs. 66.7 %). There was a correlation between nightly increases in diastolic blood pressure and changes in BP during the orthostatic test. Patients taking higher doses of treatment had less decreases in SBP (cc:-0.25; p = 0.007) and DBP (cc:-0.33; p < 0.001) at night, however there was no relation with drug type. The majority of patients with Parkinson's disease show an altered circadian rhythm of blood pressure. Patients with a non-dipper or riser pattern on 24 h ABPM exhibited a higher prevalence of autonomic disorders (orthostatic hypotension) and received higher doses of dopaminergic treatment. A day-night variation in diastolic blood pressure was the most important marker of these findings.

[Controversial aspects in WHO grade II gliomas management: review of recent literature]. / Aspectos controvertidos en el manejo de gliomas hemisféricos OMS grado II: revisión de la bibliografía reciente.

INTRODUCTION: The optimal management of WHO grade II gliomas (GIIG) is currently one of the most controversial issues in neuro-oncology. AIM: This paper discusses the most controversial aspects of management of these tumors, based on a review of the literature of the last 20 years. DEVELOPMENT: Patients with GIIG often suffer cognitive and emotional disorders that go unnoticed in daily clinic, which significantly affect the quality of life. These tumors do not remain stable, as they grow slowly and steadily. In fact, growth rate is a reliable marker of their biological behaviour, with rapid growth being a marker of malignancy. Current neuro-imaging studies and stereotactic biopsy are not reliable enough for diagnosis of GIIG, thus many authors consider that a definitive diagnosis can only be obtained with a detailed histological examination after extensive surgical resection. An increasing number of studies support the prognostic impact of surgery, as it delays malignant transformation and increases survival. GIIG are frequently located in eloquent brain areas; intraoperative electrical stimulation mapping is now considered the gold standard to remove these tumors with a low risk of sequelae. CONCLUSIONS: There is growing scientific evidence against a 'wait-and-see' management of GIIG. Surgery is considered nowadays by most authors as the first step in the diagnosis and treatment of these tumors.

Impact of psychiatric symptoms and sleep disorders on the quality of life of patients with Parkinson's disease.

The objective of this study is to assess how the non-motor symptoms of Parkinson's disease (PD), such as depression, cognitive deterioration, neuropsychiatric and sleep disorders, affect the quality of life, and to compare them with the motor symptoms in order to determine their real impact. A cross-sectional study was designed including 99 patients (mean age 68.5 ± 9.9 years, duration of disease 8.7 ± 6.2 years). Demographic data, onset of PD, years on treatment with levodopa (LD), class of dopaminergic drug prescribed, and dosages were obtained. The following scales were used: quality of life (PDQ-39), Unified Parkinson's Disease Rating Scale (UPDRS I-IV), Parkinson Disease Sleep Scale (PDSS) and daytime sleepiness (Epworth), Mini-Mental State Examination, depression (HAM-D), and the neuropsychiatric inventory (NPI-10). The PDQ-39 summary index (PDQ-39 SI) was 24.7 ± 13.2. A linear regression model including all variables showed that four independent variables accounted for 67.2% of the variance in the PDQ-39 SI (F = 33,277; p < 0.001): NPI, PDSS, UPDRS IV, and UPDRS I. When sub-items of the NPI, PDSS and UPDRS IV scales are analyzed, significant correlations (p < 0.001) are found between the PDQ-39 SI and depression, agitation, apathy, anxiety, hallucinations, delusions, incontinence of urine, morning painful posturing, restlessness in bed, morning fatigue, duration of off periods, unpredictable and predictable off periods, early morning dystonia, and sudden off periods. Neuropsychiatric symptoms, especially depression, nighttime sleep disorders such as urinary incontinence, nighttime restlessness, morning fatigue and somnolence, off-period dystonia and motor fluctuations are the variables that most affect the quality of life of patients with PD.

Factors influencing the symmetry of Parkinson's disease symptoms.

INTRODUCTION: The presence of asymmetry in symptoms and clinical signs favours the diagnosis of Parkinson's disease (PD). The aim of this study is to analyse this symptom asymmetry as a function of different variables and compare it with other parkinsonisms. MATERIALS AND METHODS: 201 Patients with PD were studied. The sample was supplemented with 29 patients diagnosed with MSA-P (according to the criteria established by the American Academy of Neurology) and 17 with PSP (according to the criteria established by the NINDS-SPSP International Workshop). The symmetry was evaluated, based on items 20-23, 25 and 26 of the Unified Parkinson's Disease Rating Scale, by subtracting the motor score for the left side from that for the right side. Those patients with a difference of one point or more were designated as being asymmetric. RESULTS: Around 16.4% of patients presented symmetrical clinical symptoms. There were no differences between those patients with or without family history of the disease. Those patients with symmetric symptoms were found to have longer symptomatic disease duration (10.8 vs. 7.9 years), a worse mental state (UPDRS I: 3.9 vs. 3.2), a higher incidence of complications (UPDRS IV: 4.5 vs. 3.2) and had their activities of daily living (ADL) affected to a greater degree (UPDRS II: 13.0 vs. 11.0). Around 48.3% of the MSA-P patients and 52.9% of the PSP patients showed symmetric symptoms. CONCLUSIONS: The degree of symmetry is not useful in differentiating between sporadic and familial PD. However, the observation of highly symmetrical symptoms in a patient with short evolution time indicates that an atypical parkinsonism should be suspected.

Sleep complaints and their relation with drug treatment in patients suffering from Parkinson's disease.

The aim of this research was to quantify sleep problems in patients suffering from Parkinson's disease by means of the new Parkinson's Disease Sleep Scale (PDSS) and to correlate such problems with the possible influence of current drug treatment. A total of 70 patients (36 men and 34 women) with a diagnosis of Parkinson's disease were enrolled. Their mean age was 69.7 +/- 8.2 years, and duration of disease was 7.4 +/- 4.8 years. All patients completed the PDSS and the Unified Parkinson's Disease Rating Scale (UPDRS Parts I-IV). Drug consumption and doses were registered. The mean score on the PDSS scale was 109.23 +/- 19.75 and on the UPDRS III scale was 25.24 +/- 11.35. The lowest scores were obtained in Item 3 (sleep fragmentation): 5.53 (2.46); and in Item 8 (nocturia): 5.75 (2.91). There was a weak correlation between the PDSS and UPDRS III (cc = -0.355, P = 0.003), PDSS and UPDRS I (cc = -0.272, P = 0.02), and PDSS and UPDRS IV (cc = -0.416, P < 0.001). Motor conditions, mental state, and drug complications influence sleep quality. Although this effect was significant, it was not of a great magnitude. Dopaminergic drugs did not increase daytime sleepiness. As a whole, sleep quality in patients who took dopaminergic agonists did not differ from that of patients who took levodopa in monotherapy.