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Bruno Estañol

Publications and source records attributed to Bruno Estañol.

7 recordsLinked to original sources
PubMed2005

Brain magnetic resonance imaging findings in patients with mitochondrial cytopathies.

BACKGROUND: Mitochondrial cytopathies (MCs) are a heterogeneous group of clinical entities, some of which have classic phenotypes. Magnetic resonance imaging (MRI) has been reported to be helpful in the diagnosis of MC. OBJECTIVE: To correlate the most common brain MRI findings reported in patients with MC with the clinical findings in patients in different MC subgroups. DESIGN: Case series. SETTING: Patients with MCs seen at the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubiran, Mexico City, Mexico. PATIENTS: Twenty-one patients with MC with the following phenotypes: chronic progressive external ophthalmoplegia (n = 7), Kearns-Sayre syndrome (n = 7), mitochondrial neurogastrointestinal encephalopathy (n = 6), and myoclonic epilepsy with ragged red fiber myopathy (n = 1). RESULTS: Brain MRI abnormalities were found in 20 (95%) of 21 patients. The most frequent abnormalities were widespread white matter hyperintensity in 19 patients (90%), supratentorial cortical atrophy in 18 patients (86%), and cerebellar atrophy in 13 patients (62%). Widespread white matter hyperintensity (P<.001) and supratentorial cortical atrophy (P = .001) were each correlated significantly with MC. Subsequent subgroup analyses showed that the absence of basal ganglia hyperintensity was correlated with Kearns-Sayre syndrome (P < .001) and the presence of supratentorial cortical atrophy was correlated with mitochondrial neurogastrointestinal encephalopathy (P = .005). CONCLUSIONS: The presence of widespread white matter hyperintensity and/or supratentorial cortical atrophy in brain MRI may help to establish the diagnosis of MC. The radiologist has a role to play in the workup of MC by confirming the diagnosis and possibly distinguishing different subgroups of MC.

Adult↗
PubMed2004

Sympathetic co-activation of skin blood vessels and sweat glands.

Skin blood vessels and sweat glands are both innervated by sympathetic C fibers. We investigated whether during diverse respiratory maneuvers the vasomotor responses (VRs) and the sympathetic skin responses (SSRs) were frequently or occasionally co-activated. We simultaneously recorded the amplitude of the vasomotor responses and the sympathetic skin responses, the ECG and the respiratory movements in 30 healthy subjects during natural breathing at rest, rhythmic respirations at 6 per minute, sudden deep inspiration and Valsalva maneuver. We found: 1) The SSR habituates with all respiratory maneuvers whereas the VRs do not habituate. 2) There was slight co-activation between the SSRs and VRs during natural default breathing (56 percent). 3) During rhythmic breathing at 6 per minute the VRs and the SSRs were frequently co-activated (97 percent). The SSR appeared at the end of the inspiration coinciding with the end of the decreased blood flow. However the SSR habituated after few rhythmic respirations. 4) During sudden deep inspiration one hundred percent of co-activations were between the initial phase of the VRs and the SSR. The SSR is large in amplitude and longer in duration than during rhythmic breathing. 5) During the Valsalva maneuver there was a strong co-activation (100 percent) particularly during the phases II and III that are characterized by vaso-constriction but also during phase IV. The SSR is the longest of duration in all of the maneuvers. The sympathetic innervation to the sweat glands of the palm of the hand and to the skin blood vessels of the fingertips is differentiated. Under normothermic conditions sudden deep inspiration and Valsalva maneuver induced a large sympathetic simultaneous outflow to the skin blood vessels and sweat glands. The simultaneous recording of skin blood flow and the SSRs provides a more complete assessment of the sympathetic outflow to the skin than either one alone.

Adolescent↗
PubMedDate not supplied

Thymectomy in myasthenia gravis: response, complications, and associated conditions.

BACKGROUND: Thymectomy is considered the most effective treatment for achieving sustained improvement as well as remission in patients with myasthenia gravis (MG), and most neurologists favor the use of this procedure. The main focus of many current studies is to determine response-predicting factors. METHODS: Clinical records of 152 patients with an established diagnosis of MG who underwent thymectomy at our institution were reviewed. The purpose was to evaluate outcome of surgical management for MG and prognostic factors that influence that outcome. RESULTS: The majority of patients were women (119 of 152); mean age was 32.10 +/- 14.42 years, while time elapsed from diagnosis to surgery was 20.67 +/- 19.7 months. Transsternal thymectomy was performed on 113 patients and transcervical on 39. Forty percent of patients achieved remission and 28% showed improvement; with this, a good response to thymectomy was seen in 68% of patients (n = 103). The most important variables associated with remission were <60 years of age, <2 years of preoperative symptoms, and use of pyridostigmine at low doses. Factors related with poor response were >60 years of age, preoperative Osserman stage other than II, use of high doses of pyridostigmine, use of corticosteroids, and presence of thymic atrophy or thymoma in histopathologic analyses. There was no mortality, although 20 patients (13%) presented complications. CONCLUSIONS: Mexican patients with MG undergoing thymectomies show improvement and remission rates similar to those reported by other studies. Age, length of symptoms, thymic pathology, and medications appear to be predictors of response to thymectomy for MG.

Adolescent↗
PubMedDate not supplied

[Effect of muscle contraction on heart rate and skin blood flow. Preliminary results].

Evaluation of heart rate variability is an important tool to study several diseases. The most important tests include the variability of the heart rate as a function of time and as function of frequency, variability of blood pressure and pulsatile cutaneous blood flow (PCBF). We studied the effect of a sustained contraction of the quadriceps muscle on the instantaneous heart rate and pulsatile cutaneous blood flow in 10 healthy subjects. We measured the R-R interval and the instantaneous heart rate, in the time domain, 10 beats before the contraction, the R-R interval that coincided with the onset of the contraction, and the 30 R-R intervals after the beginning of the contraction. To measure the PCBF we used a photopletismograph designed for this purpose. We recorded the surface electromyographic activity (EMG) of the quadriceps muscle to determine the onset and end of the muscle contraction, and measured respiratory movements with a belt. We found an increase of the instantaneous heart rate at the first and second beats after the onset of the contraction. There was a decrease of the pulsatile cutaneous blood flow of the finger pad immediately after the contraction, which lasted for approximately eight beats. The findings support the idea of a central command and co-activation of the motor and sympathetic nervous systems. This efferent sympathetic activity seems to act not just on the contracting muscle but also on the heart and the cutaneous blood vessels of the extremities. These findings imply that it is quite likely that the sympathetic efferent effect has a supraspinal origin. This is a test that could be used to study cardiovascular control by the sympathetic nervous system.

Adult↗
PubMedDate not supplied

[Response of skin blood flow to several respiratory maneuvers in healthy subjects].

INTRODUCTION: In the diagnosis of autonomic disturbances, the variability of skin blood flow (SBF) and the sympathetic skin response (SSR) in response to several respiratory maneuvers are not routinely studied. OBJECTIVE: We sought to standardize the method of SBF variability and SSR in healthy subjects during four respiratory maneuvers: 1) spontaneous breathing (SB); 2) rhythmic breathing at a rate of 6 per minute (RB); 3) sudden deep inspiration (SDI); 4) Valsalva maneuver (VM). SUBJECTS, MATERIAL AND METHOD: We studied 30 healthy subjects with a mean age of 32 years, 60% were men and 40% women. We used a photopletysmograph in the finger pad to measure SBF and surface electrodes on the palms of the hand to register the SSR. We also recorded the ECG and the respiratory movements. The variables were: 1) amplitude of SBF; 2) latency and duration of SSR; 3) percentage of decrease of the SBF during the maneuvers compared with the basal flow. RESULTS: During spontaneous breathing there was no respiratory modulation of the SBF and during RB the SBF was modulated with respiration. With SDI there was a 60% decrease of the SBF. VM induced a larger SBF decrease of 72 per cent. A significant statistical difference was revealed when we compared the decrease of SBF basal breathing with SDI and VM (P < 0.001). The difference was also significant between the SDI and VM (P < 0.001). The SBF decrease recovered more slowly after the SDI (beat 7) than after the VM (veat 6). The SBF decrease was more pronounced in magnitude during the VM. The latency of the SSR appears spontaneously and at random in 57% of subjects during normal breathing but it appeared consistently during RB, SDI, and VM. The latency of SSR was longer during SB (1.7 +/- 0.7 s), RB (1.6 +/- 0.7 s) than SDI (0.7 +/- 0.5 s) and VM (0.8 +/- 0.9 s) (P > 0.050). Duration of the SSR was significantly longer in the SDI (around 7.8 +/- 2.4 s) and during the VM (8 +/- 2.5 s) as compared to normal breathing (6.3 +/- 1.0 s) and RB (6.4 +/- 1.5 s) (P < 0.009). CONCLUSIONS: SB and RB induce periodic modulation of sympathetic activity to skin blood vessels and sweat glands. SDI and VM produced a much greater activation of sympathetic skin activity than SB and RB. The increased SSR duration and the profound SBF decrease with SDI and VM as compared to SB or RB are consistent with this hypothesis. The latency of the SSR is much shorter with SDI and VM than with SB or RB. The most likely explanation is that the SSR, under these circumstances, results from a central command. The recovery to normal SBF is faster after the VM as compared to SDI. This suggests that the withdrawal of sympathetic responses is faster after the VM. The explanation for this is unknown but suggests that it might be baroreceptor mediated.

Adult↗
PubMedDate not supplied

[Secondary Raynaud's syndrome: skin blood flow decrease between the ischemic episodes and prolongation of the vasoconstriction during the respiratory maneuvers].

UNLABELLED: The Raynaud's syndrome is an episodic skin ischemia manifested by pallor, cyanosis and erythema of the fingers in response to cold or emotional stress. The exact pathophysiology is unknown but it has been hypothetised that may be due to an autonomic alteration in the sympathetic innervation of skin blood vessels. OBJECTIVE: To study the changes of heart rate and skin blood flow (SBF) in healthy subjects and in patients with secondary Raynaud's syndrome during different respiratory maneuvers: 1. spontaneous respiration; 2. rhythmic respirations (RR), 3. sudden inspirations (SI), and 4. Valsalva maneuver (VM). METHODS: We studied 22 healthy subjects and 22 patients with secondary Raynaud's syndrome. The variables measured were: 1) RR intervals; 2) amplitudes of SBF; 3) percentage of decrease of SBF; 4) latency of the maximum decrease of SBF. RESULTS: In all patients with secondary Raynaud's syndrome the SBF was decreased basally during spontaneous rations and during all respiratory maneuvers (p < 0.001). The mean latency of recovery of the SBF was prolonged during sudden deep inspiration. The patients with Raynaud also had significant basal tachycardia at rest (p < 0.003). CONCLUSIONS: The basal skin blood flow during spontaneous respirations and in asymptomatic periods is decreased in patients with Raynaud's syndrome; this may be related to endothelial arterioral damage. The SBF was also significantly decreased dynamically during sudden inspirations (SI), rhythmic breathing (RR) and Valsalva maneuver (VM). This dynamic change suggests sympathetic hyperactivity.

Adult↗