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1.
Eur J Neurol ; 27(6): 1039-1047, 2020 06.
Article in English | MEDLINE | ID: mdl-32149450

ABSTRACT

BACKGROUND AND PURPOSE: We investigated the effectiveness of intravenous thrombolysis (IVT) in acute ischaemic stroke (AIS) patients with large vessel or distal occlusions and mild neurological deficits, defined as National Institutes of Health Stroke Scale scores < 6 points. METHODS: The primary efficacy outcome was 3-month functional independence (FI) [modified Rankin Scale (mRS) scores 0-2] that was compared between patients with and without IVT treatment. Other efficacy outcomes of interest included 3-month favorable functional outcome (mRS scores 0-1) and mRS score distribution at discharge and at 3 months. The safety outcomes comprised all-cause 3-month mortality, symptomatic intracranial hemorrhage (ICH), asymptomatic ICH and severe systemic bleeding. RESULTS: We evaluated 336 AIS patients with large vessel or distal occlusions and mild stroke severity (mean age 63 ± 15 years, 45% women). Patients treated with IVT (n = 162) had higher FI (85.6% vs. 74.8%, P = 0.027) with lower mRS scores at hospital discharge (P = 0.034) compared with the remaining patients. No differences were detected in any of the safety outcomes including symptomatic ICH, asymptomatic ICH, severe systemic bleeding and 3-month mortality. IVT was associated with higher likelihood of 3-month FI [odds ratio (OR), 2.19; 95% confidence intervals (CI), 1.09-4.42], 3-month favorable functional outcome (OR, 1.99; 95% CI, 1.10-3.57), functional improvement at discharge [common OR (per 1-point decrease in mRS score), 2.94; 95% CI, 1.67-5.26)] and at 3 months (common OR, 1.72; 95% CI, 1.06-2.86) on multivariable logistic regression models adjusting for potential confounders, including mechanical thrombectomy. CONCLUSIONS: Intravenous thrombolysis is independently associated with higher odds of improved discharge and 3-month functional outcomes in AIS patients with large vessel or distal occlusions and mild stroke severity. IVT appears not to increase the risk of systemic or symptomatic intracranial bleeding.


Subject(s)
Brain Ischemia , Stroke , Administration, Intravenous , Aged , Brain Ischemia/drug therapy , Female , Fibrinolytic Agents/therapeutic use , Humans , Intracranial Hemorrhages , Male , Middle Aged , Retrospective Studies , Stroke/drug therapy , Thrombectomy , Thrombolytic Therapy , Treatment Outcome
2.
J Neurointerv Surg ; 8(12): 1226-1230, 2016 Dec.
Article in English | MEDLINE | ID: mdl-26819447

ABSTRACT

BACKGROUND: The use of retrievable stents for endovascular clot retrieval has dramatically improved successful revascularization and clinical outcome in selected patients with acute stroke. OBJECTIVE: To describe the rate and clinical consequences of unwanted spontaneous detachment of these devices during mechanical thrombectomy. METHODS: We studied 262 consecutive patients treated with the retrievable stent, Solitaire, for acute ischemic stroke between November 2008 and April 2015. Clinical, procedural, and outcome variables were compared between patients with and without unexpected detachment of this device. Detachment was classified as proximal to the stent proximal marker (type A) or distal to the marker (type B). Poor functional outcome was defined as modified Rankin scale score >2 at 90 days. RESULTS: Unwanted detachment occurred in 6/262 (2.3%) cases, four of type A and two of type B. Stent recovery was possible in three patients, all of 'type A', but in none of 'type B'. The number of prior passes was higher in patients with undesired detachment (3 (2-5) vs 2 (1-3), p=0.007). Detachment was associated with higher rate of symptomatic intracranial hemorrhage (SICH) (33.3% vs 4.3%, p=0.001), poorer outcome (100% vs 54.8%, p=0.028), and higher mortality rate at 90 days (50% vs 17%, p=0.038). CONCLUSIONS: Unwanted detachment of a Solitaire is an uncommon complication during mechanical thrombectomy in patients with acute ischemic stroke and is associated with the clot retrieval attempts, SICH, poor outcome, and higher mortality.

3.
Neurología (Barc., Ed. impr.) ; 30(6): 331-338, jul.-ago. 2015. ilus, tab
Article in Spanish | IBECS | ID: ibc-138897

ABSTRACT

Introducción: La disección de arterias cervicales (DAC) es la causa del 2-3% de ictus isquémicos y del 10-25% en pacientes jóvenes. Nuestro objetivo es evaluar si la implementación de un centro terciario de ictus (CTI) facilita el diagnóstico y modifica el pronóstico de los pacientes con ictus agudo por DAC. Pacientes y métodos: Estudio retrospectivo de un registro de pacientes consecutivos con ictus agudo por DAC. Se clasificaron según el periodo de atención: pre-CTI (octubre 2004-marzo 2008, 42 meses) o post-CTI (abril 2008-junio 2012, 51 meses). Se compararon las características basales, el método diagnóstico, el tratamiento y la evolución de estos pacientes entre ambos periodos. Resultados: Se diagnosticó a 9 pacientes con DAC en el periodo pre-CTI y 26 en el post-CTI, representando el 0,8 y el 2,1% de los ictus isquémicos atendidos en cada periodo. El diagnóstico de DAC se realizó en las primeras 24 h en el 42,3% de pacientes en el periodo post-CTI frente al 0% en el pre-CTI, gracias al uso de la arteriografía cerebral urgente como prueba diagnóstica en el 46,2% de los casos en el segundo periodo frente al 0% en el primero. La gravedad del ictus (mediana puntuación escala NIHSS 11 vs. 3, p = 0,014) y el tiempo hasta la atención neurológica (265 minutos vs. 148, p = 0,056) fueron mayores en la fase post-CTI. Se realizó tratamiento endovascular en el 34,3%, todos en el periodo post-CTI. El pronóstico funcional fue comparable en ambos periodos. Conclusiones: La implementación de un CTI incrementa la frecuencia en el diagnóstico de DAC y aumenta las opciones terapéuticas en la fase aguda del ictus en estos pacientes


Introduction: Cervical artery dissection (CAD) is the cause of 2% to 3% of ischaemic strokes and 10% to 25% of the ischaemic strokes in young people. Our objective is to evaluate whether implementation of a comprehensive stroke centre (CSC) improves the diagnosis and modifies the prognosis of patients with acute stroke due to CAD. Patients and methods: Retrospective study of a registry of consecutive patients with acute stroke due to CAD. They were classified according to the period of care at our centre: pre-CSC (October 2004-March 2008, 42 months) or post-CSC (April 2008-June 2012, 51 months). We compared baseline characteristics, methods of diagnosis, treatment and outcome of these patients in both periods. Results: Nine patients were diagnosed with CAD in pre-CSC and 26 in post-CSC, representing 0.8% and 2.1% of all ischaemic strokes treated in each period, respectively. The diagnosis of CAD was made within the first 24 hours in 42.3% of the patients in post-CSC versus 0% in pre-CSC, by using urgent cerebral angiography as a diagnostic test in 46.2% of cases in the second period compared to 0% in the first. Both severity of stroke (median NIHSS score 11 vs. 3, P = .014) and time to neurological care (265 min vs 148,P = .056) were higher in the post-CSC period. Endovascular treatment was performed in 34.3%, and all treatments were post-CSC. The functional outcome was comparable for both periods. Conclusions: Implementation of a CSC increases the frequency of the diagnosis of CAD, as well as the treatment options for these patients in the acute phase of stroke


Subject(s)
Humans , Stroke/epidemiology , Vertebral Artery Dissection/epidemiology , Ischemic Attack, Transient/epidemiology , /organization & administration , Psychosocial Impact , Angiography , Thrombolytic Therapy , Endovascular Procedures , Treatment Outcome , Early Diagnosis , Retrospective Studies
4.
J Neurointerv Surg ; 7(10): 748-51, 2015 Oct.
Article in English | MEDLINE | ID: mdl-25165385

ABSTRACT

BACKGROUND: Clinical experience with the Pipeline Embolization Device (PED) has been widely described in the literature since it obtained its European CE and FDA approvals in 2008 and 2011, respectively. The new generation of PED, the Pipeline Flex Embolization Device, received the CE mark of approval in March 2014. While the implant composition has not changed, its new delivery system has some differences. One of the main changes from the previous generation is a new delivery system that makes the device resheathable until deployed over 90% of its length. We present our preliminary experience using this device. METHODS: Between May and June 2014, six patients with six aneurysms were treated with the Pipeline Flex device. RESULTS: All devices were placed properly, without technical difficulties. We successfully resheathed and repositioned the device in two cases. Minor and major intraprocedural or periprocedural events were noted. CONCLUSIONS: The Pipeline Flex device allows more precise and controlled deployment than the current PED device. Although this preliminary experience seems positive, multicenter larger series will be needed to confirm the safety and durability of this new device.


Subject(s)
Embolization, Therapeutic/instrumentation , Intracranial Aneurysm/therapy , Outcome Assessment, Health Care , Prostheses and Implants/standards , Humans
5.
Neurologia ; 30(6): 331-8, 2015.
Article in English, Spanish | MEDLINE | ID: mdl-24560473

ABSTRACT

INTRODUCTION: Cervical artery dissection (CAD) is the cause of 2% to 3% of ischaemic strokes and 10% to 25% of the ischaemic strokes in young people. Our objective is to evaluate whether implementation of a comprehensive stroke centre (CSC) improves the diagnosis and modifies the prognosis of patients with acute stroke due to CAD. PATIENTS AND METHODS: Retrospective study of a registry of consecutive patients with acute stroke due to CAD. They were classified according to the period of care at our centre: pre-CSC (October 2004-March 2008, 42 months) or post-CSC (April 2008-June 2012, 51 months). We compared baseline characteristics, methods of diagnosis, treatment and outcome of these patients in both periods. RESULTS: Nine patients were diagnosed with CAD in pre-CSC and 26 in post-CSC, representing 0.8% and 2.1% of all ischaemic strokes treated in each period, respectively. The diagnosis of CAD was made within the first 24 hours in 42.3% of the patients in post-CSC versus 0% in pre-CSC, by using urgent cerebral angiography as a diagnostic test in 46.2% of cases in the second period compared to 0% in the first. Both severity of stroke (median NIHSS score 11 vs. 3, P=.014) and time to neurological care (265 min vs 148, P=.056) were higher in the post-CSC period. Endovascular treatment was performed in 34.3%, and all treatments were post-CSC. The functional outcome was comparable for both periods. CONCLUSIONS: Implementation of a CSC increases the frequency of the diagnosis of CAD, as well as the treatment options for these patients in the acute phase of stroke.


Subject(s)
Carotid Artery, Internal, Dissection/complications , Stroke/etiology , Acute Disease , Adult , Aged , Cerebral Angiography , Disease Progression , Emergency Medical Services , Female , Fibrinolytic Agents/therapeutic use , Humans , Male , Middle Aged , Outcome Assessment, Health Care , Retrospective Studies , Stroke/diagnosis , Stroke/drug therapy , Thrombolytic Therapy/methods , Tissue Plasminogen Activator/therapeutic use
6.
Cerebrovasc Dis ; 37(2): 134-40, 2014.
Article in English | MEDLINE | ID: mdl-24481476

ABSTRACT

BACKGROUND: Currently, treatment options for patients with strokes with unknown time of onset (UKO) remain limited. With the advance of neuroimaging and endovascular treatment (EVT), selected patients might have a chance of a therapeutic option. We sought to compare clinical outcome after EVT in patients with known time of stroke onset (KO) and in those with UKO. METHODS: We prospectively registered consecutive patients with acute large artery occlusion of the anterior territory who underwent EVT. Multimodal MR or Alberta Stroke Program early CT score (ASPECTS) and transcranial color-coded Duplex sonography were used to select patients for EVT. Recanalization, periprocedural complications, intracranial hemorrhage (ICH) and outcome were recorded. Symptomatic ICH (sICH) was defined as a worsening of ≥4 points in the National Institutes of Health Stroke Scale (NIHSS) score within 36 h in any bleeding. Favorable outcome was defined as a modified Rankin score ≤2 at 3 months. RESULTS: A total of 141 patients were studied, 109 with KO and 32 with UKO. Mean age was 66.5 versus 64.7 years (p = 0.005) and median baseline NIHSS was 18 versus 17 (p = 0.095), respectively. Prior IV tPA was more frequently administered to KO patients (62.4 vs. 9.4%, p < 0.001), whereas patient selection using multimodal MR was more frequent in patients with UKO (78.1 vs. 45.4%, p < 0.001). Median time from stroke onset or from the last time the patient was seen well to groin puncture and to recanalization was significantly longer in patients with UKO, but no differences were found in the duration of the procedure. For KO/UKO patients recanalization was seen in 77.1 vs. 65.7% (p = 0.084), sICH occurred in 10 versus 0% (p = 0.061) and favorable outcome at 3 months was achieved in 41.3 versus 50% (p = 0.382), respectively. CONCLUSIONS: Clinical outcomes in this series of EVT in ischemic stroke patients due to large anterior arterial occlusion with salvageable brain are similar for patients treated with KO and UKO. These data support a randomized study of EVT in extended or uncertain time windows..


Subject(s)
Fibrinolytic Agents/therapeutic use , Infarction, Middle Cerebral Artery/drug therapy , Stroke/therapy , Tissue Plasminogen Activator/therapeutic use , Adult , Aged , Aged, 80 and over , Female , Fibrinolytic Agents/administration & dosage , Fibrinolytic Agents/adverse effects , Humans , Intracranial Hemorrhages/chemically induced , Intracranial Hemorrhages/complications , Male , Middle Aged , Risk Factors , Stroke/diagnosis , Time Factors , Tissue Plasminogen Activator/administration & dosage , Tissue Plasminogen Activator/adverse effects , Treatment Outcome
7.
Acta Neurol Scand ; 129(3): 178-83, 2014 Mar.
Article in English | MEDLINE | ID: mdl-23848212

ABSTRACT

BACKGROUND: Acute ischemic stroke patients with unclear onset time presenting >4.5 h from last-seen-normal (LSN) time are considered late patients and excluded from i.v. thrombolysis. We aimed to evaluate whether this subgroup of patients is different from patients presenting >4.5 h from a witnessed onset, in terms of eligibility and response to computed tomography perfusion (CTP)-guided i.v. thrombolysis. METHODS: We prospectively studied consecutive acute non-lacunar middle cerebral artery (MCA) ischemic stroke patients presenting >4.5 h from LSN. All patients underwent multimodal CT and were considered eligible for i.v. thrombolysis according to CTP criteria. Two patient groups were established based on the knowledge of the stroke onset time. We compared the proportion of candidates suitable for intravenous thrombolysis between both groups, and their outcome after thrombolytic therapy. RESULTS: Among 147 MCA ischemic stroke patients presenting >4.5 h from LSN, stroke onset was witnessed in 74 and unknown in 73. Thirty-seven (50%) patients in the first group and 32 (44%) in the second met CTP criteria for thrombolysis (P = 0.7). Baseline variables were comparable between both groups with the exception of age, which was higher in the unclear onset group. The rates of early neurological improvement (54.1% vs 46.9%), 2-h MCA recanalization (43.5% vs 37%), symptomatic hemorrhagic transformation (3% vs 0%) and good 3-month functional outcome (62.2% vs 56.3%) did not differ significantly between both groups. CONCLUSION: Delayed stroke patients with unknown onset time were no different than patients >4.5 h regarding eligibility and response to CTP-based i.v. thrombolysis.


Subject(s)
Infarction, Middle Cerebral Artery/diagnostic imaging , Infarction, Middle Cerebral Artery/therapy , Thrombolytic Therapy/methods , Tomography, X-Ray Computed , Administration, Intravenous , Aged , Aged, 80 and over , Female , Humans , Longitudinal Studies , Male , Middle Aged , Retrospective Studies , Severity of Illness Index
8.
Eur J Neurol ; 20(5): 795-802, 2013 May.
Article in English | MEDLINE | ID: mdl-23278976

ABSTRACT

BACKGROUND AND PURPOSE: Perfusion-computed tomography-source images (PCT-SI) may allow a dynamic assessment of leptomeningeal collateral arteries (LMC) filling and emptying in middle cerebral artery (MCA) ischaemic stroke. We described a regional LMC scale on PCT-SI and hypothesized that a higher collateral score would predict a better response to intravenous (iv) thrombolysis. METHODS: We studied consecutive ischaemic stroke patients with an acute MCA occlusion documented by transcranial Doppler/transcranial color-coded duplex, treated with iv thrombolysis who underwent PCT prior to treatment. Readers evaluated PCT-SI in a blinded fashion to assess LMC within the hypoperfused MCA territory. LMC scored as follows: 0, absence of vessels; 1, collateral supply filling ≤ 50%; 2, between> 50% and < 100%; 3, equal or more prominent when compared with the unaffected hemisphere. The scale was divided into good (scores 2-3) vs. poor (scores 0-1) collaterals. The predetermined primary end-point was a good 3-month functional outcome, while early neurological recovery, transcranial duplex-assessed 24-h MCA recanalization, 24-h hypodensity volume and hemorrhagic transformation were considered secondary end-points. RESULTS: Fifty-four patients were included (55.5% women, median NIHSS 10), and 4-13-23-14 patients had LMC score (LMCs) of 0-1-2-3, respectively. The probability of a good long-term outcome augmented gradually with increasing LMCs: (0) 0%; (1) 15.4%; (2) 65.2%; (3) 64.3%, P = 0.004. Good-LMCs was independently associated with a good outcome [OR 21.02 (95% CI 2.23-197.75), P = 0.008]. Patients with good LMCs had better early neurological recovery (P = 0.001), smaller hypodensity volumes (P < 0.001) and a clear trend towards a higher recanalization rate. CONCLUSIONS: A higher degree of LMC assessed by PCT-SI predicts good response to iv thrombolysis in MCA ischaemic stroke patients.


Subject(s)
Cerebrovascular Circulation/physiology , Collateral Circulation/physiology , Fibrinolytic Agents/therapeutic use , Infarction, Middle Cerebral Artery/physiopathology , Perfusion Imaging , Tissue Plasminogen Activator/therapeutic use , Administration, Intravenous , Aged , Cerebrovascular Circulation/drug effects , Female , Fibrinolytic Agents/administration & dosage , Humans , Infarction, Middle Cerebral Artery/diagnostic imaging , Infarction, Middle Cerebral Artery/drug therapy , Male , Prospective Studies , Tissue Plasminogen Activator/administration & dosage , Ultrasonography
9.
Rev Neurol ; 54(5): 271-6, 2012 Mar 01.
Article in Spanish | MEDLINE | ID: mdl-22362475

ABSTRACT

AIM. To study the frequency, safety and efficacy of perfusion computed tomography (PCT), through identification of brain tissue-at-risk, to guide intravenous thrombolysis in stroke patients with regulatory exclusion criteria (SITS-MOST and ECASS-3). PATIENTS AND METHODS. We studied consecutive acute non-lacunar ischemic stroke patients. After conventional CT was considered eligible, PCT was performed in the following circumstances: 4.5 to 6 h window, wake-up stroke or unknown time of onset; extent early infarct signs on CT; minor or severe stroke; seizures or loss of consciousness. Intravenous 0.9 mg/kg alteplase was indicated if: cerebral blood volume lesion covered < 1/3 of middle cerebral artery territory; mismatch > 20% between mean transit time and cerebral blood volume maps existed; and informed consent. SITS-MOST safety-efficacy parameters were used as endpoint variables. RESULTS. Between May 2009-April 2010, 66 hyperacute ischemic stroke patients a priori not eligible for intravenous thrombolysis underwent PCT. Indications were: > 4.5 h in 18 patients, wake up stroke or unknown onset in 25, extent infarct signs in 6, seizures at onset in 11, and minor stroke (NIHSS < 4) in 6. Twenty-nine (44%) of them finally received intravenous thrombolysis. Symptomatic hemorrhagic transformation occurred in 2 (6.9%) patient and 18 (62.1%) achieved a modified Rankin scale score equal or less than 2 on day 90. CONCLUSION. A high proportion of acute stroke patients with SITS-MOST and ECASS-3 exclusion criteria can be safely and efficaciously treated with intravenous thrombolysis using a PCT selection protocol. However randomized control trials will be needed to confirm our results.


Subject(s)
Cerebral Infarction/diagnostic imaging , Cerebral Infarction/drug therapy , Stroke/diagnostic imaging , Stroke/drug therapy , Thrombolytic Therapy/methods , Tomography, X-Ray Computed/methods , Aged , Female , Humans , Male , Prospective Studies
10.
Neuroradiol J ; 25(5): 548-62, 2012 Nov.
Article in English | MEDLINE | ID: mdl-24029090

ABSTRACT

Atherosclerotic intracranial arterial stenosis is an important cause of stroke that is increasingly being treated with percutaneous transluminal angioplasty and stenting (PTAS) to prevent recurrent stroke. However, PTAS has been compared with medical management in a randomized trial (SAMMPRIS), where aggressive medical management was superior to PTAS with the use of the Wingspan stent system, however in our experience we have had good results and have experienced no complications with this therapy. In a retrospective, single-center study we enrolled seven consecutive patients with a symptomatic angiographically proven atherosclerotic intracranial arterial stenosis of the anterior and posterior circulation. All cases received adjuvant therapy (aspirin and clopidogrel or ticlopidine) before and after deployment of the device. The procedures were performed with the patient under general anesthesia. We use the Wingspan stent system. The occlusion site was middle cerebral artery (MCA) in three patients, proximal internal carotid artery (ICA) in one patient and vertebrobasilar artery in three patients. Primary interventional successful revascularization was achieved in all cases. Four patients had no residual stenosis, and the other three had 20%, 30% and 40% residual stenosis (Table 1). All patients showed a clinical improvement after stent deployment. No peri-interventional events or neurologic complications occurred directly related to the technique. Patency rate was 100% at the last examination in six cases, one case had a pre-occlusive stenosis, requiring angioplasty. No patients died during the follow-up period, and 100% of patients showed good functional outcome at three months (modified Rankin Scale score ≤ 2). Although the SAMMPRIS study showed that aggressive medical management was superior to PTAS, our results suggest that intracranial stenting is safe and effective, probably due to an extraordinary selection of candidates and to an exquisite technique.

11.
Rev Neurol ; 47(5): 249-52, 2008.
Article in Spanish | MEDLINE | ID: mdl-18780271

ABSTRACT

INTRODUCTION: Stiff-person (stiff-man) syndrome is characterised by symptoms of muscular rigidity and spasms, which are generally of an axial nature. Involuntary contractions of the agonist and antagonist muscles caused by activity of the motor units during rest are the main clinical and electrophysiological marker of the disease. The nature of the syndrome is considered to be autoimmune, with positive glutamic acid decarboxylase (anti-GAD) antibodies in most patients. These antibodies exert an influence over GABAergic transmission. CASE REPORT: A 29-year-old female who was admitted to hospital with a diagnosis of psychogenic mutism. While in hospital the patient developed a clinical picture consisting in generalised stiffness that was predominantly axial and proximal with hyperreflexia in the four limbs and strong contraction of the muscles of the abdomen. The most striking lab finding was the presence of anti-GAD, anti-parietal cells, anti-microsomal/TPO and antithyroglobulin antibodies, together with oligoclonal immunoglobulin G bands in the cerebrospinal fluid. Treatment was established with benzodiazepines, antispastic agents and corticosteroids, and the clinical symptoms progressively improved until they had partially remitted at two months. The lab findings and clinical features are compatible with stiff-person syndrome in a patient with associated psychiatric comorbidity. CONCLUSIONS: Anti-GAD antibodies are not exclusive to stiff-person syndrome and can also be found in a number of other autoimmune disorders. Other mechanisms which can also produce a dysfunction of the GABAergic system have also been suggested. The syndrome can be difficult to diagnose from the clinical point of view and it must therefore be borne in mind in patients who begin with unexplainable stiffness and spasms because it is a potentially treatable pathology.


Subject(s)
Autoantibodies/immunology , Glutamate Decarboxylase/immunology , Stiff-Person Syndrome , Adult , Autoimmune Diseases/diagnosis , Autoimmune Diseases/physiopathology , Female , Humans , Muscle Rigidity/physiopathology , Stiff-Person Syndrome/diagnosis , Stiff-Person Syndrome/physiopathology
12.
Rev. neurol. (Ed. impr.) ; 47(5): 249-252, 1 sept., 2008.
Article in Es | IBECS | ID: ibc-69875

ABSTRACT

Introducción. El síndrome de la persona rígida (stiff-man) se caracteriza por un cuadro de rigidez muscular y espasmos, generalmente de carácter axial. Las contracciones involuntarias de los músculos agonistas y antagonistas causadas por la actividad de las unidades motoras durante el reposo son el principal marcador clínico y electrofisiológico de la enfermedad. La naturaleza del síndrome se considera autoinmune, con anticuerpos antidecarboxilasa glutámica ácida (anti-GAD)positivos en la mayoría de los pacientes. Estos anticuerpos influyen en la transmisión gabérgica. Caso clínico. Mujer de 29 años que ingresó con el diagnóstico de mutismo psicógeno. Durante su ingreso desarrolló un cuadro que consistía en una rigidez generalizada de predominio axial y proximal con hiperreflexia en las cuatro extremidades y una importante contracción de la musculatura abdominal. En los estudios analíticos destacó la presencia de anticuerpos anti-GAD, anticélulas parietales, antimicrosomales TPO y antitiroglobulina, junto con la presencia de bandas oligoclonales de inmunoglobulina G en el líquido cefalorraquídeo. Se instauró un tratamiento con benzodiacepinas, antiespásticos y corticosteroides, con una mejoría progresiva del cuadro, hasta remitir parcialmente a los dos meses. Los hallazgos de laboratorio y el cuadro clínico son compatibles con un síndrome de la persona rígida en una paciente con comorbilidad psiquiátrica asociada. Conclusiones. Los anticuerpos anti-GAD no son exclusivos del síndrome de la persona rígida y también se pueden encontrar en numerosos trastornos autoinmunes. También se han postulado otros mecanismos por los cuales se produce una disfunción del sistema gabérgico.El síndrome puede ser de difícil diagnóstico desde el punto de vista clínico, por lo que debemos tenerlo presente en pacientes que comiencen con rigidez y espasmos inexplicables, dado que se trata de una patología potencialmente tratable


Introduction. Stiff-person (stiff-man) syndrome is characterised by symptoms of muscular rigidity and spasms, which are generally of an axial nature. Involuntary contractions of the agonist and antagonist muscles caused by activity of the motor units during rest are the main clinical and electrophysiological marker of the disease. The nature of the syndrome isconsidered to be autoimmune, with positive glutamic acid decarboxylase (anti-GAD) antibodies in most patients. These antibodies exert an influence over GABAergic transmission. Case report. A 29-year-old female who was admitted to hospital with a diagnosis of psychogenic mutism. While in hospital the patient developed a clinical picture consisting in generalised stiffness that was predominantly axial and proximal with hyperreflexia in the four limbs and strong contraction of the musclesof the abdomen. The most striking lab finding was the presence of anti-GAD, anti-parietal cells, anti-microsomal/TPO and antithyroglobulin antibodies, together with oligoclonal immunoglobulin G bands in the cerebrospinal fluid. Treatment was established with benzodiazepines, antispastic agents and corticosteroids, and the clinical symptoms progressively improveduntil they had partially remitted at two months. The lab findings and clinical features are compatible with stiff-person syndrome in a patient with associated psychiatric comorbidity. Conclusions. Anti-GAD antibodies are not exclusive to stiffperson syndrome and can also be found in a number of other autoimmune disorders. Other mechanisms which can also produce a dysfunction of the GABAergic system have also been suggested. The syndrome can be difficult to diagnose from the clinical point of view and it must therefore be borne in mind in patients who begin with unexplainable stiffness and spasms because it is a potentially treatable pathology


Subject(s)
Humans , Female , Adult , Stiff-Person Syndrome/diagnosis , Comorbidity , Autoimmunity , Mutism/psychology , Benzodiazepines/therapeutic use , Diazepam/therapeutic use
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