Publicación Oficial de la Sociedad Ecuatoriana de Neurología, de la Liga Ecuatoriana Contra la Epilepsia y de la Sociedad Iberoamericana de Enfermedad Cerebrovascular

Reportes de Casos Clínicos

 

Síndrome de Arteria Espinal Anterior. Anterior Spinal Artery Syndrome.

Spinal cord infarction is not common to see during daily clinical practice, it is a much rarer condition than a cerebral infarction. However, it is still important, although sometimes it is a challenge, the recognition and correct early management of this pathology that can have a catastrophic prognosis. The meticulous neurological examination is the key to suspect this disease. The most frequent clinical presentation is the anterior spinal artery syndrome. Typically, this syndrome is composed of loss of motor function, pain and temperature sensitivity loss, relatively preserving the proprioceptive and vibratory sensitivity below the level of the lesion. The most important imaging study is magnetic resonance imaging. The treatment is aimed at the general support of the patient, looking for the cause and secondary prevention. We present the case of a 33-year-old male patient, admitted with weakness and paresthesias in the lower limbs, limitation for gait, in addition to 1/5 dysfunctional paraparesis, sensitive level in D5, abolished abdominal reflexes and distal socks type hypoesthesia. Magnetic resonance imaging of the lumbar spine with gadolinium revealed longitudinal hyperintensity in anterior spinal medullary T2 in sagittal sequences from T1 to T8.

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Encefalitis Límbica Autoinmune Asociada a Anticuerpos LGI1. Autoimmune Limbic Encephalitis Associated With LGI1 Antibodies.

Introduction: Leucine rich glioma inactivated protein 1 (LGI1) antibody encephalitis is a rare disease characterized by subacute memory impairment, behavioral disorders and epileptic seizures. Even most cases have a good outcome, residual cognitive deficits are common.

Case report. A 76 years old woman who started with acute onset generalized tonic – clonic seizures and subsequent impaired level of consciousness. Antiepileptic treatment was started with slight clinical improvement. In magnetic resonance imaging performed during admission left hippocampal hyperintensity was seen in T2 and T2-FLAIR sequences. As autoimmune limbic encephalitis was suspected, immunomodulatory treatment with intravenous corticosteroids and immunoglobulins was started with clinical improvement. Afterwards, anti–LGI1 antibodies were positive in cerebrospinal fluid testing.

Conclusions. Anti–LGI1 antibody related encephalitis can produce different neurological manifestations and diverse onset, even acute. Early immunomodulatory treatment is important to improve both clinical manifestations and long–term outcome.

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Encefalitis Autoinmune Anti-Receptor NMDA Reporte de Caso Clínico y Revisión de Literatura. Anti-NMDA Receptor Encephalitis Case Report And Review Of Literature

Anti-NMDA Antibody encephalitis is an autoimmune entity characterized by the presence of autoantibodies against NMDA receptors. It is important to have in mind this entity, because it can be misdiagnosed initially due to its symptoms erroneously attributed to a psychiatric disorder, and diagnosis and treatment may be delayed. In this case report we describe a young woman who entered with neurological – psychiatric symptoms that simulate an acute schizophrenia and finally was diagnosed of autoimmune encephalitis.

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Síndrome de Encefalopatía Posterior Reversible de Etiología Atípica. Posterior Reversible Encephalopathy Síndrome of Atypical Etiology

Posterior reversible encephalopathy syndrome is a clinical-radiological syndrome, characterized by images compatible with vasogenic edema, whose pathophysiology is not fully clarified. There are multiple described causes of this syndrome, mainly severe arterial hypertension, renal insufficiency, sepsis, preeclampsia or eclampsia, immunosuppressive therapy and others. We report a case of a 38-year-old man, renal post-transplant in treatment with mycophenolate and prednisone, with renal failure of the transplant and chronic anemia, hospitalized by urinary focus sepsis plus lymph node tuberculosis, who after receiving 1 concentrate of red blood cells presents seizures, Hb elevation of 3.1 g/dl and images compatible with vasogenic edema and almost complete resolution of them at 16 days.

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Parálisis Facial Secundaria a Arteritis de la Arteria Carótida Interna en Paciente Escolar. Facial Palsy Secondary To Arteritis Of The Internal Carotid Artery In A School Patient

Facial palsy can be secondary to multiple etiologies such as infectious, traumatic, neoplastic, metabolic and neurological; with resolution in most cases. It has an incidence of between 11.5 and 40.2 cases per 100,000 people/year including adult and pediatric patients. We present the case of a 9-year-old patient who consulted for facial paralysis in whom a diagnosis of stenosis of the internal carotid artery was made, with subsequent symptomatic improvement with specific treatment.

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Demencia Rápidamente Progresiva Como Manifestación de Recaída en Linfoma de Células Del Manto: Experiencia en Diagnóstico y Tratamiento. Rapidly Progressive Dementia As A Manifestation Of Relapse In Mantle Cell Lymphoma: Experience In Diagnosis And Treatment

Introduction: Rapidly progressive dementia is an entity that has a multiple and heterogeneous etiology. It is characterized by the alteration of two or more cognitive domains in a period of less than 1 to 2 years. The involvement of the central nervous system attributed to mantle cell lymphoma is rare with a poor prognosis and mainly debuts in the late stages of the disease as a relapse. Case Report: A 61-year-old male with a history of mantle cell lymphoma who presents a relapse of the central nervous system, given by a clinical course compatible with a rapidly progressive dementia and which is confirmed by flow cytometry studies in cerebrospinal fluid. It presents an adequate response to management with a tyrosine kinase inhibitor (Ibrutinib), resolving clinical symptoms and imaging findings. Discussion: The involvement of the central nervous system secondary to mantle cell lymphoma is a rare complication and debuts as a relapse with variable clinical manifestations that requires a timely intervention with the aim of improving patient survival. Therapy with a single agent such as Ibrutinib seems to be a good alternative in cases of refractoriness and neurological involvement.

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Schwannoma Con Una Localización Cervical Infrecuente en un Paciente Pediátrico: Reporte De Caso. Schwannoma With A Non-Frequent Location In Pediatric Patient. Case Report

Schwannomas are usually benign, slow-growing tumors, usually found in adults. They are very rare in the pediatric population and their diagnosis requires a high index of clinical suspicion. We present the case of a 9-year-old male patient with a history of an asymptomatic cervical mass, with no other relevant clinical history. Total excision of the mass was possible, and histopathology confirmed the diagnosis of schwannoma. The patient recovered completely, with excellent prognosis. It is necessary to keep in mind schwannomas in the differential diagnosis in cases of cervical masses in pediatric patients.

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Encefalitis Límbica Autoinmune Asociada a Anticuerpos LGI1: Presentación de un Caso y Revisión de la Bibliografía. Autoimmune Limbic Encephalitis Associated With LGI1 Antibodies: Case Report And Review Of The Literature

Introduction: Leucine rich glioma inactivated protein 1 (LGI1) antibody encephalitis is a rare disease characterized by subacute memory impairment, behavioral disorders and epileptic seizures. Even most cases have a good outcome, residual cognitive deficits are common. Case report: 76-year-old woman who started with acute onset generalized tonic – clonic seizures and subsequent impaired level of consciousness. Antiepileptic treatment was started with slight clinical improvement. In magnetic resonance imaging performed during admission left hippocampal hyperintensity was seen in T2 and T2-FLAIR sequences. As autoimmune limbic encephalitis was suspected, immunomodulatory treatment with intravenous corticosteroids and immunoglobulins was started with clinical improvement. Afterwards, anti –LGI1 antibodies were positive in cerebrospinal fluid testing. Conclusions: anti – LGI1 antibody related encephalitis can produce different neurological manifestations and diverse onset, even acute. Early immunomodulatory treatment is important to improve both clinical manifestations and long – term outcome.

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Disfunción Mioneural Periférica Como Único Elemento Para el Diagnóstico de la Distrofia Miotónica Tipo I. Myoneural Disfunction For The Diagnosis Of Myotonic Dystrophy Type I

Myotonic dystrophy type 1, also known as Steinert’s disease, is a mulsystemic disorder that primarily affects the skeletal and smooth muscle, as well as the eye, heart, endocrine system and central nervous system. This pathology is uncommon and is characterized by generalized myotonia and multiorgan damage. Its clinical expression is variable, but in most cases, there is a variable degree of muscle weakness, cardiac arrhythmias and other conduction disorders, endocrine disorders, sleep disorders, cataracts and baldness. This is a hereditary disease with three recognizable phenotypes: mild, classic and congenital. Depending on the presentation, it may show poor prognosis and a usually rapid progression, which lacks of effective treatment. Case presentation: 54-year-old female patient who enters the Traumatology service of San Vicente de Paul Hospital in Ibarra, Ecuador for presenting a left femur fracture resulting from a fall of her own height. During hospitalization, the patient presented with type II respiratory failure without apparent cause, so she was admitted to the ICU for ventilatory support. The patient had difficulty achieving ventilatory weaning due to distal and proximal muscle weakness. Electromyography reveals a myopathic pattern compatible with the diagnosis of myotonic dystrophy type I. A tracheotomy was performed, and she was discharged for follow-up by the Internal Medicine service. The performance of a molecular diagnostic study was suggested. Conclusions: The molecular study is the diagnostic gold standard to determine with certainty the presence of myotonic dystrophy type I, besides allowing to determine its severity depending on the number of repeated. However, resource limitations in the present case forced evidence to be sought for diagnosis through electromyography. The treatment remains symptomatic. Because of its inheritance pattern being autosomal dominant, due to the expansion of trinucleotides, family members must be evaluated because they may have the diagnosis even though asymptomatic.

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Imitador de Ictus Debido a Trombosis Venosa Cerebral. Stroke Mimic Due To Cerebral Venous Thrombosis

Introduction: A stroke mimic is any non-vascular pathology that presents as an acute ischemic stroke. The clinical presentation, the epidemiological factors, the time to onset, vascular distribution and the availability of imaging tests are factors that help to differentiate them. Case report: We present a case, of a woman of the third age who was taken to the emergency department due to a five-hour history of left hemiparesis. The clinical evolution and the supplementary tests allowed to exclude the diagnosis of acute ischemic stroke. The semiology was explained by a prolonged Todd’s palsy that behaved as a stroke mimic at the acute phase and the seizures occurred in the context of a cerebral venous thrombosis. Comments: Stroke mimics, in contrast to acute ischemic stroke, occur in younger patients, have fewer vascular risk factors, lower NIHSS score, less aphasia and dysphagia. The main causes are seizures and syncope. Migraines, neoplasms, toxic or metabolic alterations, encephalopathies and functional disorders should also be considered. To perform a complete neurovascular study and directed tests will allow us to make the diagnosis.

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Síndrome de Embolia Grasa Postraumática, Síntomas Neurológicos Sin Clínica Respiratoria en Ausencia de Foramen Oval Permeable. Posttraumatic Fat Embolism Syndrome, Neurological Symptoms Without Respiratory Symptoms In The Absence Of Permeable Foramen Ovale.

Fat embolism syndrome is a potentially catastrophic complication of long-bone fractures. The classic triad of symptoms are petechial skin rashes, hypoxemia, and neurological abnormalities, which usually occur within 24 to 72 hours after the fracture. The respiratory component occurs in practically all of the reports. We present the case of a patient with post traumatic fat embolism with predominant neurological symptoms, without respiratory involvement in the absence of patent foramen ovale.

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Síndrome de Fahr Secundario a Hiperparatiroidismo Primario e Isquemia Cerebral: A Propósito de un Caso. Fahr Syndrome Secondary To Primary Hyperparathyroidism And Cerebral Ischemia: A Case Report.

Fahr’s syndrome (FS) is a rare neurodegenerative condition, characterized by cerebral calcifications mainly in the basal ganglia. Although its most common presentation are the disorders of movement, cognition, behavior and epilepsy, in recent years cases of cerebrovascular disease (CVD) related to this entity have been appearing.

We report a 59-year-old male patient who presented 2 transitory ischemic attacks (TIAs), the 1st of 5 minutes, of speech alteration what happened unnoticed, and the second of 45 minutes, 2 weeks later, of speech alteration, loss of muscle strength and tingling sensation in the left side of the body. Brain computed tomography and magnetic resonance imaging revealed calcifications suggestive of FS and a treatable cause was found (primary hyperparathyroidism with hypovitaminosis D). The patient was treated with aspirin, atorvastatin and colecalciferol without vascular recurrence and the levels of vitamin D and PTH normalized. Although the association between CVD in young people and SF has not yet been determined, the occurrence of these cases leads us to suspect that ischemic CVD could be part of the natural history of this entity. Being the prevalence of FS unknown, we alert clinicians to keep CVD in mind as a form of presentation of this condition. We review the association between FS and ischemic CVD (without in clusion of aneurysmal disease).

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Estimulación Magnética Transcraneal “Theta-Burst Intermitente” en un Paciente con Trastorno del Espectro Autista: Reporte de un Caso. Transcranial Magnetic Stimulation “Theta-Burst Intermittent” In A Patient With Autism Spectrum Disorder: Case Report.

Background. Transcranial magnetic stimulation (TMS) is a potential treatment option for autism spectrum disorder (ASD) symptomatology.

Objectives. To determine the efficacy of the TMS intermittent theta-burst stimulation (iTBS) protocol over the left dorsolateral prefrontal cortex (DLPFC) in the management of pathological behaviors associated with ASD.

Patient/Methods. A 10-year-old male diagnosed with ASD by the Autism Diagnostic Observation Schedule (ADOS) presented with a family history of ASD with a younger sister diagnosed with the same disorder. Both magnetic resonance imaging (MRI) and functional MRI (fMRI) scans for emotional paradigms were performed before the iTBS treatment, immediately after the iTBS treatment, and after 6 months of iTBS treatment.

Results. The Autism Treatment Evaluation Checklist (ATEC) reports revealed improvement in communication, cognition, sociability, and behavior scales by 83%, 81%, 72%, and 52%, respectively. The overall score improved by 66% immediately after the treatment and by 55% lasting over a 6-month period. The fMRI revealed the activation of the frontal, parietal, and occipital cortex before iTBS treatment, and a better integration and activation of the frontal, temporal, and occipital cortex after iTBS treatment and persisted after 6 months.

Conclusions. iTBS is a well-tolerated, non-invasive neuromodulation technique that requires relatively less administration than the standard treatment.

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Agenesia de la Arteria Carótida Interna: Presentación de dos Casos y Revisión de la Literatura. Agenesis Of The Internal Carotid Artery: Presentation Of Two Cases And Review Of The Literature.

Introduction: Two cases of patients with left internal carotid artery agenesis are presented, the first case with symptoms of open angle glaucoma and ipsilateral cataract and the second case of a patient with tension headache, as well as a review of the literature due to the Low frequency of this development anomaly.

Clinical cases: case 1, A 66-year-old woman with visual disorders associated with cataract and glaucoma in whom, due to suspicion of a carotid fistula, a left internal carotid agenesis was documented, with collaterality through the posterior system, case 2, patient A 58-year-old man with tension headache underwent an MRI where the agenesis of the left carotid artery, segments from C3 to C7, was documented.

Conclusion: Abnormalities in the development of the carotid system occur early in embryogenesis. In relation to the moment in which the alteration occurs, different circulatory patterns have been described. It is possible that the presence of collateral circulation is the reason that the majority of cases are reported asymptomatic from the vascular neurological point of view. Despite being a rare entity, it must be known in order to differentiate it from other conditions and monitor the appearance of associated complications.

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Meningioma Intraventricular. Intraventricular Meningioma.

Intraventricular meningioma is an infrequent disorder. We report a case of a 20 years old woman with a clinical picture of headache, nausea, vomiting and gait disorder. Intraventricular meningioma was diagnosed with magnetic resonance and histopathology. A transcortical right parietal surgical approach was performed through ventricular trigone. The procedure was done without complications or sequelae.

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Temblor Mentoniano: Reporte de un Caso Aislado. Chin Tremor: Report of an Isolated Case.

Chin tremor or geniospasm is an involuntary, rhythmic, autosomal dominant hereditary movement disorder, triggered by stress or emotional situations. Registered for the first time in 1894, in Italy.

We report a 4-year-old girl with mental tremor from one year of age, with no previous inheritance, with a successful response to the use of Botulinum Toxin.

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Hiperlisinemia Como Hallazgo Sugestivo de Acidemia Propiónica. Reporte de Caso. Hyperlisinemia As A Suggestive Finding Of Propionic Acidemia. Case Report.

Introduction. Propionic acidemia (AP) is an organic acidemia (AO) with clinical presentation of neonatal onset or late. Caused by deficiency of the enzyme propionil-CoA carboxilasa that causes accumulation of propionic acid and metabolites related to propionyl-CoA in tissues. Hyperglycinemia is characteristic, but hyperlysinemia may occur. This work describes a clinical case of AP of neonatal onset with fatal outcome and striking alteration of amino acids.

Clinical case. Female newborn (RN) admitted in the neonatal unit on the third day of life due to hypoactivity, vomiting and lethargy. Subsequent respiratory distress and cardiac arrest occurred, dying before a biochemical diagnosis was established. Initial paraclinics evidenced metabolic acidosis, leukopenia, hypoglycemia, later documented hyperglycinemia, hypercystinemia and severe hyperlysinemia. The organic acid chromatography in urine identified 3-hydroxy-propionic acid, methyl citrate and propionylglycine among other toxic metabolites, confirming the diagnosis.

Conclusions. AP is an inborn error of autosomal recessive metabolism of low incidence. The presence of severe metabolic acidosis, pancytopenia, hypoglycemia and family history should alert about this diagnosis. Additionally, although the definitive biochemical diagnosis is organic acids in urine, the presence of hyperammonemia, hyperglycinemia and hyperlysinemia can be highly suggestive of this disorder.

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Complejo Esclerosis Tuberosa Atípico Con Espasmos Epilépticos de Inicio Tardío: Diagnóstico Complejo Por Telemedicina En Una Comunidad Rural de Honduras. Atypical Tuberous Sclerosis Complex With Late Onset Epileptic Spasms: Complex Diagnosis by Telemedicine in a Rural Community of Honduras.

Tuberous sclerosis complex (TSC) is an uncommon neurocutaneous disease often underdiagnosed in remote areas where specialists in neurology are usually not available. A 22 month-old girl was taken to a clinic in a rural village presenting refractory epilepsy of undetermined cause that started at 18 months of age with upward eye deviation episodes and epileptic extensor spasms. She also presented perinasal angiofibromas and hypomelanotic skin lesions in trunk. The interictal encephalogram done in the community showed multifocal and generalized epileptic activity. The girl had been taken to the city for a brain magnetic resonance, which revealed multifocal subcortical hyperintensities and subependymal nodules. By teleconsult between community doctors and national and international specialists, atypical TSC with late onset epileptic spasms was diagnosed. The recommended treatment resulted in controlled seizures, and a follow-up plan was established. This case illustrates that telemedicine units in rural areas are a technological option to provi de access to specialized epilepsy care.

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Demencia por Cuerpos de Lewy, un Reto Diagnóstico. Dementia With Lewy Bodies, A Diagnostic Challenge.

Dementia with Lewy bodies is a neurodegenerative disease of unknown etiology, it is the second cause of dementia of the sixth decade of life; Its diagnosis is a challenge, because certain signs and symptoms that it presents are typical of Parkinson’s Disease and Alzheimer’s Disease. The following case report is one of the few documented patients with Dementia with Lewy bodies in Ecuador. We report this in order to state the diagnostic difficulty that this pathology generates and describe the main characteristics that differentiate it from other dementia syndromes, highlighted in the recently updated criteria by the Consortium of Dementia with Lewy bodies. A meticulous neurological examination and neuropsychological assessment were essential in the study and prognosis of the patient. Dementia with Lewy bodies requires a thorough diagnosis, due to the challenge that originates its early recognition; the criteria described accelerated their recognition due the update of the recommendations on the clinical diagnosis of Dementia with Lewy bodies.

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Síndrome de Kinsbourne: Papel de Rituximab. Kinsbourne Syndrome: Role of Rituximab.

Kinsbourne syndrome, also known as “Opsoclonus-myoclonus syndrome” or “Dancing eyes syndrome” is a rare condition characterized by rapid, irregular, multi-directional eye movements (opsoclonus), myoclonic movements in the trunk, face and/or limbs and ataxia. It occurs in children aged between 6 and 36 months. Its etiology may be paraneoplasic (neuroblastoma), nonparaneoplasic (infectious processes) or idiopathic. Regardless of its etiology, immunosuppressive drugs have been used in order to reduce the formation of antibodies possibly involved in the pathophysiology. We report the case of a four years-old boy with this syndrome secondary to an infectious urinary illness. He had ataxia, opsoclonus, upper limbs myoclonus, irritability and altered sleep pattern. Neuroblastoma was ruled out. Initial management was done with methylprednisolone pulses, followed by oral prednisolone and intravenous gammaglobulin. He started with rituximab with a good response. He had progressive clinical improvement, and is currently asymptomatic with no sequelae. In these patients a paraneoplasic syndrome should always be ruled out.

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Leucoencefalopatía Post-Radioterapia: Reporte de Caso y Revisión de Literatura. Radiation-Induced Leukoencephalopathy: Case Report And Literature Review.

Radiation-induced leukoencephalopathy presents clinically as a cognitive disorder chronologically associated with radiotherapy. The characteristic findings are: acute reversible edema, demyelination and vascular necrosis associated with thrombosis. The diagnostic test of choice is magnetic resonance imaging (MRI) and the treatment is with corticosteroids. We present a case of a patient with brain metastases who developed a post-radiotherapy leukoencephalopathy.

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Síndrome de Guillain Barré: Reporte de un Caso Causado por Enterovirus. Guillain Barre Syndrome: Report Of a Case Caused By Enterovirus.

Guillain Barre syndrome (GBS) is an acute inflammatory polyradiculoneuropathy of autoimmune etiology  that is usually preceded by a respiratory or gastrointestinal infection. It is characterized by a flaccid, symmetric, ascendent, arreflexic paralysis of rapid evolution. We describe a case of a 33 years old male patient, with a history of flu two weeks before admission, in addition to liquid stools that reverted spontaneously; his  clinical picture consisted of a six days history of cuadriparesis aggravated by respiratory weakness, ending in mechanical ventilation. Examination of cerebrospinal fluid (CSF) revealed albumino-cytological disociation and PCR test resulted positive for Enterovirus. The patient received treatment with intravenous immunoglobulin with a favorable outcome. The purpose of this paper is to report a clinical case of GBS due to a new causal agent.

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Síndrome de Tourette Familiar: Reporte De Caso y Revisión de la Literatura. Familial Tourette Syndrome: Case Report And Review Of Literature.

Tourette syndrome is a childhood neuropsychiatric disorder characterized by multiple motor and vocal tics of at least one year. Its exact etiology is unknown, but fronto-subcortical neural pathways and complex interactions between social, environmental and genetic factors have been involved. Genetic studies have reported susceptibility loci in genes involved in synaptic connection, however, evidence is needed in larger samples. This case report describes a 14-year-old boy with a personal and family history of Tourette’s syndrome, suggesting an autosomal dominant mechanism of inheritance.

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Manifestaciones Neuropsiquiátricas de la Enfermedad de Whipple en una Adolescente: A Propósito de un Caso. Neuropsychiatric Manifestations Of Whipple Disease In An Adolescent: Case Report.

Whipple disease (WD) is a rare chronic systemic infection caused by the actinomycete Tropheryma Whipplei. The clinical manifestations are mainly intestinal, although occasionally  may present extraintestinal locations (joints, heart, pulmonary, central nervous system…) , and even the last can be the only symptom.

We present a 15-year-old female patient referred to the psychiatry service, with a diagnosis of depression, with a 6-month evolution with worsening, admission due to autolytic attempt and, at the same time, atypical neurological symptomatology. She is referred to Neurology to rule out organic pathology, including autoimmune pathologies (antiNMDA encephalitis, …), finding in the  study, PCR positive Tropheryma Whipplei, and being diagnosed of Whipple disease with neurological involvement. Antibiotic treatment was performed according to the established guidelines, repeated at the end of the treatment the lumbar puncture, resulting this negative and with clinical neurological improvement  as well as psychiatric.

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Oclusión Endovascular de Arteria Carótida Interna Aberrante Como Causa de Tinnitus Pulsátil. Endovascular Occlusion Of Aberrant Internal Carotid Artery Causing Tinnitus.

We present a case of a 43-years old male patient with a history of chronic and intractable pulsatile tinnitus due to an aberrant course of the right internal carotid artery. In complementary studies, the angiography showed a preserved Willis’ arterial polygon and a balloon-occlusion test with adequate patency and no neurological symptoms. We performed the endovascular occlusion of the right internal carotid artery in the petrous and lacerum portions with the relief of symptoms.

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Presentación de Mieloma Múltiple en Sistema Nervioso Central. Multiple Myeloma Presentation In Central Nervous System.

Multiple myeloma is a monoclonal gammopathy with proliferation of plasma cells. Its form of presentation is hypercalcemia, osteolytic lesions, anemia and renal failure. The involvement of the central nervous system is rare. It occurs approximately in 1% of patients, whose diagnosis is made through the study of cerebrospinal fluid, with the presence of plasma cells. There is no specific treatment due to the low incidence. But there have been good results with intrathecal treatment.

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Dolor en Síndrome de Guillain Barré: Una Manifestación Subestimada. Pain In Guillain Barré Syndrome: An Underestimated Manifestation.

Guillain-Barré syndrome is an acute demyelinating polyneuropathy that presents clinically with muscular weakness and autonomic disorders in its typical form, while the sensory symptoms usually go unnoticed.

We describe the clinical history of a male patient with acute flaccid quadriparesis and sensory disorders such as paresthesia and allodynia that hinder diagnosis within the initial approach.

Complete diagnostic work up confirmed the presence of Guillain-Barré syndrome. Although the sensory symptoms confused the diagnosis during admission, it is important to highlight that pain is a frequent manifestation of this disorder, being underestimated in many patients.

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Fibrodisplasia Osificante Progresiva: Reporte de Casos. Progressive Ossifying Fibrodysplasia: Case Reports.

We report two cases with diagnosis of progressive ossifying fibrodysplasia (FOP) at the “Eugenio Espejo” Specialty Hospital. The FOP is a rare autosomal dominant disease. In most of the patients is due to a mutation in families not affected previously. It’s characterized by progressive ossification of connective tissue, aponeurosis, fascia, ligaments, tendons and skeletal muscle. The early diagnosis improves the prognosis and the quality of life.

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Miastenia Gravis de Presentación Bulbar: Reporte de Caso y Revisión de Literatura. Myasthenia Gravis Of Bulbar Onset: Case Report And Review Of Literature.

Myasthenia gravis is a neuromuscular autoimmune disorder in which the predominant symptom is muscle weakness that fluctuates during the day and mainly affects the ocular and bulbar muscles. There is an intimate relationship between thymus abnormalities, the extirpation allows in many cases a better control of the disease and as long as they are resectable it must be part of the initial treatment. We present a case of a patient with myasthenia gravis and thymoma in whom the initial treatment was surgical.

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Síndrome de Parry Romberg y Epilepsia Refractaria al Tratamiento. Parry Romberg Syndrome And Pharmacoresistant Epilepsy.

Parry Romberg syndrome is an idiopathic disease, characterized by subcutaneous tissue atrophy. Its main clinical sign is progressive facial hemiatrophy including: skin, subcutaneous tissue, nerves, muscle and/or bone. Several theories have been mentioned, among them the possible hereditary and genetic origin of this disorder, as the manifestation of this disease sometimes is seen in members of the same family. We present a case: a patient with late presentation Parry Romberg, with right side face atrophy associated with refractory epilepsy and family history of a brother who presents right side face atrophy as well and a marked scleroderma in coup of saber.

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