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

 

Psicosis orgánica, tipo trastorno esquizofreniforme, posterior a hemorragia subaracnoidea. Organic psychosis, schizophreniform disorder type, after subarachnoid hemorrhage

Organic psychosis refers to a group of diseases currently classified as “mental disorders due to a medical illness”, unlike the absence of specific organic causality that exists in primary disorders. These are secondary to long-term processes , which is necessary to re-evaluate frequently due to the association between age-related deterioration, comorbidity and the evolution of psychosis itself. We present the case of a patient with subarachnoid hemorrhage secondary to a ruptured arteriovenous malformation, who suffered from a slight change in behavior and decreased cognitive functions with subsequent evolution to dissociative symptoms, episodes of hypersomnia and disconnection from the environment, through assessment joint of specialties was reached the diagnosis of organic psychosis type schizophreniform disorder.

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Infarto cerebeloso bilateral simultáneo en un paciente con fibrilación auricular paroxística no valvular: una presentación atípica. Simultaneous bilateral cerebellar infarction in a patient with paroxysmal atrial fibrillation: an atypical presentation

Introduction: Cerebellar infarcts usually occur in the territory of the posterior inferior cerebellar artery (PICA) and tend to be unilateral. Simultaneous bilateral involvement is extremely rare.

Case report: We present the case of a 67-year-old male who developed an acute cerebellar syndrome secondary to acute infarction in the territory of both PICA confirmed by nuclear magnetic resonance imaging. Non-valvular paroxysmal atrial fibrillation was the most plausible etiology after digital cerebral angiography ruled out vascular abnormalities.

Conclusion: This case shows that bilateral infarction in the territory of both PICA can occur in the context of cardiac embolism, even in the absence of an anomalous common PICA.

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Calcificaciones ganglio basales simétricas bilaterales (Enfermedad de Fahr) en pediatría: Reporte de caso. Bilateral symmetric basal ganglia calcifications (Fahr’s disease) in pediatrics: Case report

Introduction: Fahr syndrome is defined as the presence of predominantly bilateral symmetric calcifications in the basal ganglia (mainly striated nucleus). It is considered primary when the etiology is genetic, which is also known as Fahr’s disease; and secondary if metabolic alterations, endocrine alterations, exposure to radiation, infections, vascular disorders, mitochondrial diseases or others, are found, being the most frequent: hypoparathyroidism and pseudo-hypoparathyroidism. Fahr’s disease is a pathology of genetic origin, rare in pediatrics, that is associated with movement, neuropsychiatric and cognitive disorders. 

Clincal case: We present the case of a 9-year-old boy with evidence of bilateral symmetric basal ganglia calcifications on brain computed tomography (CT) scanning. In the literature there are very few descriptions of this finding in pediatrics. Clinically, the child presented WITH epilepsy, right hand dystonia, mild cognitive deficit and progressive impairment of speech and gait. He was clinically diagnosed as a Fahr’s disease, given the abscense of other metabolic or endocrinological alterations, without genetic studies, and treatment with Levodopa-carbidopa was started for the management of dystonia with clincal evidence of improvement, an a comprehensive rehabilitation program was indicated.

Conclusions: The finding of bilateral symmetric basal ganglion calcifications should lead to rule out endocrinological or metabolic alterations; In the event that they do not occur, all possible related manifestations in the cognitive, psychiatric, motor, speech and other fields should be handled in a comprehensive manner.

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Encefalitis autoinmune por anticuerpos anti Iglon5, reporte de caso clínico. Autoinmune encephalitis due to anti Iglon5 antibodies, clinical case report

Introduction: New clinical entities related to the discovery of autoantibodies either against intracellular antigens or against cell membrane surface receptors or synapses are being identified with increasing frequency. In the latter group, patients with apnea have been described in recent years, REM and NON-REM sleep behavior disorder, respiratory failure and presence in serum and CSF of antibodies against the LON5 antigen. These antibodies appear to alter the interaction with the cytoskeletal system of the neuron and induce accumulation of TAU, conditioning a wide clinical expression.

Clinical case: 76-year-old male patient with eight years evolution of symptoms before diagnosis. He had a mixture of respiratory symptoms, parasomnias, ataxia, movement disorder, autonomic symptoms, cognitive and mood disorder, and associated a chronic demyelinating polyneuropathy. The diagnosis was confirmed by detection of antibodies against IGLON5 in cerebrospinal fluid.

Conclusion: Anti-IGLON5 antibody encephalitis is a unique entity that challenges what we know about autoimmunity and neurodegeneration, leading to a diagnostic challenge due to the wide expression of symptoms and the possibility of improvement with early treatment.

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Enfermedad de Wilson: A propósito de un caso neuropsiquiátrico de diagnóstico tardío. Wilson’s Disease: About a late-diagnosed neuropsychiatric case

The comprehensive assessment of a psychiatric case requires evaluating the symptoms from a comprehensive perspective, without bias, and suspecting unlikely pathologies where appropriate. We emphasize this from a case of Wilson’s disease in which, as is frequent, the diagnosis was late. Clinical case: 36 year-old woman who from the age of 18 presented depressive and later psychotic symptoms, and her diagnosis was paranoid schizophrenia. The three-year diagnostic delay led to a poor evolution with permanent disabilities. The biased diagnostic attribution about parkinsonism as secondary to antipsychotics clouded the adequate diagnostic evaluation. Our case shows the need for a comprehensive neuropsychiatric perspective in the diagnostic evaluation. In a first case of psychosis, Wilson’s disease should be considered within the differential diagnosis.

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Meningioangiomatosis y displasia cortical focal. Meningioangiomatosis and focal cortical dysplasia

The association between meningioangiomatosis (MA) with focal cortical dysplasia (FCD) has been scarcely published. We present the case of 15-year-old adolescent suffering 10 years evolving drug-resistant epilepsy without history of neurofibromatosis. Magnetic Resonance Image showed an increase in the volume of the hippocampus and the right parahippocampal region. The lesion was considered as a possible tumor. A right temporal lobectomy, guided by trans-surgical electrocorticography (EcoG) was performed. Histology of the resected tissue evidenced a FCD type IIIc (MA mainly vascular associated to FCD). The patient has been seizure free (according to the Engel IA scale) after 4 years of post-surgical evolution. When MA is suspected, we recommend trans-surgical ECoG considering the possible association with FCD in the surrounding neocortex. It could increase the incidence and knowledge about these two lesions. The histological study provides the definitive diagnosis.

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CLIPPERS (Inflamación crónica linfocítica con realce perivascular pontino y respuesta a esteroides) con compromiso longitudinalmente extenso de la médula espinal. Una presentación atípica. CLIPPERS (Chronic lymphocytic inflammation with pontine perivascular enhancement and steroid response) with longitudinally extensive spinal cord involvement. An atypical presentation

CLIPPERS (Chronic Lymphocytic Inflammation with Perivascular Pontine Enhancement and Steroid Response) is a recently described neuroinflammatory disorder in which symptoms involving the brainstem predominate, with variable pattern on neuroimaging and a perivascular infiltrate of T lymphocytes on brain biopsy. We present a case of a 50-year-old male who presented symptoms of asthenia, adynamia, weakness in lower limbs extremities, sphincter disorder and ataxia. Patient was extensively studied to rule out infectious, neoplastic, autoimmune, and demyelinating conditions. Diagnosis was made taking into account significant clinical-imaging elements, remarking the spinal cord involvement in this patient. Treatment with steroids and steroid-sparing agents, such as methotrexate, was found to be effective. Very few cases in the literature to date describe CLIPPERS-associated spinal cord involvement.

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Trombectomía mecánica eficaz en paciente con ictus e infección por SARS-Cov-2 con desenlace fatal. Effective mechanical thrombectomy in a patient with stroke and SARS-Cov-2 infection with fatal outcome

Ischemic stroke has been reported in patients with SARS-CoV-2 infection. It is not clear if COVID-19 is causal or simply coexists or triggers the onset of stroke. Stroke is relatively rare in the context of COVID-19 and mostly occurs in the elderly with vascular risk factors. The underlying mechanism of stroke is multiple. We present an 84-year-old male with a stroke due to large vessel occlusion coincident with severe COVID-19 infection, that despite an initial successful mechanical thrombectomy, had a fatal outcome due to respiratory complications and contralateral massive cerebral infarction due to early recurrence. Consequently, vigilance in this type of patients should be extreme since ischemic stroke with active SARS-CoV-2 infection may have a poor prognosis.

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Neuroimagen en síndrome de desmielinización osmótica. Neuroimaging in asmotic demyelination syndrome

The osmotic demyelination syndrome includes pontine and extrapontine myelinolysis, the first being its classic form of presentation. Usually related to rapid corrections of hyponatremia (serum sodium less than 135mEq/L), it has been described in multiple conditions that can alter plasma osmolality. The symptoms are varied and include spastic tetra paresis, pseudobulbar paralysis, coma, seizures or the characteristic “locked in” syndrome, as well as behavioral disorders such as lack of impulse control, aggressiveness, depressive syndromes, catatonia, mutism, and emotional lability. Years ago, it was a very difficult entity to diagnose, but with the introduction of magnetic resonance imaging, many oligosymptomatic or asymptomatic cases have been detected, although there is no known treatment, spontaneous recovery can occur.

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Bilateral necrosis of the globus pallidus after binge-drinking. Necrosis bilateral de globos pálidos luego de ingesta excesiva y sostenida de alcohol

A 77-year-old man developed progressive slurry speech, action tremor in both upper limbs, generalized bradykinesia, and unsteady and shuffling gait several weeks after heavy binge-drinking followed by an unconsciousness state in which the patient did not respond to verbal commands or painful stimuli. MRI disclosed bilateral and symmetrical necrosis of globus pallidus as well as severe cortical and hippocampal atrophy. Damage of the globus pallidus was likely related to respiratory acidosis during the event. Pallidal Parkinsonism should be considered in the differential diagnosis of a progressive movement disorder after an acute toxic or hypoxic insult to the nervous system.

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Hemorragia intracerebral espontánea en pacientes con Covid-19 severa: Reporte de dos casos. Spontaneous Intracerebral Hemorrhage in severe Covid-19 patients: Report of two cases

The respiratory system is mainly affected by the SARS-CoV-2 infection, producing a severe acute respiratory syndrome known as COVID-19 (Coronavirus disease 2019), patients with severe disease usually develop multiorgan failure; among these we can focus on the nervous system, due to its potential neurotropism. Recent clinical data reveal that patients may manifest symptoms such as anosmia, dysgeusia, impaired consciousness, headache, seizures, and cerebrovascular disease (CVD). We describe two physically active male patients with complicated SARS-CoV-2 infection without significant comorbidities related to the development of intracranial hemorrhage, nor a history of head trauma or documented anatomic malformations. Both were admitted by the emergency department and during their stay in the ICU they developed intracranial hemorrhage diagnosed by computed tomography. The paraclinical findings in the two cases were compatible with a prothrombotic state as possible etiologies of bleeding in both.

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Alteración del estado de conciencia secundario a intoxicación por litio. Altered state of consciousness secondary to lithium intoxication

Introduction: Lithium is a pharmacological molecule that is commonly used in patients with bipolar affective disorder. Due to its chemical similarity to sodium, alterations in the latter have a direct effect on its plasma concentrations. Among the complications that can occur are nephrogenic diabetes insipidus, thyroid dysfunction, and some central nervous system defects such as nystagmus, ataxia, tremor, fasciculations, seizures, and coma.

Objective: To present the case of a patient with a syndrome of altered state of consciousness secondary to lithium poisoning coincide.

Clinical case: 61-year-old woman with a history of bipolar affective disorder who presented the syndrome of altered state of consciousness associated with lithium poisoning, for which reason the drug was withdrawn and hemodialysis sessions were carried out, obtaining an adequate response. with improvement of renal function and normalization of serum lithium values (0.76 mmol / L).

Conclusions: Lithium may involve alterations at the renal level that are associated with modifications in serum electrolytes. This can facilitate the appearance of neurological symptoms or symptoms, as evidence in the present case.

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Canalopatía de calcio CACNA1E: Nueva encefalopatía epiléptica en un paciente pediátrico. CACNA1E calcium channelopathy: New epileptic encephalopathy in a pediatric patient

Introduction: Epileptic and developmental encephalopathies, related to voltage-associated calcium channel mutations, are heterogeneous entities recently described in pediatric population.

Objective: To describe the case of pediatric patient with epileptic and developmental encephalopathy caused by a mutation in the CACNA1E gene that codes for a calcium channel.

Clinical case: Preschool male patient with refractory epilepsy starting at two months of life, associated with global developmental delay, behavioral disorder, hyperkinetic movements (dystonia) and hypotonia, with multifocal interictal activity and normal brain resonance, with pathogenic mutation in the gene CACNA1E.

Conclusion: Mutations in the CACNA1E gene cause functional alteration of the calcium channel CaV2.3, originating a clinical picture of a recently described phenotype.

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Aplasia unilateral del nervio vestibulococlear. Unilateral Vestibulocochlear nerve aplasia

Introduction: Vestibulo cochlear aplasia is an embryological defect in the formation of the inner ear that causes a sensorineural hearing loss. We report the case of an 8 year-old boy with left sided hearing loss, not previously detected. On neurological examination, a hearing loss in the left ear was found. Through imaging studies, the diagnosis is confirmed.

Conclusion: When hearing loss is suspected, imaging studies are the ideal method for detecting pathological findings in the inner ear.

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Síndrome de Arlequín. Harlequin syndrome

We herein report the case of a 37-year-old woman who sought medical attention due to facial erythema on the right side of her face as well as diaphoresis following stressful events or exercise. Clinical examination was unremarkable. Blood tests and Thorax CT excluded any structural causes. Given this, she was diagnosed with Harlequin syndrome. Therapeutic options were explained, and follow-up provided.

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Asterixis and dysarthria-clumsy hand originated of lacunar infarction: A series of six cases. Asterixis y disartria-mano torpe originado a partir de los infartos lacunares: Una serie de seis casos

La asterixis y la disartria-mano torpe son signos neurológicos poco frecuentes que pertenecen a los trastornos del movimiento después de un accidente cerebrovascular. Clínicamente ellos son clasificados como parte del infarto lacunar y la mayoría de los casos se resuelven espontáneamente en un periodo entre 10 semanas y un mes. El objetivo de este estudio fue reportar seis casos de pacientes masculinos con infarto lacunar y describir los síntomas, localización y tamaño de las lesiones. Se describen casos de infarto lacunar y síntomas motores leves (disartria y asterixis) sin ningún indicio de demencia. En conclusión, nuestros casos presentan movimientos neurológicos anormales como asterixis y disartria-mano torpe en pacientes con hipertensión y / o diabetes mellitus tipo 2. Debido a la transitoriedad de esos movimientos, el diagnóstico en el tiempo adecuado es importante, a partir de eso los médicos pueden solicitar los exámenes de imagen, tratar al paciente y luego acompañarlo previniendo futuros ictus con consecuencias aún más graves. Así, estudios como el nuestro pueden contribuir al correcto diagnóstico de los infartos lacunares.

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Giant bilateral open-lip schizencephaly. Esquizencefalia bilateral gigante de labio abierto

La esquizencefalia es una malformación cerebral congénita caracterizada por hendiduras en la corteza cerebral, se clasifica en Tipo I (labio abierto) y tipo II (labio cerrado). Los pacientes con esquizencefalia presentan convulsiones, hidrocefalia, déficit motor y mental. La ecografía se utiliza para el diagnóstico intra útero y recién nacidos, y la resonancia magnética o tomografía computarizada en pacientes ya nacidos. El manejo de la esquizencefalia es conservador, con rehabilitacción de los déficits motores o mentales, medicación o cirugía para convulsiones y derivación en hidrocefalia con aumento de la presión intraacraneal. En la literatura, solo se han informado unos pocos casos bilaterales gigantes. Presentamos un caso de esquizencefalia gigante bilateral de labio abierto, en un paciente masculino de 10 días, que se presenta con hipotonía leve y sin convulsiones. Este caso es raro debido a las características relativamente beningnas en comparación con otros casos notificados.

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Aplicación móvil de realidad virtual para el entrenamiento de la mano espástica. Virtual reality mobile application for spastic hand training

Background: The hand is a structure with a complex neuromuscular organization that in hemiparesis is compromised, affecting function.

Aim: To determine the changes in the motor function of the spastic hand of an adult with hemiparesis through the use of a mobile application for virtual reality.

Method: Case report of a 29-year-old adult with hemiparesis, evaluated with the Fugl Meyer upper extremity assessment (FMA-ES), the Trail Making test and the force dynamometry test. A therapeutic intervention was performed with exercises of the least affected hand for 12 sessions using special glasses for a total immersion 20 minutes using mobile application Mirror box Therapy VR®.

Results: The virtual reality therapy applied through the Mirror Box Therapy VR® mobile application, shows in a short time favorable changes in the motor and sensory hand function of the patient.

Discussion: Virtual reality provides a functional interaction, making it an effective tool to motivate patients during sessions.

Conclusion: An intervention through the use of virtual reality generates changes in the motor and sensory function of the hand of a patient with hemiparesis after 12 work sessions.

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Tuberculoma intramedular espinal que simulaba una lesión tumoral, en un paciente con desnutrición y consumo de estupefacientes. Spinal intramedullary tuberculoma that simulates a tumor lesion, in a patient with malnutrition and drug use

Intramedullary spinal tuberculoma is a rare form of extrapulmonary tuberculosis. In most cases the diagnosis is made by biopsy of the lesion and histopathological study. We present the case of a 28-year-old male with a history of malnutrition and drug use who presented progressive paraparesis with sphincter compromise secondary to a dorsal intramedullary tuberculoma. At the beginning a tumor lesion was suspected, however, findings of pulmonary tuberculosis, lymph node biopsy and a positive tuberculin protein derivative study confirmed the clinical diagnosis of spinal tuberculoma.

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Síndrome alcohólico fetal. Reporte de un caso. Fetal alcohol syndrome. Case report

Prenatal exposure to alcohol is one of the main preventable causes of birth defects and developmental disabilities. Fetal alcohol spectrum disorder (FASD) is a term used to describe the physical, behavioral, and neurodevelopmental disorders that can be detected in an individual who was prenatally exposed to alcohol.

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Encefalitis autoinmune. Autoimmune encephalitis

Autoimmune encephalitis is a recently described pathology as inflammatory disease of the central nervous system, previously characterized as a paraneoplastic syndrome. However, advances in technology have allowed to categorize it as an entity, with an specific physiopathology, and a clinical presentation based on the developed auto-antibody, but, the wide clinical presentation, the insidious development of the symptoms, the mimic with other pathologies and the lack of knowledge of this condition in the emergency room and hospitalization services, does not permit to diagnose this condition, leading to potentially fatal complications. We report the case of a 26-years-old female with autoimmune encephalitis, and a literature review regarding this topic.

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Discapacidad asociada a manifestaciones neurológicas de COVID-19. Disability associated with COVID-19’s neurological manifestations

Introduction: COVID-19 is mainly a respiratory illness, however, with an incidence in nearly a third of patients of neurological manifestations secondary to affection to the central or peripheral nervous system, used to be more frequent in severe illness, having a wide range of disability and mortality.

Clinical case: We present the clinical case of a 39 years old female ,previously healthy, with diagnosis of COVID-19, initiating her illness with encephalitis and status epilepticus, posteriorly longitudinally extensive myelitis and anoxic-ischemic encephalopathy, which, despite of opportune diagnosis and treatment, she had a poor outcome developing persistent vegetative state.

Conclusions: COVID-19’s neurological manifestations could lead to a high risk of disability and mortality, needing an opportune detection and treatment, with a high suspicion of encephalitis and status epilepticus in patients with decrease of alertness and focal motor symptoms.

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Estatus epiléptico no convulsivo por Lupus Eritematoso Sistémico y Síndrome Antifosfolípido. Non convulsive status epilepticus due to Systemic Lupus Erythematosus and Anti- phospholipid syndrome

Systemic Lupus Erythematosus is a chronic disease, with the presence of autoantibodies and lymphocyte hyperactivity. The presence of antiphospholipid antibodies is associated with seizures and vasculocerebral disease. We present the case of a patient with atypical absence seizures and mild cognitive impairment.

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Calcificaciones en Ganglios de la Base en un Caso de Hipoparatiroidismo Primario. Basal Ganglia Calcification In A Case Of Primary Hypoparathyroidism

Hypoparathyroidism is characterized by an insufficient production of parathyroid hormone, resulting in hypocalcemia and hyperphosphatemia, that without proper treatment can lead to multiple complications in different organs. We present the case of a patient with primary hypoparathyroidism, with basal ganglia calcifications as a complication of the disease and the therapeutic challenge it presents to improve the symptoms and quality of life.

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Síndrome Lumbocostovertebral Asociado a Mielomeningocele. Lumbo-Costovertebral Syndrome Associated With Myelomeningocele

Lumbo-costo-vertebral syndrome is a rare anomaly that affects the vertebral bodies, the ribs and the muscles of the abdo- minal wall, and multiple additional congenital abnormalities requiring multidisciplinary management and early surgical inter- vention to avoid complications. Case presentation: A 10-day-old male infant presents with two right lumbar tumors, one that increased in size with crying consistent with a lumbar hernia, and the other a myelomeningocele. In complementary studies, it was found that the patient had fusion of the 1st and 2nd, 3rd and 4th and 11th and 12th ribs on the right, a 13th accessory rib on the right, and a 13th and 14th accessory rib on the left in addition to multiple defects in the fusion of the lumbar and sacral vertebral bodies, and a 1cm defect in the abdominal wall with protrusion of abdominal contents. The patient was diagnosed with LCVS with an associated myelomeningocele and underwent a simple closure and repair of the abdominal defect with a bovine pericardial patch, and plasty of the myelomeningocele without complications. We present this case due to the rarity with which it is reported in the literature. Conclusions: LCVS is a rare entity that merits early surgical resolution to avoid complications, in addition to multidisciplinary management.

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Carcinoma de Células de Merkel en Paciente con Esclerosis Múltiple en Tratamiento con Fingolimod. Merkel Cell Carcinoma In A Patient With Multiple Sclerosis Treated With Fingolimod

Fingolimod is a disease-modifying therapy widely used in Relapsing-Remittent Multiple Sclerosis. It blocks the capacity of lymphocytes to leave the lymph nodes, causing lymphopenia. This increases the risk of infections, but also non-melanocytic skin tumours. We report a case of Merkel cell carcinoma in a patient treated with fingolimod and a review of the literature, which helps to understand the relation between immunosuppression, opportunistic infections and cancer.

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Síndrome de Persona Rígida de Causa Paraneoplásica. Claves Diagnósticas en una Entidad Rara. Stiff Person Syndrome Of Paraneoplastic Cause. Diagnostic Keys In A Rare Entity

Introduction: Paraneoplastic disorders exert their effect through immunological mechanisms and not by direct invasion of the tumor; in this way tumors located outside the nervous system induce the production of antibodies against antigens: on the neu- ronal surface, intracellular or synaptic and affect distant sites from its location to either the central or peripheral nervous system, thus producing various clinical manifestations. One of these syndromes is the Stiff Person syndrome, which, apart from being a rare entity, occurs as a paraneoplastic syndrome in only 5% of cases.

Clinical Case: A 72-year-old male patient, a 2-month evolution that began with painful muscle spasms in the dorsal and lumbar region, evolved to rigidity with posture in axial and appendicular hyperextension, pseudospastic gait, hyperekplexia; due to the suspicion of Stiff Person syndrome a diagnostic work-up was begun finding high levels of Ab anti GAD65 and later a Non- Hodgkin’s Lymphoma. The patient received immunotherapy and cancer treatment.

Conclusion: Paraneoplastic syndromes must be identified according to the clinical phenotype and the determination of an- tineuronal antibodies. High suspicion and rapid diagnosis are necessary to achieve timely treatment and avoid permanent injury.

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Síndrome de Joubert Asociado a Apnea Central del Sueño en un Adulto de Colombia. Joubert Syndrome Associated With Central Sleep Apnea In An Adult From Colombia

Introduction: The Joubert syndrome is an autosomal recessive disorder, classified as a ciliopathy. The primary cilia have a role in neuronal proliferation and axonal migration in the cerebellum and brainstem. Marie Joubert and colleagues in 1969 pu- blished four cases with partial or total agenesis of the cerebellar vermis and apnea-hyperpnea neonatal episodic disorder. From an early age the principal manifestations are: abnormal breathing pattern, nystagmus, swallowing alterations, hypotonia, ataxia and intellectual disability. Classically, the neonatal breathing disorder improve with age.

Clinical case: A 39 years old, with intellectual disability and a history of a movement disorder involving a lower extremity, snoring, episodes of central apnea, hypotonia and nystagmus. He was the second child, with an uncomplicated, full-term gestation. In the clinical exam was found dysarthria, short term and semanticmemory loss, Epworth scale: 9/24. Brain magnetic resonance imaging (MRI) showed cerebellar vermis hypoplasia and elongation of the hemispheres as the “molar tooth sign.” Moreover, a polysomnography study found a central sleep apnea disorder.

Discussion: It is fundamental to recognize this syndrome to establish a prenatal or an early age diagnosis, for a multidisciplinary approach and an early genetic counseling. The treatment of the sleep disorder in these patients is crucial for a better quality of life.

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Cómo Reconocer el Meningioma Intraóseo: Etiología y Hallazgos por Imagen. How To Recognize Intraosseous Meningioma: Etiology And Imaging Findings

Meningiomas are the most common primary brain tumors. Primary intraosseous meningioma is a rare extradural meningioma subtype. They are usually asymptomatic but may cause proptosis or neurological symptoms depending on size and location. The most common finding in imaging tests is hyperostosis although a lytic or even mixed pattern can also be observed, so it should be considered in the differential diagnosis of cranial sclerotic bone tumors. Although most are benign, they are more likely to develop malignancy than intradural meningiomas. Imaging techniques (CT and MRI) are very useful in preoperative diagnosis and evaluation of adjacent anatomical structures. Surgical resection followed by cranial reconstruction is the treatment of choice.

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Razonamiento Clínico: Mujer de 38 Años de Edad en Puerperio Alejado con Debilidad Muscular en Hemicara Derecha. Clinical Reasoning: 38-Year-Old Woman In Remote Puerperium With Weakness In Right Side Of The Face

A case of 38-year-old woman is presented with muscular weakness in right side of the face during her remote puerperium. We reviewed the history, physical examination, and analyze the differential diagnosis, etiology and the final diagnosis.

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