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

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Mielopatía en enfermedad por descompresión. Myelopathy in decompression sickness.

Decompression sickness (DCS) refers to the clinical manifestations caused by the formation of bubbles of an inert gas in
the tissues. It is associated with changes in environmental pressure during diving, and injuries to the central nervous system in the disease are not uncommon. We present a case of severe thoracic spinal cord injury in DCS in a diver.

We highlight the typical aspects of DCS in the case for easy recognition by non-specialist physicians. Prevention and prompt initiation of recompression therapy are the cornerstones of DCS management.

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Palabras clave: buceo, enfermedad por descompresión (ED), Mielopatía,

Keywords: decompression sickness, myelopathy, scuba diving,

Análisis neuropsicológico en un caso de Afasia Global. Neuropsychological analysis in a case of Global Aphasia.

Acquired brain damage generates a series of neuropsychological alterations, among which we can highlight aphasia. This syndrome is characterized by language impairment, with its main features being deficits in fluency, comprehension, repetition, naming, reading, writing and calculation. In this article we present the case of a patient suffering from global aphasia, who presents severe linguistic alterations. The state of the disease and its neuropsychological condition are analyzed. Finally, we close this analysis, highlighting the need for a correct neuropsychological approach at the level of diagnosis and rehabilitation which can help the patient.

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Palabras clave: afasia, Daño cerebral adquirido, estudio de caso, neuropsicología,

Keywords: Acquired brain injury, aphasia, case study, neuropsychology,

Encefalomielitis aguda diseminada tras infección por herpes virus. Acute disseminated encephalomyelitis after herpes virus infection.

Acute disseminated encephalomyelitis (ADEM) is a rapid inflammatory and demyelinating process of the central nervous
system, mainly affecting the white matter, but also the gray matter. It usually occurs after viral infections or vaccinations. The case of a 26-year-old patient with sensory symptoms and weakness in the left extremities is presented. Tests showed brain and cerebellar lesions with possible demyelination. ADEM is suspected due to a history of herpes zoster. Corticosteroids are administered with satisfactory evolution. ADEM is a serious process that can affect children and adolescents and presents MRI findings similar to Multiple Sclerosis. The diagnosis is exclusionary, the treatment is with corticosteroids, but it can leave sequelae.

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Palabras clave: Adulto, Encefalomielitis, Polirradiculoneuropatía,

Keywords: Adult, Encephalomyelitis, polyradiculoneuropathy,

Neurodegeneración con acumulación de hierro en el cerebro: Primer reporte de caso en Ecuador. Neurodegeneration with brain iron accumulation: First case report in Ecuador

Neurodegeneration with brain iron accumulation is a rare group of neurodegenerative diseases that present extrapyramidal symptoms, mood disorders, and cognitive impairment. A prevalence of 1 to 2 cases per million inhabitants is estimated. In Latin America, there is no estimated prevalence, although there are some reported cases with typical presentation. The first case report of this disease in Ecuador is presented, in an atypical presentation, under the ruling out of differentials and compliance with diagnostic criteria.

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Palabras clave: Acumulación cerebral de hierro, Neurodegeneración,

Keywords: Brain iron accumulation, Neurodegeneration,

Síndrome de Millard-Gubler en un paciente joven: diagnóstico y neurorrehabilitación temprana. Millard-Gubler syndrome in a young patient: diagnosis and early neurorehabilitation.

Introduction: Millard-Gubler syndrome (MGS) is a rare neurological entity that affects the brainstem by multiple etiologies. The diagnosis is clinical and requires confirmation by imaging studies such as CT or MRI. Treatment is generally non-surgical with a rehabilitative approach with functional goals.

Clinical findings: In this case we present a 44-year-old male patient from southwestern Colombia, with a history of arterial hypertension who was admitted to the emergency department of a national reference university hospital with a hypertensive emergency and white organ lesion, presenting a sudden loss of mobility of his left hemibody accompanied by involvement of the VI and VII cranial nerves.

Treatment and outcome: Interventions were initiated by a multidisciplinary team, including the rehabilitation service, making the diagnosis of crossed brainstem syndrome (Millard-Gubler) of hemorrhagic vascular etiology. He required monitoring in the NICU until stabilization of his clinical condition, after which early neurological rehabilitation interventions were performed (physical therapy, occupational therapy, speech therapy, orthotics, psychology and pain), achieving an improvement in symptoms and functionality of the motor and cognitive component before h ospital discharge.

Conclusions: This case reflects the importance of a multidisciplinary team with a physician specializing in physical medicine and rehabilitation to ensure the best possible functional outcome, in addition to reporting the interventions performed in a young patient with a rare neurovascular entity.

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Palabras clave: hemiplejía, Rehabilitación, vascular,

Keywords: hemiplegia, Rehabilitation, vascular,

Síndrome de Debilidad Crónica como Presentación del Síndrome Postpolio. Chronic Weakness Syndrome as a Presentation of Post-polio Syndrome.

Post-polio syndrome (PPS) is characterized by progressive weakness, muscle fatigue, joint pain, and an increase in disability, which occurs decades after an episode of poliomyelitis. The cause of progressive neurological deterioration is unknown; however, the main hypotheses suggest induction of autoimmunity. There are no pathognomonic findings or specific tests that diagnose PPS; therefore, the diagnosis is made based on the clinic and the history of a polio episode at least 15 years before the onset of new symptoms. We present a case of a man, with a history of poliomyelitis who approximately 40 years later presents a slow progression of weakness, areflexia, atrophy, and fasciculation. Although the frequency of PPS is low, symptoms are disabling, and they cause great impairment in the patient’s functionality. Since there is no specific treatment, the management of PPS is mainly symptomatic and supportive.

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Palabras clave: debilidad muscular progresiva, fatiga muscular, motoneurona inferior, poliomielitis, Síndrome Postpolio,

Keywords: lower motor neuron, muscle fatigue, poliomyelitis, Post-polio syndrome, progressive muscle weakness,

Hemorragia en área dependiente de circulación cerebral posterior por síndrome de hiperperfusión tras stenting en arteria carótida interna. Hemorrhage on a dependent area of posterior cerebral circulation due to hyperperfusion syndrome after stenting in internal carotid artery.

Cerebral hyperperfusion syndrome (CHS) after stent placement has a low incidence, between 0.37- 1.9% (1,2). We present a case of CHS due to recanalization in right internal carotid artery (ICA) which, contrary to expectations, produces a hematoma on a dependent area of the posterior cerebral circulation. Through the clinical case, we proceed to delve into the CHS and the characteristics that give the patient a higher risk. In addition, we expose the potential usefulness of the neurosonological study of inter-individual variability for better perioperative management. In this way, we can ensure a good therapeutic approach that minimizes morbidity and mortality as much as possible.

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Palabras clave: Hemorragia intracerebral, Neurorradiología, Síndrome de hiperp erfusión cerebral, Stent, Tálamo,

Keywords: Cerebral hyperperfusion syndrome, Intracerebral hemorrhage, Neuroradiology, Stent, Thalamus,

Trombosis venosa cerebral profunda e infarto venoso, secundario al uso prolongado de anticonceptivos hormonales. Deep Cerebral Vein Thrombosis and venous infarction, secondary to prolonged use of Hormonal Contraceptives.

Introduction: Cerebral venous thrombosis represents an infrequent cause of cerebrovascular disease, but can result in highly lethal complications such as venous infarction. It affects women of childbearing age more frequently, and the use of oral hormonal contraceptives has shown a causal relationship with this pathology.

Clinical case: We present a 28-year-old patient, with a history of using oral hormonal contraceptives for 5 consecutive years, who came to the emergency room complaining of oppressive holocephalic headache, vomiting and diplopia, of 6 days of evolution. Consciousness with a tendency to obtundation, hypobulia, bradylalia, bradypsychia, paresis of the sixth cranial nerve (left) and bilateral Babinski’s sign were appreciated. Funduscopic examination revealed bilateral developed papilledema. Non-contrast computed tomography of the skull shows direct and indirect signs of thrombosis of the deep cerebral venous system involving internal cerebral veins, vein of Galen, and straight sinus. Nuclear magnetic resonance with T2-FLAIR, Diffusion and vascular sequences confirmed the diagnosis, as well as the presence of infarction in the corresponding venous territory.. Anticoagulant therapy with low molecular weight heparin was performed for ten days and anticoagulation with warfarin was maintained for three months. The causative agent is discontinued. The patient evolved favorably and to date there have been no re currences of the thrombotic event.

Conclusions: Cerebral venous thrombosis shows a worse prognosis when it involves the deep venous system; Its evolution also depends on early diagnosis and timely treatment.

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Palabras clave: Anticonceptivos Hormonales, Infarto venoso, Seno recto, Trombosis del Sistema Venoso Cerebral Profundo, Vena de Galeno, Venas cerebrales internas,

Keywords: Galen vein, Hormonal Contraceptives, Internal cerebral veins, Straight sinus, Thrombosis of the Deep Cerebral Venous System, Venous infarction,

Síndrome de Tolosa-Hunt con compromiso de tres ramas del nervio trigémino y nervio facial. Tolosa-Hunt Syndrome with involvement of three branches of the trigeminal nerve and facial nerve

Introduction: Tolosa-Hunt Syndrome consists of a sensory neuropathy of the trigeminal nerve associated with the paralysis of one or more ocular motor nerves, secondary to an idiopathic granulomatous inflammation of the cavernous sinus, the superior orbital fissure or the orbit.

Clinical case: 19-year-old patient with Tolosa-Hunt Syndrome accompanied by peripheral facial paralysis, with enhancement of the facial nerve.

Conclusions: Compromise of trigeminal nerve branches and facial nerve is a rare finding. Imaging studies contribute to the characterization of the etiology. Steroid treatment continues to be effective.

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Palabras clave: Desórdenes motores oculares, Oftalmoplejía (Descriptores DeSC/MeSH), Parálisis facial, Síndrome de Tolosa-Hunt, Tejido de granulación,

Keywords: Facial paralysis, Granulation Tissue, Ocular Motility Disorders, Ophthalmoplegia (Medical Subject Headings DeSC/MeSH), Tolosa-Hunt syndrome,

Complicaciones neurológicas post-vacuna COVID. Neurological complications post-COVID vaccine.

Post-vaccine SARS-CoV-2 neurological complications are rare. However, due to the SARS-CoV-2 pandemic, massive vaccination has been carried out worldwide, which is why we have seen a greater report of post-vaccination neurological adverse effects.
We present 4 cases, which after receiving the SARS-CoV-2 vaccine presented 4 different neurological pathologies at the Hospital Comarcal de Vinaroz during the year 2021.
A relationship between vaccination and the onset of neurological symptoms is observed. Three patients presented clinical manifestations in relation to the BNT162b2 vaccine. The first patient after administration of the first dose of the BNT162b2 vaccine presented diplopia and palpebral ptosis, a condition compatible with ocular Myasthenia gravis. The second patient had symptoms of acute sensory motor axonal polyneuropathy after the third dose. The third patient, who after the third dose of BNT162b2 presented lethargic encephalopathy. The fourth patient presented symptoms compatible with aseptic meningitis after AZD1222 and mRNA-1273 vaccination.
Due to the worldwide mass vaccination campaign, reports of accidentally related or causally linked neurological complications are emerging. These are very varied; they could be related to immunological and/or toxic mechanisms. Physicians must be aware of these possible adverse effects and rule out other causes. Studies should be carried out that allow us to clarify the pathological mechanisms in relation to post-vaccination complic ations.

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Palabras clave: Encefalopatía, meningitis, miastenia, polineuropatía, SARS-CoV-2,

Keywords: Encephalopathy, meningitis, myasthenia, polyneuropathy, SARS-CoV-2,

Síndrome de Fahr e hipoparatiroidismo. Fahr syndrome and hypoparathyroidism.

Introduction: Fahr’s disease is a rare pathology characterized by brain calcinosis syndrome, usually found incidentally and late in individuals with neurological and psychiatric symptoms, with these manifestations from an early age. It is a genetic disorder of unclear etiology of autosomal dominant behavior and can be a ssociated with metabolic, hereditary, or acquired etiology.
Objective: To present the case of a patient with Fahr’s disease treated in a second-level regional hospital in a rural area of central Andean Colombia.
Clinical case: This is a 54-year-old woman who consulted for the first seizure episode, with a significant pathological history of schizophrenia and a family history of maternal Alzheimer’s. Simple cerebral computed tomography was performed, which reported multiple periventricular calcifications and symmetric calcifications at the level of the basal ganglia with hypocalcemia and hypoparathyroidism.
Conclusions: Fahr’s disease is a rare entity, however, it is important to suspect and know about it early; Diagnostic images are essential for detection, and the search for differential diagnoses or associated metabolic causes is important for treating these patients in a timely and adequate manner.

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Palabras clave: calcificaciones, Convulsiones, hipoparatiroidismo, reporte de caso, Síndrome de Fahr,

Keywords: calcifications, case report, Fahr's disease, hypoparathyroidism, Seizures,

Análisis neuropsicológico de un caso de daño talámico. ¿Por qué el paciente presenta una nueva holgazanería y una ingenuidad adquirida?. Neuropsychological analysis of a case of thalamic damage. Why does he present a new laziness and acquired naiveté?.

Acquired brain damage is a topic of central interest in neuropsychology, since it allows us to understand the relationship between cognition, behavior and emotion with pathological brain functioning. On this occasion, we present a case with a cerebrovascular disease that, as a result of damage at the thalamic level, presents executive, emotional, linguistic, and memory disorders. This is a 52-year-old patient who presents signs and symptoms such as personal insouciance, lack of motivation to seek employment, problems regulating his behavior, among others. We analyze the case regarding the role of the thalamus in global brain functioning and the need to carry out a correct follow-up with the patient.

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Palabras clave: Daño cerebral adquirido, disfunción ejecutiva, enfermedad cerebrovascular, neuropsicología, Tálamo,

Keywords: Acquired brain injury, Cerebrovascular disease, executive dysfunction, neuropsychology, Thalamus,

Accidente cerebrovascular en un paciente con meningitis por Streptococcus Constellatus. Stroke in a patient with Streptococcus Constellatus meningitis.

Streptococcus Constellatus ssp pharingi belongs to the group of Streptococcus Anginosus, it constitutes normal flora of th respiratory, gastrointestinal and genitourinary tracts, it infrequently produces localized or disseminated infections in patients with risk factors. The case of a 41-year-old male patient is reported, with a history of pituitary macroadenoma resection in 2003, undergoing treatment, who consulted the emergency department for 4 days with intense holocranial headache, fever, emesis, associated with temporospatial disorientation and weakness in lower limbs in the last 24 hours. He required management in the intensive care unit, mechanical ventilation, vasopressor support, and tracheostomy, due to respiratory and hemodynamic deterioration. In extension studies, meningitis due to Streptococcus Constellatus ssp pharingi was documented in the presence of sinusitis, cerebrospinal fluid fistula and multi-infarct cerebral vascular compromise, product of vasculitis of infectious origin, a severe manifestation of
bacterial meningitis, not previously reported by this microorganism.

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Palabras clave: Accidente Cerebrovascular, Meningitis bacteriana, Streptococcus Constellatus, Vasculitis del sistema nervioso central,

Keywords: Bacterial meningitis, Central nervous system, Streptococcus constellatus, stroke, vasculitis,

Hemorragia en área dependiente de circulación cerebral posterior por síndrome de hiperperfusión tras stenting en arteria carótida interna. Hemorrhage on a dependent area of posterior cerebral circulation due to hyperperfusion syndrome after stenting in internal carotid artery

Cerebral hyperperfusion syndrome (CHS) after stent placement has a low incidence, between 0.37- 1.9% (1,2). We present a case of CHS due to recanalization in right internal carotid artery (ICA) which, contrary to expectations, produces a hematoma on a dependent area of the posterior cerebral circulation. Through the clinical case, we proceed to delve into the CHS and the characteristics that give the patient a higher risk. In addition, we expose the potential usefulness of the neurosonological study of inter-individual variability for better perioperative management. In this way, we can ensure a good therapeutic approach that minimizes morbidity and mortality as much as possible.

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Palabras clave: Hemorragia intracerebral, Neurorradiología, Síndrome de hiperperfusión cerebral, Stent, Tálamo,

Keywords: Cerebral hyperperfusion syndrome, Intracerebral hemorrhage, Neuroradiology, Stent, Thalamus,

Neuropatía craneal múltiple: Posible asociación a infección por HTLV 1. Multiple cranial neuropathy: Possible association with HTLV 1 infection

Human lymphotropic virus type 1 (HTLV 1) is an oncoretrovirus that chronically and persistently infects T lymphocytes; which can cause serious neoplastic or inflammatory diseases, however, most are asymptomatic and only a small number of patients develop the disease. Although this virus particularly affects the hematological system in the form of T-cell leukemia or lymphoma, the nervous system is also an important target, both the central and peripheral components. In recent years, different associations have been found between this viral infection and multiple neuroinflammatory pathologies such as encephalitis, motor neuron diseases, polyneuropathies, cranial neuropathies and even myopathies, enriching the syndromic spectrum of this entity. On this occasion, the clinical particularities of a rare and possible association between HTLV 1 and multiple cranial neuropathy expressed in a patient managed in a tertiary care hospital in Cali, Colom bia will be described.

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Palabras clave: HTLV-1, Neuropatía craneal múltiple, paraparesia espástica tropical,

Keywords: HTLV-1, multiple cranial neuropathy, tropical spastic paraparesis,

Síndrome de cabeza caída en Miastenia gravis tras una infección por SARS-CoV2. Dropped head syndrome in Myasthenia gravis after a SARS‐CoV2 infection

Objective: To report a case with an unusual presentation of myasthenia gravis after a severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and literature review.

Method: We report a case of a patient with head drop and a postsynaptic myasthenic syndrome after a SARS-CoV-2 infection and a retrospective review of the literature of all the reported cases of myasthenia gravis de novo associated with SARS-CoV2 infection from march-2020 to september-2022. Written informed consent was obtained from the patient for publication of this case report and accompanying images or videos.

Conclusion: Myasthenia gravis could also be the result of an autoimmune re action triggered by SARS-CoV-2 infection.

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Palabras clave: autoinmune, cabeza caída, Miastenia gravis, SARS-CoV2,

Keywords: autoimmune, dropped head, Myasthenia gravis, SARS-CoV-2,

Rehabilitación neuropsicológica de la memoria de trabajo sobre la conducta ejecutiva y el rendimiento académico en un niño con trastorno específico del aprendizaje. Neuropsychological rehabilitation of working memory on executive behavior and academic performance in a child with specific learning disorder

Introduction: Learning disorders are a clinical condition that manifests itself at school age, affecting the cognitive, social and emotional functioning of the individuals who suffer from it. There are studies that show a positive effect of interventions on executive functioning, impacting the executive behaviors and ac ademic performance of children.

Objective: To assess the effect of a neuropsychological rehabilitation program for working memory on executive behavior and academic performance in a child with specific learning disor der.

Clinical case: An 11-year-old boy, diagnosed with a specific learning disorder according to the DSM-5, a working memory rehabilitation program was designed and applied and its impact on executive behaviors and academic performance was measured.

Results: The measurements of the treatment phase show a downward trend reflecting a positive impact. For the global index of executive function, the percentage of data that exceeds the median (PEM= 100%) indicates a very effective treatment and the non-overlapping of all pairs (NAP=89%) indicates a medium effect of the intervention.

Discussion: The results suggest a positive impact at the level of cognitive, behavioral and emotional functioning of the patient, reflected in executive behavior and academic performance.

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Palabras clave: Conductas ejecutivas, Memoria de Trabajo, rehabilitación neuropsicológica, Trastornos Específicos del Aprendizaje,

Keywords: executive behaviors, neuropsychological rehabilitation, Specific Learning Disorders, working memory,

Embolismo gaseoso cerebral: a propósito de un caso de ictus masivo. Cerebral air embolism: about a case of massive stroke

Introduction: Cerebral air embolism is a rare but potentially fatal entity, that occurs due to the entry of air into the cerebral blood circulation and is caused by multiple causes, including t hose associated with medical procedures.

Clinical case: We present the case of an 81-year-old male patient who, after removal of the high-flow hemodialysis catheter, begins with a deterioration of the level of consciousness, which worsens progressively. Multiple hypodense lesions, compatible with gas embolism, are evidenced in the cerebral tomography. Due to his slow evolution, he was transferred to the intensive care unit and died a few hours later.

Conclusion: It is essential to know the preventive measures to avoid this complication and the general and specific measures to adopt when it occurs.

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Palabras clave: Accidente Cerebrovascular, Diálisis Renal, Embolia Aérea,

Keywords: Air Embolism, Renal Dialysis, stroke,

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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Palabras clave: adulto mayor, Esquizofrenia tardía, Hemorragia Subaracnoidea, Psicosis orgánica, Psiquiatría geriátrica,

Keywords: elderly, Geriatric psychiatry, Late-onset schizophrenia, Organic psychosis, Subarachnoid Hemorrhage,

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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Palabras clave: arteriografía, cerebelo, embolia, Fibrilación auricular, infarto cerebral (DeCS),

Keywords: angiography, atrial fibrillation, cerebellum, cerebral infarction (MeSH), embolism,

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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Palabras clave: Calcificaciones ganglio basales idiopáticas, calcinosis estriado-pálido-dentada, déficit cognitivo, enfermedad de Fahr, enfermedad ganglio basal, hipoparatiroidismo, pediatría,

Keywords: basal ganglia disease, cognitive deficit, Fahr's disease, hypoparathyroidism, Idiopathic basal ganglia calcifications, pediatrics, strio-pallido-dentate calcinosis,

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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Palabras clave: Ataxia, encefalitis, IgLON5 proteína humana, parasomnias, polirradiculoneuropatía inflamatoria desmielinizante crónica, proteína tau,

Keywords: ataxia, chronic inflammatory demyelinating polyradiculoneuropathy, encephalitis, IgLON5 human protein, parasomnias, tau protein,

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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Palabras clave: Degeneración hepatolenticular, neuropsiquiatría, rigidez muscular,

Keywords: Hepatolenticular Degeneration, muscle rigidity, neuropsychiatry,

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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Palabras clave: Cirugía de epilepsia, displasia cortical focal tipo IIIc, epilepsia farmacorresistente, malformaciones del desarrollo cortical, meningioangiomatosis esporádica,

Keywords: drug-resistant epilepsy, Epilepsy Surgery, focal cortical dysplasia type IIIc, malformations of cortical development, sporadic meningioangiomatosis,

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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Palabras clave: Ataxia, CLIPPERS, médula espinal, metrotexato (DeCS), tallo cerebral,

Keywords: ataxia, brainstem, CLIPPERS, methotrexate (MeSH)s, spinal cord,

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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Palabras clave: Ictus isquémico, Infección por SARS-CoV-2, trombectomía mecánica,

Keywords: ischemic stroke, mechanical thrombectomy, SARS-CoV-2 infection,

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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Palabras clave: ganglios basales, Hiponatremia, Mielinólisis pontino central,

Keywords: basal ganglia, Central pontine myelinolysis, Hyponatremia,

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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Palabras clave: ingesta de alcohol, Necrosis palidal, Parkinsonismo palidal,

Keywords: alcohol intake, Pallidal necrosis, Pallidal Parkinsonism,

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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Palabras clave: estado protrombótico, hemorragia intracraneal, SARS-CoV-2,

Keywords: intracranial hemorrhage, prothrombotic state, SARS-Cov2,

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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Palabras clave: conciencia, electrolitos, intoxicación por litio, litemia, litio,

Keywords: awareness, electrolytes, litemia, lithium, lithium poisoning,
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