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.
Reportes de Casos Clínicos
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.
Leer artículo completo
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.
Leer artículo completo
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.
Leer artículo completo
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.
Leer artículo completo
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.
Leer artículo completo
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.
Leer artículo completo
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.
Leer artículo completo
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.
Leer artículo completo
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.
Leer artículo completo
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.
Leer artículo completo
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.
Leer artículo completo
Desmielinización del Sistema Nervioso Central Asociado a Encefalomielitis Diseminada Aguda: Reporte de un Caso. Demyelination Of Central Nervous System Associated With Acute Disseminated Encephalomyelitis: Case Report.
Inflammatory demyelinating diseases comprise a series of autoimmune disorders affecting myelin at the level of the central nervous system (CNS) and peripheral nervous system. They can be monophasic, multiphasic, progressive, monofocal or multifocal, and their diagnosis is usually of exclusion. We report the case of a 15-year-old female patient with headache, ataxia, hemiparesis, ophthalmoplegia and altered consciousness. Magnetic resonance imaging showed lesions compatible with demyelinating disease. Acute disseminated encephalomyelitis was diagnosed. The patient responded to treatment with corticosteroids.
Leer artículo completo
Síndrome de Locked-In: Reporte de Caso y Revisión de Literatura. Locked-In Syndrome: Case Report And Review Of Literature.
Locked-In Syndrome, also known as Enclaustration, Deseferentization, or Enclosure Syndrome, was first defined in 1966 by Plum and Posner. Causes can be grouped into vascular and non-vascular, the former being the most frequent. Clinically this syndrome is characterized by consciousness and the state of watch conserved, but there is quadriplegia, anartria, dysphagia and difficulty to coordinate the ventilatory mechanics, representing pulmonary complications the main cause of death. In most cases, the patient retains vertical eye mobility, so the only method of communication is through eye blinking and vertical eye movements. Next, a case presentation and review of the literature with the most relevant pathophysiological, clinical, diagnostic and therapeutic aspects is done.
Leer artículo completo
Foramen Oval Permeable, un Diagnóstico Posible en Manos del Neurólogo. Descripción de Caso. Patent Foramen Ovale, A Possible Diagnosis From The Neurologist. Case Report.
Introduction: A high prevalence of atrial septal defects is reported in patients with cryptogenic ischemic stroke, also related to an increase of the risk of recurrence. Objective: To report case of a young patient with a history of diabetes mellitus and ischemic stroke without arterial changes proven. A transcraneal Doppler study with a bubble test helped to diagnose a patent foramen ovale. Conclusion: As part of the initial evaluation of this patient, a TCD study has helped to clarify the stroke etiology. The demonstration of a patent foramen ovale has implications fo r the secondary prevention of stroke.
Leer artículo completo
Hemorragia Talámica Bilateral. Bilateral Thalamic Hemorrhage.
Thalamic hemorrhages represent a small group of cerebrovascular diseases of which only 0.7-3% is manifested bilaterally, being chronic hypertension the key factor for its development. We report a case of a 45-year-old patient with 10-years history of arterial hypertension who presented with brachiocrural motor deterioration accompanied by dysarthria, dyspnea, vomiting in number of two, with increased basal blood pressure. Neurimaging demostrated a bilateral thalamic hematoma.
Leer artículo completo
Evolución Natural de la Siringomielia: Presentación de un Caso. Natural Evolution Of Syringomyelia: Case Report.
Syringomyelia is a chronic degenerative and progressive disease of the spinal cord characterized by a dissociated loss of brachial sensitivity and amyotrophy, with evidence of centromedullary cavitation.
We report the case of a 62-year-old female patient with syringomyelia associated with Arnold Chiari malformation (type 1) who spontaneously rejected any surgical intervention five years after her diagnosis, which has been followed up for 21 years. Magnetic resonance imaging demonstrates the natural evolution of syringomyelia from 1998 to the present.
Leer artículo completo
Síndrome de Encefalopatía Posterior Reversible (PRES). Posterior Reversible Encephalopathy Syndrome.
Síndrome de Guillain-Barré: Un Caso Atípico
Guillain Barre Syndrome (GBS), is an acute inflammatory polyneuropathy, of autoimmune etiology that produces an inflammation in the peripheral nerves and its spinal roots. GBS generally presents as flaccid, symmetric, ascendent and areflexic palsy, along with a previous respiratory or gastrointestinal infection. It has been reported atypical forms of presentation. We describe the case of a 53 years old male patient, without previous infections in the last months, who is admitted to the emergency department presenting walking difficulties due to decreased muscle strength, pain and numbness in the lower limbs, deep tendon reflexes present, followed few days later by a right peripheral facial palsy. Examination of cerebrospinal fluid (CSF) revealed albumino-cytological dissociation, leading to diagnose GBS of atypical presentation, which was treated with intravenous immunoglobulin followed by a favorable outcome.
Leer artículo completo
Parkinsonismo y Mielinolisis Osmótica. Descripción de caso
Introduction: Osmotic demyelination syndrome is a rare medical condition in which parkinsonian signs are relatively non frequent. Objective: To report a case of a patient with hyponatremia rapidly corrected and treated with neuroleptics. The cranial MRI showed demyelinating lesions in pons and basal ganglia. The patient clinically developed parkinsonism, with no response to dopamine. Conclusion: The inadequate correction of hyponatremia causes demyelinating lesions. As we appreciate in this case, the prescription of neuroleptic drugs may negatively affect the response to dopaminergic therapy.
Leer artículo completo
Tuberculous Encephalitis Associated With Basal Ganglia Lesions And Movement Disorders
An increasing number of autoimmune disorders with predominant involvement of the basal ganglia which result in movement disorders and psychiatric symptoms have been described. We report a 26 year old patient who, three weeks after initiation of treatment for tuberculous meningitis, presented with acute right hemichorea-ballism and confusion One week later the patient presented acute left hemiparkinsonism. The CSF showed oligoclonal bands. The MRI showed bilateral lesions in the basal ganglia in the T1W and FLAIR sequences. Antituberculous therapy with concomitant steroids and L dopa treatment ,resulted in clinical improvement .To our knowledge this is the first report of tuberculous encephalopathy associated with involvement of the basal ganglia and movement disorders.This case suggests involvement of the basal ganglia through an immune mediated pathogenesis.
Leer artículo completo
Mielinólisis Extrapontina Secundaria a Hiponatremia por Crisis Adrenal
Introduction: Extrapontine myelinolysis is an acute unusual process, included in demyelinating syndromes of metabolic etiology, which differentiate from central pontine myelinolysis, by affecting other brain areas with clinical manifestation such as dystonia, catatonia, postural tremor, myoclonic spasm, parkinsonism or pyramidal sings.
Objective: Present clinical, electrolytic, hormonal and neuroradiologic findings with emphasis in the unusual location of this disease.
Conclusion: Unlike central pontine myelinolysis, extrapontine has a lower incidence. Trismus, manifested in this patient is an unusual sign. The treatment is only supportive, with a high probability of mortality.
Leer artículo completo
Disappearing Cerebral Microbleed After A Lacunar Infarct
Both, lacunar infarcts and deep cerebral microbleeds are recognized markers of cerebral small vessel disease. However, the “de novo” appearance of an infarct at the site of a previously documented microbleeds has not been described. Here, we report a patient who suffered a lacunar infarct at the site of a microbleed, associated with its disappearing on MRI. This case illustrate a previously unrecognized progression of a cerebral microbleed, which may help to elucidate pathogenetic mechanisms involved in this uncommon evolution.
Leer artículo completo
“Brain Holes.” A Diagnostic Challenge In Cysticercosis-Endemic Areas.
The introduction of modern neuroimaging and immunologic techniques has increased the diagnostic accuracy for neurocysticercosis.
However, there are some conditions that mimic neurocysticercosis by the time of the initial evaluation of the patient. We present six patients with a single abnormally dilated perivascular Virchow-Robin space mimicking a cysticercus.
In such cases, administration of repeated trials of cysticidal drugs together with long-term follow-up observation, excluded the diagnosis of neurocysticercosis. Physicians working in cysticercosis-endemic areas must keep in mind the possible occurrence of a single dilated perivascular space, to avoid the practice of unnecessary invasive procedures in patients with “cysticerci-like” lesions that do not change after several courses of cysticidal drug therapy.
Leer artículo completo
Epilepsia Benigna Occipital tipo Panayiotopoulos y Epilepsia Benigna con espigas centrotemporales concomitantemente en el mismo paciente.
The Panayiotopoulos type benign childhood occipital epilepsy, (PBCOE) and the benign childhood occipital epilepsy with centro-temporal spikes (BCECT) are two forms of focal idiopathic epilepsy recognized by the International League Against Epilepsy. Several authors have reported that some patients with PBCOE evolve later in life toward a BCECT, remarking the fact of the morphological similarities of their paroxysms and the spikes migration phenomena. However, few cases of patients with PBCOE have been described concomitantly clinical characteristics of a BCECT in the same evolving process. One clinical case with the mentioned concomitant manifestation is shown, analyzing its clinical characteristics, differential diagnosis and treatment. Finally it is postulated that the PBCOE could be an early form of the BCECT
Leer artículo completo
Resolución clínica de un hematoma subdural crónico en un niño con quiste aracnoideo conocido.
Chronic subdural haematoma is a well-known and relatively frequent entity in the elderly. It is originated by a slight trauma which provokes subdural veins to rupture after suffering stretching due to cortical atrophy.
We report the case of a 11 year old patient with a previous known arachnoid cyst who was diagnosed as chronic subdural haematoma without association to cranial trauma and with slight neurological impairment. He responded successfully to pharmacological therapy. The following clinical case permits review pathophysiology of chronic subdural haematoma and considers subarachnoid cyst as a risk factor.
Leer artículo completo
Esclerosis Lateral Amiotrófica Secundaria a Infección por VIH.
Motor neuron diseases are not frequently associated to Human Immunodeficiency Virus (HIV) infection, although some reports have mentioned this relationship, suspecting a viral involvement in the pathogenesis of this disease. A 43-years-old male with HIV diagnosis is described, who started with progressive weakness of his legs, showing further worsening 6 months later, with upper extremity and bulbar involvement. Neurophysiological studies demonstrated upper and lower motor neuron compromise in all the extremities and bulbar muscles. Other causes were ruled out. Auto-antibodies against Human Herpes Virus type 8 were positive for IgG. There were no anomalies on image studies. A change in the antiviral scheme stopped temporarily the progression of the clinical features. However, posterior withdrawal of medications due to toxic hepatitis led to worsening of signs and symptoms. This case, among the previously reported worldwide, suggests that the association between motor neuron diseases and HIV is not coincidental, this must raise suspicion in every subject with risk factors for HIV and coexistent motor neuron disease.
Leer artículo completo
Síndrome de Guillain Barré imitando Muerte Encefálica.
Guillain Barré Syndrome is a neurological disease frequently admitted to the Intensive Care Unit, especially if there is associated hemodynamic or ventilatory compromise. Brain death is the irreversible cessation of all functions of Central Nervous System, and rarely, Guillain Barré Syndrome can mimick it, with subsequent recovery in the next few days or weeks. We report a case admitted to the Intensive Care Unit, with this type of evolution.
Leer artículo completo
Síndrome de Tolosa-Hunt: Hallazgos en Resonancia Magnética Cerebral antes y después del tratamiento con Terapia Corticoidal Sistémica.
Tolosa-Hunt syndrome is characterized by painful ophthalmoplegia due to a granulomatous inflammation in the cavernous sinus. Corticosteroid therapy dramatically resolves both clinical and radiological findings. We report a case of a patient 55 years old, initially managed as cavernous sinus thrombophlebitis, and by means of magnetic resonance imaging studies, was then diagnosed as Tolosa-Hunt syndrome. After receiving adequate therapy, clinical symptoms improved. We recommend serial magnetic resonance imaging studies when Tolosa-Hunt syndrome is suspected in order to differentiate it from other cavernous sinus lesions that can mimic it clinically and radiologically.
Leer artículo completo
Una propuesta para la evaluación de la Prueba de Wada en pacientes analfabetos: Presentación de un caso.
In the preoperative neuropsychological evaluation of certain patients with epilepsy it is important to determine the hemispheric dominance; particularly in those of doubtful laterality, due to the high postoperative risks of affectation of the language and the memory. The test of Wada allows guiding the neurosurgeon about the possible impact of the surgical procedure in the hemispheric areas related to the language and the memory. Although it is well-known that the execution in diagnostic neuropsychological tests is under the influence of an important series of variables, as the culture and the educational level, until now there are not specific protocols for this kind of exploration in illiterate patients. In this study we describe the experience in the test of WADA of an illiterate patient with epilepsy diagnosis and cerebral congenital left hemiatrophy, of left-handed manual laterality. With base in the protocol of Trenerry and Loring the language tasks and memory were implemented according to the patient’s cultural context. It was found a right hemispheric representation for the language and the memory. These results confirm the effects of the cerebral plasticity in the reorganization and /or compensation of the cognitive functions, as the language and the memory in the cerebral hemisphere not affected.
