[Subacute encephalopathy associated with relapsing polychondritis. Report of one case]. / Encefalopatía subaguda en una paciente con policondritis recidivante. Caso clínico.

Relapsing polychondritis (RP) is a rare multisystemic autoimmune disorder characterized by the inflammation and destruction of cartilages, with preference for auricular, nasal and laryngotracheal cartilages. RP may also affect proteoglycan-rich structures, such as, blood vessels, eyes, kidneys, and heart. The central nervous system (CNS) is involved in less than 3% of patients. We report a 32-year-old female with RP associated with a progressive subacute encephalopathy characterized by behavioral disturbances, auditory and visual hallucinations. The EEG showed generalized slow activity and a mononuclear pleocytosis with increased protein was found in the cerebrospinal fluid. The brain magnetic resonance imaging showed multiple supra and infratentorial nodular inflammatory lesions. After initiating treatment with corticosteroids and cyclophosphamide, a significant improvement in chondritis and neurological status was observed.

Encefalopatía subaguda en una paciente con policondritis recidivante: caso clínico / Subacute encephalopathy associated with relapsing polychondritis: report of one case

Relapsing polychondritis (RP) is a rare multisystemic autoimmune disorder characterized by the inflammation and destruction of cartilages, with preference for auricular, nasal and laryngotracheal cartilages. RP may also affect proteoglycan-rich structures, such as, blood vessels, eyes, kidneys, and heart. The central nervous system (CNS) is involved in less than 3% of patients. We report a 32-year-old female with RP associated with a progressive subacute encephalopathy characterized by behavioral disturbances, auditory and visual hallucinations. The EEG showed generalized slow activity and a mononuclear pleocytosis with increased protein was found in the cerebrospinal fluid. The brain magnetic resonance imaging showed multiple supra and infratentorial nodular inflammatory lesions. After initiating treatment with corticosteroids and cyclophosphamide, a significant improvement in chondritis and neurological status was observed.

[Monomelic amyotrophy. Report of one case]. / Atrofia monomiélica distal de extremidad superior. Caso clínico.

Monomelic amyotrophy, also known as Hirayama disease, is a rare lower motor neuron syndrome due to localized lower motor neuron loss in the spinal cord at the cervical level. Clinically, monomelic amyotrophy is defined by the insidious onset of unilateral atrophy and weakness involving the hand and forearm, typically beginning in the second or third decade of life. We report 19-year-old man with a two years history of slowly progressive unilateral weakness and atrophy of his right-hand muscles. Neurological examination revealed weakness and atrophy in his intrinsic hand muscles, with sparing of the abductor pollicis brevis muscle. Also, mild atrophy of the ulnar aspect of the forearm was detected with sparing of the brachioradialis muscle. Electromyography showed active and chronic neurogenic changes affecting C8 and T1 myotomes, with mild chronic neurogenic changes on C7 myotome. Magnetic resonance imaging of his cervical spine revealed spinal cord atrophy involving C5 to C7 segments, associated with forward displacement of the posterior wall of the dura in cervical spine flexion. The clinical features associated with the imaging and electrophysiological findings support the diagnosis of monomelic amyotrophy.

High-frequency spinal cord stimulation as rescue therapy for chronic pain patients with failure of conventional spinal cord stimulation.

BACKGROUND: This study aims to evaluate the efficacy of 10-kHz high-frequency (HF10) devices as a rescue treatment in patients with failure of conventional spinal cord stimulation (SCS) therapy for chronic pain without the need to change the spinal hardware. METHODS: In this real-world prospective study, patients with neuropathic pain treated with conventional tonic SCS in whom the therapy had failed, either during the trial phase or after a period of optimal functioning, were recruited throughout 2 years for HF10-SCS therapy. Data on analgesia, functionality, analgesics use and treatment safety were collected 12 months after treatment. RESULTS: Eleven of the 18 (61%) patients included in the study were successfully rescued with HF10-SCS. Of them, 5 out of 12 (45%) were in the trial phase and six out of six (100%) had previously functioning implants. A significant improvement in low-back and limb pain was obtained (p = 0.003 and p = 0.0001, respectively). Treatment success was significantly associated with gender (p = 0.037), weight (p = 0.014), body mass index (BMI) (p = 0.007) and time of rescue (p = 0.015). A linear regression test confirmed a significant association between treatment failure and BMI and gender (p = 0.004). CONCLUSIONS: Our results suggest that analgesic rescue with HF10-SCS is an effective therapeutic option for non-responders to conventional SCS, although obesity might be a limiting factor for treatment success. Nevertheless, more comprehensive studies are needed to corroborate our findings. SIGNIFICANCE: This study shows that high-frequency stimulation may be useful in patients with failure of conventional tonic stimulation for chronic pain, both in the trial phase and in previously implanted subjects. The novelty of this study lies in the use of the implanted epidural electrodes, which avoids the need for further surgery. The results in terms of pain control and recovery of functionality are satisfactory. In addition, variables such as male gender and high body mass index could be predictors of therapy failure.

Atrofia monomiélica distal de extremidad superior. Caso clínico / Monomelic amyotrophy. Report of one case

Monomelic amyotrophy, also known as Hirayama disease, is a rare lower motor neuron syndrome due to localized lower motor neuron loss in the spinal cord at the cervical level. Clinically, monomelic amyotrophy is defined by the insidious onset of unilateral atrophy and weakness involving the hand and forearm, typically beginning in the second or third decade of life. We report 19-year-old man with a two years history of slowly progressive unilateral weakness and atrophy of his right-hand muscles. Neurological examination revealed weakness and atrophy in his intrinsic hand muscles, with sparing of the abductor pollicis brevis muscle. Also, mild atrophy of the ulnar aspect of the forearm was detected with sparing of the brachioradialis muscle. Electromyography showed active and chronic neurogenic changes affecting C8 and T1 myotomes, with mild chronic neurogenic changes on C7 myotome. Magnetic resonance imaging of his cervical spine revealed spinal cord atrophy involving C5 to C7 segments, associated with forward displacement of the posterior wall of the dura in cervical spine flexion. The clinical features associated with the imaging and electrophysiological findings support the diagnosis of monomelic amyotrophy.

Cavernous Sinus Hemangioma: Imaging Diagnosis and Surgical Considerations.

BACKGROUND: Cavernous sinus hemangiomas (CSHs) are extraaxial vascular malformations that tend to bleed during surgery. METHODS: We reviewed 12 magnetic resonance imaging scans with CSH, 5 of them biopsy proven. RESULTS: In our review, CSH commonly presented as a lobulated mass with high, uniform signal intensity on T2-weighted images, a dumbbell shape, and a sellar extension. Two thirds presented a "filling-in" pattern of enhancement on dynamic imaging. These features should lead to a correct preoperative diagnosis, which is essential for surgical planning and avoiding hemorrhagic complications. CONCLUSIONS: A combination of low signal on T1, high signal on T2 and fluid-attenuated inversion recovery, no diffusion restriction and homogenous enhancement should place CSH at the top of the list of differential diagnoses. This is especially true when there is a "filling-in" pattern on dynamic or delayed imaging. Doing so may alert surgeons to the possibility of copious intraoperative bleeding and therefore avoid complications of hemorrhage.

[Creutzfeldt-Jakob disease: Report of one case]. / Desafíos en el diagnóstico de enfermedad de Creutzfeldt-Jakob: Caso clínico.

Creutzfeldt-Jakob disease has a higher incidence in Chile than in other countries. The post mortem pathological characterization of brain tissue is necessary to reach a definitive diagnosis. We report a 73 years old man with a history compatible with of a rapidly progressive dementia, in which the first electroencephalographic study showed a pattern consistent with non-convulsive status epilepticus. Besides discarding this diagnosis, it was necessary to rule out other causes of rapidly progressive dementia such as Hashimoto encephalopathy. Finally, the sustained clinical deterioration with no response to anticonvulsants and corticosteroids, the imaging studies, a serial electroencephalographic monitoring study and the detection of 14-3-3 protein in cerebrospinal fluid were the keys to achieve the diagnosis of the disease.

Desafíos en el diagnóstico de enfermedad de Creutzfeldt-Jakob: caso clínico / Creutzfeldt-Jakob disease: report of one case

Creutzfeldt-Jakob disease has a higher incidence in Chile than in other countries. The post mortem pathological characterization of brain tissue is necessary to reach a definitive diagnosis. We report a 73 years old man with a history compatible with of a rapidly progressive dementia, in which the first electroencephalographic study showed a pattern consistent with non-convulsive status epilepticus. Besides discarding this diagnosis, it was necessary to rule out other causes of rapidly progressive dementia such as Hashimoto encephalopathy. Finally, the sustained clinical deterioration with no response to anticonvulsants and corticosteroids, the imaging studies, a serial electroencephalographic monitoring study and the detection of 14-3-3 protein in cerebrospinal fluid were the keys to achieve the diagnosis of the disease.

Factores asociados al consumo de drogas entre estudiantes de enseñanza media en la Región de Magallanes / Risk factors associated to drug consumption among secondary education students in the Magallanes Region

El objetivo de este estudio es la identificación de posibles factores de riesgo para el consumo de drogas entre jóvenes de enseñanza secundaria en la Región de Magallanes, lo que contribuiría al desarrollo de programas preventivos que logren una mayor efectividad y eficiencia social. La población estudiada estuvo constituida por el universo de estudiantes de 3° año de enseñanza media, de los colegios de esa región durante 1994 (1.270 jóvenes). Se aplicó un set de encuestas en forma autoadministrada, que indagaba por los datos personales, la relación con el colegio, consumo de sustancia por los padres y los amigos, varias dimensiones psicosociales y las características del consumo de drogas (cuando estaba presente). Se encontró asociación significativa con el consumo de drogas para las variables que se refieren a la relación del joven con el colegio, para el consumo de drogas por parte de los padres y de los amigos, para una mayor intensidad del hábito tabáquico y el consumo de bebidas alcohólicas, para variables psicosociales como el nivel de síntomas ansioso-depresivos, el nivel de estrés de los últimos meses y el apoyo social que el joven siente que sus padres le dan. La mayoría de los jóvenes considera que el consumo de drogas es dañino y esta creencia se asocia en forma inversa con el uso de sustancias. Sin embargo, la mayoría de los consumidores funciona con una disonancia cognitivo - conductual al respecto. La mayoría de los jóvenes ha dejado de consumir drogas por períodos y sólo una proporción muy pequeña señala que no fue capaz de hacerlo. Los resultados encontrados son relevantes para el diseño de programas preventivos. Se apreciaron importantes diferencias para varones y mujeres, lo que plantea la necesidad de incorporar una perspectiva de género en estos programas.