ABSTRACT
Resumen El síndrome de leucoencefalopatía posterior reversible (PRES) es un desorden neurológico agudo caracterizado por cefalea, alteración de la conciencia, convulsiones y alteraciones visuales, con imágenes de edema vasogénico reversible en regiones cerebrales posteriores. Nos propusimos describir una serie de casos de pacientes trasplantados que desarrollaron PRES, caracterizando su presentación, evolución clínica, imágenes y terapéutica. Se analizaron historias clínicas informatizadas desde enero 2009 hasta enero 2019. Se recabaron datos demográficos, antecedentes clínicos, motivos y días de internación, tiempos desde el trasplante a la presentación clínica y diagnóstico. Se evaluó la mejoría/resolución en estudios por imágenes y la supervivencia anual. Se identificaron 27 pacientes con PRES; 22 trasplantados de órgano sólido de 1647 totales (1.3%) y 5 de médula ósea de 617 totales (0.8%). La media de edad fue de 38.2 años (DS 19.5), 62.9% de sexo femenino, 59.2% (16) antes del año del trasplante. Las comorbilidades más frecuentes enfermedad renal (14; 51%) e hipertensión arterial (11; 40%). Se realizó tomografía axial computarizada (TAC) a 23 pacientes (85.1%), siendo patológica en 11 (47.8%), y resonancia magnética nuclear (RMN) a 25 (92.6%), mostrando patrón característico en 17 (62.9%), con mejoría/resolución antes del año en 20 (74%). El tratamiento fue sintomático, modificando la inmunosupresión. Se registraron 5 óbitos durante la internación y otros 3 antes del año, con una supervivencia anual del 70.3% (19). La población de trasplantados, en crecimiento en nuestro medio, es particularmente susceptible al PRES. Tanto su presentación en estudios por imágenes, como su comorbilidad, difieren de otras poblaciones.
Abstract Posterior reversible encephalopathy syndrome (PRES) is an acute neurological disorder characterized by headache, encephalopathy, seizures and visual disturbances, with reversible vasogenic edema in posterior brain areas. The aim of this research was to describe a case series of transplanted patients who developed PRES, characterize their presentation, treatment, clinical and imaging evolution. Electronic medi cal records were analyzed from January 2009 to January 2019. Demographic data, clinical backgrounds, causes of admission, hospital length of stay and time from transplantation to PRES were collected. Image improvement/ resolution and annual survival were assessed. We identified 27 patients with PRES; 22 of 1647 total solid-organ transplant (1.3%) and 5 of 617 total bone marrow transplant (0.8%). The mean age at presentation was 38.2 years (SD 19.5), 62.9% female, 59.2% (16) before the year of transplantation. The most common comorbidities were kidney disease (14; 51%) and high blood pressure (11; 40%). Computed axial tomography (CT) was per formed in 23 patients (85.1%), with pathological findings in 11 (47.8%). Magnetic resonance imaging (MRI) of 25 (92.6%), showed a characteristic pattern in 17 (62.9%) with improvement/resolution before the year in 20 (74%). Treatment was symptomatic, modifying immunosuppression. Five deaths were recorded during hospital stay and another 3 before the year of admission, with an annual survival of 70.3% (19 patients). Organ transplant trend is growing in our region. These patients are particularly susceptible to PRES, with a different imaging presentation and comorbidities from other populations.
Subject(s)
Humans , Male , Female , Posterior Leukoencephalopathy Syndrome/etiology , Posterior Leukoencephalopathy Syndrome/epidemiology , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Hypertension , Seizures , Magnetic Resonance Imaging , Follow-Up StudiesABSTRACT
La polirradiculopatía inflamatoria crónica sensitiva es una entidad definida, frecuentemente subdiagnosticada y potencialmente tratable. Debe ser sospechada en pacientes con ataxia sensitiva, estudios de conducción nerviosa normales y una resonancia magnética que muestre engrosamiento y realce con gadolinio de las raíces lumbosacras. Presentamos el caso de un hombre de 57 años de edad con marcada ataxia sensitiva en pierna izquierda. Al examen físico presentaba fuerza conservada, reflejos osteotendinosos disminuidos, tacto fino y superficial reducidos por debajo de las rodillas; abatiestesia y apalestesia en ambos pies. Los estudios de conducción nerviosa eran normales, los potenciales evocados somatosensitivos tibiales con ausencia de respuesta bilateral. El líquido cefalorraquídeo presentaba hiperproteinorraquia sin células. La resonancia magnética mostró engrosamiento y realce con gadolinio de las raíces lumbosacras. El paciente fue tratado con inmunoglobulina endovenosa (IgEV) a 2 g/kg durante 5 días, con buena respuesta. La evolución clínica, la hiperproteinorraquia, el realce de raíces en la resonancia magnética, la buena respuesta a la inmunoterapia y la exclusión de otras causas de ataxia sensitiva fueron compatibles con el diagnóstico de polirradiculopatía inflamatoria crónica sensitiva. Para el diagnóstico de esta enfermedad se requiere la identificación del compromiso aislado de las raíces sensitivas.
Chronic inflammatory sensory polyradiculopathy is a defined entity, frequently underdiagnosed, and potentially treatable. It must be suspected in patients with sensory ataxia, normal nerve conduction studies, and MRI with thickened lumbosacral nerve roots and gadolinium enhancement. We present the case of a 57-year-old man with marked sensory ataxia on his left leg. Examination showed normal strength, decreased knee and ankle jerks. Light touch and pinprick sensations were reduced below the knees. Vibration and joint position sense were absent at the feet. Nerve conduction studies were normal. Tibial sensory evoked potentials disclosed absent responses bilaterally. CSF was acellular with elevated protein. Lumbosacral magnetic resonance showed thickening of roots, with gadolinium enhancement. The patient was treated with IV-Ig, 2 g/kg, for 5 days with improvement of symptoms. The clinical course, elevated CSF protein, the evidence of root enhancement on the MRI, good response to immunotherapy, and the exclusion of other causes of sensory ataxia, were compatible with the diagnosis of chronic inflammatory sensory polyradiculopathy. To diagnose this disease the identification of isolated involvement of the sensory roots is required.
Subject(s)
Humans , Male , Middle Aged , Gadolinium , Neural Conduction/physiology , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/diagnosis , Electromyography , Magnetic Resonance Imaging , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/cerebrospinal fluid , Spinal Nerve Roots/pathologyABSTRACT
Lethargic encephalitis (LE) is a Central Nervous System disorder following an upper respiratory tract infection, characterized by sleep disturbances, clinical symptoms corresponding to basal ganglia involvement and in some cases, neuropsychiatric sequelae. We report a 18-year-old mole with a history of sinusitis treated with azithromycin, two weeks before, presenting with fever, headache, confusion and myoclonus. Urine analysis was positive for cannabis. Cerebro spinal fluid analysis showed mononuclear pleiocytosis (109xmm³) and an increase in protein concentration ofl.6 g/dl. Forty eight hours after admission, the patient required mechanical ventilation and subsequently a status epilepticus appeared. Ten days later, fever, rigidity and resting tremor appeared. A magnetic resonance imaging showed hyperintensities in FLALR sequence in the right insular cortex. The patient continued with extreme rigidity, catatonia and mutism. Considering the possibility ofa LE, methyl prednisolone 1 g/day was administered for five consecutive days followed by prednisone 40 mgl day, observing a dramatic improvement of rigidity and tremors.