INDO appears effective in reducing ICP and improving CPP in RICH. INDO test could be a useful tool in identifying RICH patients with favorable outcome. Future studies are needed.
We have reported three cases of hyperornithinaemia associated with hyperammonaemia and homocitrullinuria (HHH). They deal with two brothers and a sister from a family where the parents and four other children are healthy on clinical and biochemical examination. The biochemical findings in our patients indicate the existence of a defect in the transport of ornithine into the mitochondria. Cultured skin fibroblasts from two of these patients incorporated six times less [14C]ornithine into protein as compared to control cells. The most characteristic sign of the clinical picture is the progressive spastic paraparesis found in one of the cases. Ornithine supplementation and restricted protein intake may be useful in the treatment of this syndrome since after three years of treatment the clinical response was favourable and the patients showed no adverse clinical effects.
com.ar L a prevalencia de los estados poliúricos en sujetos con injuria cerebral aguda es desconocida, pudiendo presentarse como consecuencia del daño neurológico per se o por la terapéutica adoptada 1-4 . Poliurias pueden tener impacto negativo sobre el cerebro injuriado, a través de hipovolemia, hipotensión arterial y cambios en la osmolaridad. Implica un verdadero reto diagnóstico-terapéutico 5,6 ; por ello, un rápido reconocimiento y adecuado análisis, permitirá instaurar la conducta más apropiada. Los estados poliúricos están relacionados con desequilibrios hidroelectrolíticos, (principalmente del contenido de sodio) y trastornos de la osmolaridad 7-10 , situaciones que contribuyen a agravar y perpetuar el daño inicial 11 . Este trabajo pretende en forma práctica y sencilla aportar un enfoque que nos permita reconocer los estados poliúricos, identificar sus causas y analizar la manera más adecuada de tratarlos. Primer paso: ¿cómo definimos poliuria?Poliuria deriva del griego "poly" (mucho); ouro (orina) 12 . Se define de diferentes maneras: a) > 3.000 cc diarios; b) > 150 ml/h; c) > 3 ml/kg/h, o d) diuresis mayor a 10 ml/min [5][6][7][8][9][10] . Sus causas son variadas al igual que su duración, pudiendo estar ocasionadas por la injuria cerebral o por intervenciones terapéuticas: reposiciones de volumen, utilización de agentes osmóticos (manitol, salino hipertónico), diuréticos, nutrición parenteral.
A 61-year-old woman with a stable traumatic anterior L4 vertebral wedge fracture without spinal canal compromise was referred because of an intense and refractory pain at the level of fracture in spite of maximal medical therapy. MRI scans showed anterior wedging of L4 vertebral body involving the superior endplate with intact posterior wall ( figure 1A). Percutaneous vertebroplasty (PV) was indicated for her high surgical risk. Under general anesthesia, right L4 transpedicular PV was performed under X-ray fluoroscopy using polymethylmethacrylate (PMMA; volume injected 3 cm 3 ; SpinePlex ® ; Kalamazoo, MI).No critical leaks or lesions of the posterior cortex or the lateral recess were identifiable in postprocedure fluoroscopy.Two hours after PV, the patient presented an intense burning pain in both legs, Lhermitte sign, and tactile hypersensitivity associated with low blood pressure (50/30 mm Hg), respiratory rate (8 breaths/ minute), and body temperature (rectal temperature: 35.0°C), together with hypoxemia (SaO 2 : 75%, in room air) and sinus tachycardia (heart rate 141/ minute). The initial symptoms were followed by sustained (35-45 seconds) and almost continuous muscle spasms in bulbar, pharyngolaryngeal, intercostal, paravertebral, and leg muscles, spontaneous and in response to minimal stimuli, stiffness of the body, lockjaw, frothing of the mouth, opisthotonus, bilateral Babinski sign, and generalized hyperreflexia. The patient was fully conscious during these episodes. During severe spasms, there was respiratory arrest. The patient was immediately referred to the neurointensive care unit (NICU). She was intubated and mechanically ventilated.
Cerebral venous sinus thrombosis (CVT) can compromise dural sinus, cerebral veins or both. It is an uncommon condition and it is more prevalent in young women. Several prothrombotic states are the principal predisposing factors. Clinical spectrum of presentation is wide, so this entity requires a high suspect index for correct and prompt diagnosis. CVT may develop serious complications that can be life-threatening such as hemorrhagic venous infarctions, cerebral edema, and intracranial hypertension.This report describes the case of a woman who was in treatment for unspecific vaginal bleeding with oral contraceptives. Suddenly she deteriorated to coma with severe respiratory compromise. Neuroimaging showed thrombosis of multiple venous sinus. Physiological neuroprotection, osmotherapy, mechanical ventilation and anticoagulation therapy were the keystones of treatment.In a few months, the patient has recovered a good functional status, while maintaining a motor deficit on the right hand.
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