RESUMEN
Treatment of restless legs syndrome (RLS) must only be considered after a definite positive diagnosis. The RLS phenotype must be characterised precisely, iron deficiency always tested for, and aggravating factors eliminated when possible. Medical treatment is considered for severe or very severe forms and based on dopaminergic agonists, α2δ-1 ligands and/or opioids. First line treatment will be a low-dose monotherapy and the choice of treatment depends on the results of the clinical examination and investigations.
Asunto(s)
Síndrome de las Piernas Inquietas/terapia , Analgésicos Opioides/uso terapéutico , Anticonvulsivantes/uso terapéutico , Consenso , Agonistas de Dopamina/uso terapéutico , Francia , Humanos , Síndrome de las Piernas Inquietas/tratamiento farmacológicoRESUMEN
BACKGROUND: Reversible posterior leukoencephalopathy syndrome (RPLS) is characterised by clinical neurological features of sudden onset and brain MRI findings such as T2/Flair white matter hyperintensities. RPLS can occur in autoimmune diseases, and rarely in systemic vasculitis. We report a case of RPLS in a woman presenting granulomatosis with polyangiitis (Wegener's granulomatosis). PATIENTS AND METHODS: A 22-year-old female patient was treated with methylprednisolone pulses for granulomatosis with polyangiitis and neurological impairment. A few hours after the second pulse, the patient had seizures, blindness and confusion associated with high blood pressure and acute renal failure. MRI revealed a high-intensity area on T2-Flair weighted images of the occipital-temporal lobes. The patient was treated with antiepileptic and antihypertensive medications, oral steroids and cyclophosphamide; the clinical and radiological findings proved reversible over the ensuing days. DISCUSSION: The occurrence of RPLS in systemic vasculitis is rare. Six cases of RPLS associated with granulomatosis and polyangiitis have been reported. It appears important to screen for high blood pressure in patients recently treated with corticosteroids for vasculitis as this condition may represent a precipitating factor for RPLS.
Asunto(s)
Granulomatosis con Poliangitis/diagnóstico , Síndrome de Leucoencefalopatía Posterior/diagnóstico , Corticoesteroides/uso terapéutico , Anticonvulsivantes/uso terapéutico , Antihipertensivos/uso terapéutico , Encéfalo/efectos de los fármacos , Encéfalo/patología , Ciclofosfamida/uso terapéutico , Femenino , Granulomatosis con Poliangitis/tratamiento farmacológico , Humanos , Imagen por Resonancia Magnética , Metilprednisolona/efectos adversos , Metilprednisolona/uso terapéutico , Examen Neurológico/efectos de los fármacos , Síndrome de Leucoencefalopatía Posterior/inducido químicamente , Síndrome de Leucoencefalopatía Posterior/tratamiento farmacológico , Factores de Riesgo , Sustancia Blanca/efectos de los fármacos , Sustancia Blanca/patología , Adulto JovenRESUMEN
INTRODUCTION: First described 15 years ago, primary progressive anarthria is a focal cortical atrophy defined as a rare progressive impairment of speech associated with orofacial apraxia and leading to mutism with a frontal lobe syndrome. The aim of this study was to analyze clinical and neuropsychological data and results of complementary tests in a series of patients presented with primary progressive anarthria. MATERIAL AND METHODS: We, retrospectively, studied five patients with primary progressive anarthria. We particularly analyzed the following parameters: age at onset, age at the diagnostic, disease time from onset to first consultation, the initial orientation, the neuropsychological and clinical data at the first visit, electromyography, brain MRI, and single photon emission computed tomography (SPECT) findings. Clinical and neuropsychological data were used to monitor disease course. RESULTS: The mean age at onset of symptoms was 75.2+/-5.8 years. Patients were primarily referred to a specialist in memory disease (n=3) or a specialist in motor neuron disease (n=2). The time from onset to first consultation was 11.2+/-3 months. Anarthria was associated with dysexecutive syndrome and sometimes, with impaired comprehension. Electromyography was always normal. Cranial MRI showed temporal or left frontal atrophy (n=3). Spect revealed decreased cerebral blood flow predominating in the left frontal or temporal region (n=4). CONCLUSION: Long delay for specialist consultation and inadequate initial orientation retard disease diagnosis, leading to severe incapacity. Complementary studies are required to confirm diagnostic and to rule out lateral amyotrophic sclerosis. During the early stages, involvement of the premotor cortex may be considered due to the speech apraxia. Secondary motor orofacial disturbances suggest an extension to the motor cortex. Primary progressive anarthria is a distinct individual entity within the spectrum of focal cortical atrophies.