Status Epilepticus in a Patient with Amyotrophic Lateral Sclerosis

Öz

Introduction: Amyotrophic Lateral Sclerosis, the most common and well-known form of motor neuron disease, is characterized by progressive degeneration of upper and lower motor neurons. Respiratory failure and aspiration due to respiratory muscle weakness is the most common causes of mortality in patients with amyotrophic lateral sclerosis. Status epilepticus is the second most frequent neurological emergency with a risk of major morbidity or mortality. Status epilepticus, due to causes such as prolonged seizures, respiratory distress, acidosis, hypoglycemia, and hypotension may lead to mortality. Case Report: A forty-eight year old male patient had been followed for 4 years with a diagnosis of amyotrophic lateral sclerosis and was referred to our clinic with the diagnosis of status epilepticus. The refractory status epilepticus patient was treated with a diagnosis of respiratory failure and the need for mechanical ventilation evolved.Conclusion: We report the development of status epilepticus in a patient with amyotrophic lateral sclerosis, and in addition to the appropriate anti-epileptic medication, ventilator support and intensive care was needed, emphasizing the life-saving procedure

Anahtar Kelimeler

• Amyotrophic lateral sclerosis, status epilepticus

Amiyotrofik Lateral Sklerozlu Bir Hastada Status Epileptikus

Öz

Giriş: Amiyotrofik Lateral Skleroz, Motor Nöron Hastalığı’nın en yaygın ve en iyi bilinen formu olup üst ve alt motor nöronların progresif dejenerasyonu ile seyreder. Amiyotrofik Lateral Skleroz’lu hastalarda solunum kaslarının güçsüzlüğü nedeniyle gelişen solunum yetmezliği mortalitenin en sık nedenleri arasında yer almaktadır. Status epileptikus önemli morbidite ve mortalite riski ile akut inmeden sonra ikinci en sık rastlanan nörolojik acildir. Status epileptikus, nöbet süresinin uzaması, solunum zorluğu, asidoz, hipoglisemi ve hipotansiyon gibi nedenlerle mortaliteye neden olabilir. Olgu Sunumu: Kırk sekiz yaşında erkek hasta, 4 yıldır Amiyotrofik Lateral Skleroz tanısıyla takip edilmekte iken status epileptikus tanısıyla kliniğimize dış merkezden sevk edildi. Dirençli status epileptikus tanısı ile tedavi verilen hastada solunum yetmezliği ve mekanik ventilatör ihtiyacı gelişti. Sonuç: Bu iki nadir hastalığın birbirleri üzerine etkileri hakkında literatürde yeterli bilgi bulunmamaktadır. Biz bu olgu sunumu ile Amiyotrofik Lateral Sklerozlu bir hastada status epileptikus gelişebileceğini bildirmek ve uygun antiepileptik tedaviye ek olarak, ventilatör ve yoğun bakım desteğinin de yaşam kurtarıcı olacağını vurgulamak istedik.

Anahtar Kelimeler

• Amiyotrofik lateral skleroz, status epileptikus

Kaynakça

1. Chio A, Mora G, Calvo A, Mazzini L, Bottacchi E, Mutani R, et al. Epide- miology of ALS in Italy: A 10-year prospective population-based study. Neurology 2009; 72: 725-31. [CrossRef]
2. Pagnini F, Banfi P, Lunetta C, Rossi G, Castelnuovo G, Marconi A, et al. Re- spiratory function of people with amyotrophic lateral sclerosis and care- giver distress level: A correlational study. Biopsychosoc Med 2012; 6: 14. [CrossRef]
3. Rona S, Rosenow F, Arnold S, Carreño M, Diehl B, Ebner A, et al. A semio- logical classification of status epilepticus. Epileptic Disord 2005; 7: 5-12.
4. DeLorenzo RJ. Epidemiology and clinical presentation of status epilep- ticus. Adv Neurol 2006; 97: 199-215.
5. Hauser WA. Status epilepticus: frequency, etiology, and neurological sequelae. Adv Neurol 1983; 34: 3-14.
6. Meldrum BS. Glutamate as a neurotransmitter in the brain: Review of physiology and pathology. J Nutr 2000; 130: 1007-15.
7. Bos WM. In search for peripheral markers for epilepsy and ALS - focus on glutamatergic signaling in blood cells. Willemina Minke Bos 2005.
8. Delorenzo RJ, Pellock JM, Towne AR, Boggs JG. Epidemiology of status epilepticus. J Clin Neurophysiol 1995; 12: 316-25. [CrossRef]

9. Kim JE, Kim DS, Kwak SE, Choi HC, Song HK, Choi SY, et al. Anti-glutama- tergic effect of riluzole: Comparison with valproic acid. Neuroscience 2007; 147: 136-45. [CrossRef]
10. Nguyen TT, Hussain E, Grimason M, Goldstein J, Wainwright MS. Neurogen- ic pulmonary edema and acute respiratory distress syndrome in a healthy child with febrile status epilepticus. J Child Neurol 2012; 10: 1287-91.
11. Rosenow F, Arzimanoglu A, Baulac M. Recent developments in treat- ment of status epilepticus: A review. Epileptic Disorder 2002; 4: 41-51.