Mills' Syndrome A Clinical Variant -Case Report
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Tarih
2013
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info:eu-repo/semantics/openAccess
Özet
Mills sendromu 1900 yılında Mills tarafından tanımlanan unilateral progresif assendan veya dessendan hemipleji ile duysal tutulum olmaksızın ortaya çıkan nadir bir motor nöron hastalığıdır. Primer lateral sklerozun bir varyantı olduğu da ileri sürülen bu sendromun kortikospinal yolun tek taraflı dejenerasyonuna bağlı olduğu düşünülmektedir. Sol spastik hemiparezi, yutma ve konuşma güçlüğü, bilateral alt ekstremitelerde belirgin spastisite ile kliniğimize başvuran 56 yaşında, sağ elli kadın hastanın yakınmalarının 3 yıldır davranış bozukluğu, ani gülme atakları, kol ve bacaklarda kasılmalar, yürüme bozukluğu, yutma güçlüğünü takiben, önce yavaş bir şekilde başlayan sol alt ekstremitede kuvvet kaybı, göz hareketlerinde beceriksizlik, solda belirgin spastisitesi ve kognitif yıkım süreci ile devam ettiği öğrenildi. Nörolojik muayenede, bilinç açık, kısmen koopere idi. Kranial sinir muayenesinde disfaji ve disfoni, motor muayenesinde sol alt ve üst ekstremite spastik plejik, sağ alt ve üst ekstremitede spastisite dışında motor kuvvet tamdı. Derin tendon refleksleri bütün odaklarda hiperaktifdi. Babinski sağda pozitifti. Duyu muayenesi normaldi. Kranial MRI sağ hemisferde belirgin atrofi dışında normal, torakal ve servikal MRI'da spondilozun yanısıra belirgin spinal kord atrofisi izlendi. EMG'de altda altta daha belirgin sol ekstremitelerde belirgin nörojenik motor ünit değişiklikleri, yer yer fasikülasyonlar dışında normal sınırlarda sinir ileti değerleri elde edildi. Mills sendromunun tanımlanan hemiplejik asendan ALS klinik özelliklerine sahip olmanın yanı sıra, literatürde tanımlanmamış ünilateral serebral atrofisi de bulunan vakamız literatür ışığında tartışıldı.
The Mills' syndrome was first described by Mills in 1900, the syndrome included progressive, ascending or descending hemiplegia, with no significant sensory impairment. Opposing to be a variant of primary lateral sclerosis, this syndrome is accepted as degeneration of the corticospinal tract unilaterally. Fifty six years old, right handed female patient was admitted to our clinic outpatient outpatient clinic with left spastic hemiparesis, dysphagia, dysarthria and bilaterally prominent lower extremity spasticity and behavioral disturbances, sudden loughing, spastic contractility of arms and legs, gait disturbance for the last three years. Following the dysartria, the loss of power belonging to left lower extremity was proceeded by spasticity prominently left side and cognitive impairment with slow progression. On neurological evaluation, she was concious and cooperative partially. The cranial nerves examination were yielded dysphagia and dysphonia, there were spastic plegia on upper and lower left sided extremities. Deep tendon reflexes were hyperactive with Babinski sign on the right. The cerebral MRI showed mild generalised atrophy prominently right sided, cervical MRI showed both spondylosis and significant spinal cord atrophy. EMG investigation revealed significant neurogenic motor unit changes on left sided extremities especially lower area. Our case is carrying a rare clinical specifications of hemiplegic ascending ALS type of Mills and also unilateral cerebral atrophy is discussed in the light of the literature.
The Mills' syndrome was first described by Mills in 1900, the syndrome included progressive, ascending or descending hemiplegia, with no significant sensory impairment. Opposing to be a variant of primary lateral sclerosis, this syndrome is accepted as degeneration of the corticospinal tract unilaterally. Fifty six years old, right handed female patient was admitted to our clinic outpatient outpatient clinic with left spastic hemiparesis, dysphagia, dysarthria and bilaterally prominent lower extremity spasticity and behavioral disturbances, sudden loughing, spastic contractility of arms and legs, gait disturbance for the last three years. Following the dysartria, the loss of power belonging to left lower extremity was proceeded by spasticity prominently left side and cognitive impairment with slow progression. On neurological evaluation, she was concious and cooperative partially. The cranial nerves examination were yielded dysphagia and dysphonia, there were spastic plegia on upper and lower left sided extremities. Deep tendon reflexes were hyperactive with Babinski sign on the right. The cerebral MRI showed mild generalised atrophy prominently right sided, cervical MRI showed both spondylosis and significant spinal cord atrophy. EMG investigation revealed significant neurogenic motor unit changes on left sided extremities especially lower area. Our case is carrying a rare clinical specifications of hemiplegic ascending ALS type of Mills and also unilateral cerebral atrophy is discussed in the light of the literature.
Açıklama
Anahtar Kelimeler
Nörolojik Bilimler
Kaynak
Journal of Neurological Sciences (Turkish)
WoS Q Değeri
Scopus Q Değeri
Cilt
30
Sayı
1
