Kasus Serial Sindrom Hemichorea-Hemiballismus terkait Non-Ketotik : Tantangan dalam Proses Diagnosis

Laksmi Sasiarini, Rahmad Budianto, Ricky C. Tarigan

Abstract

Diabetes melitus merupakan penyakit yang banyak ditemui sehari-hari, akan tetapi memiliki manifestasi klinis yang tidak lazim. Salah satu manifestasinya adalah sindroma hemichorea-hemiballismus, spektrum gerakan involunter yang berlangsung terus-menerus tanpa pola dan melibatkan satu sisi tubuh akibat hiperglikemia non-ketotik pada diabetes yang tidak terkontrol. Dilaporkan dua kasus pasien diabetes dengan hiperglikemia non-ketotik yang mengalami sindrom hemichorea-hemiballismus. Kasus pertama – wanita 57 tahun mengalami gerakan involunter, repetitif, dan tidak berirama di lengan dan tungkai kanan, disertai kedutan di wajah kanan selama dua minggu. Pasien tersebut memiliki riwayat diabetes melitus tidak terkontrol. Kasus kedua – laki-laki 60 tahun dengan kejang umum tonik-klonik. Pasien mengalami gerakan involunter pada lengan kanan selama empat hari dan riwayat diabetes sebelumnya tidak diketahui. Terapi diazepam intravena tidak memberikan respons terhadap kejang. Gambaran CT scan kepala pada kedua pasien menunjukkan lesi hiperdens pada ganglia basalis yang diduga disebabkan oleh hiperglikemia non-ketotik, akan tetapi lesi hiperdens pada pasien kedua tampak lebih luas. Gerakan involunter membaik setelah target glukosa darah tercapai dengan rehidrasi dan insulin intravena kontinyu. Respons klinis pada kasus hemichorea-hemiballismus di atas bersifat reversibel meskipun gambaran lesi hiperdens dapat bertahan selama berbulan-bulan.

 

Kata Kunci: Hiperglikemia non-ketotik, Insulin, Sindrom hemichorea-hemiballismus

 

A Case Series Hemichorea-Hemiballism Syndrome Related to Nonketotic Hyperglycemia: A Challenge to Diagnostic Process

Diabetes mellitus is quite familiar to health-care personnel but occasionally presents with unusual manifestations. One of them is hemichorea-hemiballismus syndrome, a spectrum of involuntary, continuous non-patterned movement involving one side of the body which is related to nonketotic hyperglycemia in uncontrolled diabetes. Here we present two cases of nonketotic hyperglycemic diabetic patients who presented with the hemichorea-hemiballismus syndrome. First – a 57 years old woman was presented with involuntary, arrhythmic, and repetitive movement on the right arm and leg, accompanied by twitching on the right face for two weeks. She had an uncontrolled history of diabetes mellitus. The second- a 60 years old man was presented with recurrent general tonic-clonic seizure for about four days. The history of diabetes was unknown. Initial diagnosis on admission was general tonic-clonic seizure due to thrombotic cerebrovascular accident (CVA). Intravenous diazepam did not give any response to the seizures. Their brain CT scan imaging showed non-enhancing hyperdensities in the basal ganglia due to non-ketotic hyperglycemia, but the lesion was more extensive on the second patient. An involuntary movement was resolved after achieving blood glucose target by rehydration and continuous intravenous insulin therapy. Clinical response in these cases were reversible while the hyperdensities lesion will be persistent for a few months.

 

Keywords

Hemichorea-hemiballism syndrome, Insulin, Non-ketotic hyperglycemia

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