PAROXYSMAL DISKINESIAS:
CLINICAL FEATURES AND CLASSIFICATION
Bao XH, Pei ZY, Qin J, Wu XR
Department
of Pediatrics,
First
Hospital of Peking University, Beijing 100034, China
Objective: In order to disclose the clinical features and the potential
etiology of each kind of paroxysmal diskinesias, the authors reviewed the
cases that were diagnosed in the past 5 years with respect to attack
characteristics, etiology and therapeutic response.
Methods: The clinical studies were done on 9 patients, including the
clinical manifestation, the factors of inducement, duration and frequencies
of episode, therapeutic response, etc. Etiology studies included video-EEG
monitoring, brain MRI/CT, serum ceruloplasmin level, etc. A follow-up of
six months to 5 years was conducted.
Results: Among 9 patients, five belonged to paroxysmal kinesigenic
choreoathetosis (PKC), four belonged to paroxysmal distonic choreoathetosis
(PDC). All of the patients with PKC were induced by sudden movement. They
had shorter duration and higher frequency than that with PDC. Video-EEG
test found epileptic discharge in three patients of PKC. No abnormalities
were found in the patients with PDC. The antiepileptic drugs were very
effective to PKC, and useless to PDC. Brain MRI/CT and serum ceruloplasmin
level was normal in all patients.
Conclusion: PKC is
different from PDC in respect of factors of inducement, frequency and
duration of episode and the treatment. The epileptic discharge and the
dramatic response to antiepileptic drugs in children with PKC indicated PKC
might be probably associated with epilepsy in pathogenesis.