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Ultrasonographic Evaluation of the Peripheral Nerves in a Patient with Chronic Neuropathy and Dandy-Walker Syndrome

2011; Lippincott Williams & Wilkins; Volume: 91; Issue: 9 Linguagem: Inglês

10.1097/phm.0b013e31823cab83

1537-7385

Murat Kara, Tülay Tiftik, Fatih Sarı, Melike Dolmuş, Levent Özçakar,

Urologic and reproductive health conditions

A 38-yr-old man (with a diagnosis of Dandy-Walker syndrome [DWS]) was seen for ataxia and progressive increase in his lower limb weakness in the last 4–5 yrs. He did not have any history of diabetes mellitus, vitamin B12 deficiency, renal disease, malignancy, and alcohol abuse, and his family history was noncontributory. Physical examination revealed a broad-based gait and truncal ataxia. He had a weakness in ankle dorsiflexor (3/5) and evertor (3/5) muscles bilaterally. He also had cavus feet and hammer toes. Neurologic examination revealed nystagmus with lateral gaze and intention tremor in his hands. Proprioception and vibration sense were impaired on both lower limbs (worse on the left side). All deep tendon reflexes were diminished/absent. The Babinski sign was present bilaterally. Cerebellar functional assessment revealed disturbances in finger-to-nose and heel-to-shin tests, marked dysmetria and dysdiadochokinesia. The Romberg test also produced positive findings. Nerve conduction studies demonstrated a bilateral absence of median, ulnar, and sural nerve sensory responses and bilateral absence of tibial and peroneal nerve motor responses. Median and ulnar nerve motor amplitudes and conduction velocities (53 m/sec and 57 m/sec, respectively) were normal on both sides. Needle electromyography was consistent with chronic neurogenic involvement. A substantial ultrasonographic imaging for peripheral nerves in all limbs was performed. Despite the widespread electrophysiologic findings, structural changes could be observed only in the sciatic nerves (Fig. 1). The patient was therefore diagnosed to have a chronic sensorimotor polyneuropathy in addition to his previous diagnosis of DWS.FIGURE 1: Comparative ultrasound imaging (axial view at midthigh level) of the sciatic nerves (white arrowheads), demonstrating a larger nerve on the left side (L). Intrinsic anechoic lesions (black arrows) are also observed more predominantly on the left side (L).DWS is a rare congenital malformation of the posterior fossa, with the authors being able to find only one report (of two boys) concerning peripheral nerve involvement in this syndrome.1 Herein is presented the third case with a sensorimotor polyneuropathy. Indeed, the diagnosis of DWS could delay early recognition of concomitant peripheral nerve problems in certain patients. In addition, in presenting the first ultrasonographic images of the peripheral nerves in DWS, we underscore, once again, the convenient and prompt way of peripheral nerve imaging using ultrasound.2–4