A case of linear scleroderma involving cerebellum with vertigo

Abstract

Linear scleroderma is a kind of disease that can cause complication of the central nervous system. Sometimes, ipsilateral intracerebral or white matter lesions in the brain magnetic resonance imaging are noted. Nystagmus is important for the differential diagnosis of dizziness. Positional nystagmus was classified into regular direction nystagmus, direction changing positional nystagmus (DCPN) and irregular nystagmus by their character. DCPN is defined as a nystagmus that changes its direction with different head and body positions, and it can be the sign of lesion in the central vestibular system. Recently, we experienced a 17-year-old woman who had a scalp linear scleroderma, treated for about 10 years and showed DCPN induced by positional nystagmus test. We report a case of linear scleroderma accompanied by cerebellar lesion with a brief review of literature.

Keywords: Dizziness; Linear scleroderma; Positional nystagmus.

Fig. 1

Pure tone audiometry. Right hearing was normal and sensorineural hearing loss was noted at left side, about 53 dB HL in air conduction.

Fig. 2

Spontaneous nystagmus to right (6.9 deg/s) under video nystagmography on the 1st OPD visit day (upper line: vertical nystagmus, lower line: horizontal nystagmus). OPD: outpatient department.

Fig. 3

Videonystagmography on the second hospital day. Right beating nystagmus (25 deg/s) was shown when rolling head to right (A).When rolling head to left, right beating nystagmus (25 deg/s) was shown during 1-58 seconds (B), but the direction of nystagmus was changed to left side (20 deg/s) at the point of arrow, after 58 soconds (C) (upper line: vertical nystagmus, lower line: horizontal nystagmus).

Fig. 4

Caloric test. Caloric test shows canal paresis (19%) of left side. SPV: slow phase peak velocity.

Fig. 5

Axial scans of brain magnetic resonance image and computed tomography. A: On the T2 weighted image of 2004, several signal void lesions were found on posterior lobe of left cerebellar hemisphere. B: The area of signal void lesions seemed to be enlarged on 2011. C: There were white calcified parenchymal lesions on the same site of left cerebellum on brain CT with enhancement.