Flail arm-like syndrome associated with HIV-1 infection

Abstract

During the last 20 years at least 23 cases of motor neuron disease have been reported in HIV-1 seropositive patients. In this report we describe the clinical picture of a young man with HIV-1 clade C infection and flail arm-like syndrome, who we were able to follow-up for a long period. We investigated and prospectively monitored a 34-year-old man with features of flail arm syndrome, who developed the weakness and wasting 1 year after being diagnosed with HIV-1 infection after a routine blood test. He presented in 2003 with progressive, symmetrical wasting and weakness of the proximal muscles of the upper limb of 2 years' duration. He had severe wasting and weakness of the shoulder and arm muscles. There were no pyramidal signs. He has been on HAART for the last 4 years and the weakness or wasting has not worsened. At the last follow-up in July 2007, the patient had the same neurological deficit and no other symptoms or signs of HIV-1 infection. MRI of the spinal cord in 2007 showed characteristic T2 hyperintense signals in the central part of the spinal cord, corresponding to the central gray matter. Thus, our patient had HIV-1 clade C infection associated with a 'flail arm-like syndrome.' The causal relationship between HIV-1 infection and amyotrophic lateral sclerosis (ALS)-like syndrome is still uncertain. The syndrome usually manifests as a lower motor neuron syndrome, as was seen in our young patient. It is known that treatment with antiretroviral therapy (ART) stabilizes/improves the condition. In our patient the weakness and atrophy remained stable over a period of 3.5 years after commencing HAART regimen.

Keywords: Flail arm syndrome; HIV-1; HIV-1 clade C; amyotrophic lateral sclerosis; highly active antiretroviral therapy.

Figure 1

(a) The patient at first evaluation showed severe wasting and weakness of the shoulder and arm muscles with flail arm–like syndrome. (b) Subtype C PCR. Lane 1: DNA ladder, lane 2: Patient's sample, lane 3: positive control, lane 4: negative control. The 138-bp band represents HIV-1 subtype C, while the 232-bp band represents HIV-1 common band. (c) At 3.5 years' follow-up, the wasting and weakness was stationary on HAART therapy

Figure 2a and b

(a) Axial T2-weighted image at C1 vertebral level showing two focal T2 hyperintensities in the central part of spinal cord corresponding to the central grey matter of the cord. (b) Sagittal T2-weighted image showing ill-defined linear hyperintensity of the spinal cord extending between C1 and C4 vertebral body levels