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Flail arm-like syndrome associated with HIV-1 infection.

Nalini A, Desai A, Mahato SK - Ann Indian Acad Neurol (2009)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India.

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.

No MeSH data available.


Related in: MedlinePlus

(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
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Figure 0002: (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

Mentions: The patient was started on antiretroviral therapy in November 2003 with a combination of lamivudine (150 mg), stavudine (30 mg), and nevirapine (200 mg) and is currently on follow-up. The progression of the wasting and weakness had almost ceased after commencing HAART (highly active antiretroviral therapy) and at the end of 3.5 years follow-up the patient's symptoms continued to be stable; also, he did not develop any other illness related to HIV-1 infection. He was re-evaluated in July 2007. His upper limb proximal muscle weakness and wasting had not progressed [Figure 1c]. His cognitive functions were normal. He underwent multimodal evoked potentials, including visual evoked potentials, brainstem auditory response, and somatosensory evoked potential, all of which were normal. CSF protein and glucose level and cell count were normal. A repeat brachioradialis muscle biopsy showed only neurogenic atrophy. The plasma viral load was undetectable; unfortunately, we had not checked the plasma viral load during 2003 so we cannot comment on this. Serum protein electrophoresis was normal. Chest x-ray showed no abnormal findings. MRI of the brain and spinal cord was done on a 3 Tesla machine. Brain scan showed evidence of diffuse cerebral and cerebellar atrophy. Signal intensity changes in the form of hyperintensity in T2-weighted and FLAIR images and isointensity in T1- weighted images were seen in the white matter of both superior frontal and precentral gyri and periventricular white matter; there was no contrast enhancement. T2- weighted axial slices through the cervical cord revealed symmetrically distributed hyperintense foci in the central part of the spinal cord. A part of these lesions was hypointense on T1-weighted images. [Figure 2a] The sagittal T2-weighted image showed an ill-defined linear hyperintensity in the central part of the spinal cord, extending between C1 and C4 vertebral body levels [Figure 2b]. His repeat CD4 and CD3 counts were 460/ mm3 and 2506/mm3, respectively.


Flail arm-like syndrome associated with HIV-1 infection.

Nalini A, Desai A, Mahato SK - Ann Indian Acad Neurol (2009)

(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
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2812739&req=5

Figure 0002: (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
Mentions: The patient was started on antiretroviral therapy in November 2003 with a combination of lamivudine (150 mg), stavudine (30 mg), and nevirapine (200 mg) and is currently on follow-up. The progression of the wasting and weakness had almost ceased after commencing HAART (highly active antiretroviral therapy) and at the end of 3.5 years follow-up the patient's symptoms continued to be stable; also, he did not develop any other illness related to HIV-1 infection. He was re-evaluated in July 2007. His upper limb proximal muscle weakness and wasting had not progressed [Figure 1c]. His cognitive functions were normal. He underwent multimodal evoked potentials, including visual evoked potentials, brainstem auditory response, and somatosensory evoked potential, all of which were normal. CSF protein and glucose level and cell count were normal. A repeat brachioradialis muscle biopsy showed only neurogenic atrophy. The plasma viral load was undetectable; unfortunately, we had not checked the plasma viral load during 2003 so we cannot comment on this. Serum protein electrophoresis was normal. Chest x-ray showed no abnormal findings. MRI of the brain and spinal cord was done on a 3 Tesla machine. Brain scan showed evidence of diffuse cerebral and cerebellar atrophy. Signal intensity changes in the form of hyperintensity in T2-weighted and FLAIR images and isointensity in T1- weighted images were seen in the white matter of both superior frontal and precentral gyri and periventricular white matter; there was no contrast enhancement. T2- weighted axial slices through the cervical cord revealed symmetrically distributed hyperintense foci in the central part of the spinal cord. A part of these lesions was hypointense on T1-weighted images. [Figure 2a] The sagittal T2-weighted image showed an ill-defined linear hyperintensity in the central part of the spinal cord, extending between C1 and C4 vertebral body levels [Figure 2b]. His repeat CD4 and CD3 counts were 460/ mm3 and 2506/mm3, respectively.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India.

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.

No MeSH data available.


Related in: MedlinePlus