HIV-1 clade-C-associated “ALS”-like disorder: first report from India

Sinha, Sanjib; Mathews, Thomas; Arunodaya, G. R.; Siddappa, Nagadenahalli B.; Ranga, Udaykumar; Desai, Anita; Ravi, Vasanthapuram; Taly, AB

doi:10.1016/j.jns.2004.06.006

Cited by 26 publications

(22 citation statements)

References 10 publications

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“…During the last 20 years, 19 cases (male 16, female 3) of HIV-associated ALS syndrome have been reported [8][9][10][11][12][13][14][15][16][17][18][19][20][21] ( Table 1). Four of these cases ( [8,15,16] (case 1), [18]) fulfilled the El Escorial [23] criteria for clinically definite ALS and the remainder [9,11 -14,17,19-21] were clinically probable or possible cases of ALS.…”

Section: Review Of Cases Of Als Syndrome In Patients With Hiv Infectionmentioning

confidence: 99%

“…Most patients with HIV disease and isolated lower motor neuron syndrome showed clinical or laboratory evidence of involvement outside the motor system and most who received HAART showed at least partial improvement (Table 1). Sinha et al [21] reported a 37-year-old, HIV-infected man with a predominant lower motor neuron disorder and tuberculosis. The patient showed temporary improvement in motor deficit following antiretroviral and antitubercular therapy.…”

Section: Review Of Cases Of Als Syndrome In Patients With Hiv Infectionmentioning

confidence: 99%

“…Additionally, other forms of motor neuron disease, such as, brachial amyotrophic diplegia, have also been seen with this infection [22]. Higher frequency of ALS syndrome in HIV seropositive population [16] and reports of slowing [17,19,21], arrest ( [13,16] (case 1)), or occasional reversal ( [15,16] (cases 2 and 6)) of motor deficit following antiretroviral therapy in these cases have been proposed as evidence for the causal relationship between the HIV infection and ALS. Retrovirus hypothesis of ALS is inherently attractive because retroviruses replicate by synthesizing and integrating a DNA copy that persists into the host's genome.…”

Section: Introductionmentioning

confidence: 99%

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ALS syndrome in patients with HIV-1 infection

Verma

Berger

2006

Journal of the Neurological Sciences

100

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Section: Review Of Cases Of Als Syndrome In Patients With Hiv Infectionmentioning

confidence: 99%

Section: Review Of Cases Of Als Syndrome In Patients With Hiv Infectionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

ALS syndrome in patients with HIV-1 infection

Verma

Berger

2006

Journal of the Neurological Sciences

100

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“…A direct link between ALS and the lymphohematopoietic system comes from the clinical observation that ALS patients had marked lymphopenia with reductions in CD2 + and CD8 + T cells in comparison to agematched normal controls (Provinciali et al, 1988). The "ALS"-like disorder seen in patients with HIV infection further suggests that reduced immunity with viral infection might be a key element in the pathogenesis of ALS (Verma and Berger, 2006;Sinha et al, 2004). Most recently, a significant decrease of white blood cells was observed in the SOD1 G93A transgenic mouse model of ALS, further supporting the role of immunodeficiency in ALS (Kuzmenok et al, 2006).…”

Section: Introductionmentioning

confidence: 99%

Mice deficient in the ALS2 gene exhibit lymphopenia and abnormal hematopietic function

Erie

Shim

Smith

et al. 2007

Journal of Neuroimmunology

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One form of juvenile onset autosomal recessive amyotrophic lateral sclerosis (ALS2) has been linked to the dysfunction of the ALS2 gene. The ALS2 gene is expressed in lymphoblasts, however, whether ALS2-deficiency affects periphery blood is unclear. Here we report that ALS2 knockout (ALS2 −/− ) mice developed peripheral lymphopenia but had higher proportions of hematopoietic stem and progenitor cells in which the stem cell factor-induced cell proliferation was up-regulated. Our findings reveal a novel function of the ALS2 gene in the lymphopoiesis and hematopoiesis, suggesting that the immune system is involved in the pathogenesis of ALS2.

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“…A direct link between ALS and the lymphohematopoietic system comes from clinical observations that ALS patients had marked lymphopenia with reductions in CD2 + and CD8 + T cells in comparison to age-matched controls [37]. An HIV-associated ALS disorder seen in many patients further suggests that reduced immunity from viral infections may play a key role in ALS pathogenesis [38,39]. Recently, a significant decrease of white blood cells was observed in the SOD1 G93A transgenic mouse model of ALS, further supporting a link between immunodeficiency and ALS disease onset [40].…”

Section: Als2 Knockout Micementioning

confidence: 99%

Alsin and the Molecular Pathways of Amyotrophic Lateral Sclerosis

2007

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Autosomal recessive mutations in the ALS2 gene lead to a clinical spectrum of motor dysfunction including juvenile onset amyotrophic lateral sclerosis (ALS2), primary lateral sclerosis, and hereditary spastic paraplegia. The 184-kDa alsin protein, encoded by the full-length ALS2 gene, contains three different guanine-nucleotide-exchange factor-like domains, which may play a role in the etiology of the disease. Multiple in vitro biochemical and cell biology assays suggest that alsin dysfunction affects endosome trafficking through a Rab5 small GTPase family-mediated mechanism. Four ALS2-deficient mouse models have been generated by different groups and used to study the behavioral and pathological impact of alsin deficiency. These mouse models largely fail to recapitulate hallmarks of motor neuron disease, but the subtle deficits that are observed in behavior and pathology have aided in our understanding of the relationship between alsin and motor dysfunction. In this review, we summarize recent clinical and molecular reports regarding alsin and attempt to place these results within the larger context of motor neuron disease.

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HIV-1 clade-C-associated “ALS”-like disorder: first report from India

Cited by 26 publications

References 10 publications

ALS syndrome in patients with HIV-1 infection

ALS syndrome in patients with HIV-1 infection

Mice deficient in the ALS2 gene exhibit lymphopenia and abnormal hematopietic function

Alsin and the Molecular Pathways of Amyotrophic Lateral Sclerosis

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