Brachial Amyotrophic Diplegia in a Patient With Human Immunodeficiency Virus Infection

Abstract

lthough amyotrophic lateral sclerosis and progressive spinal muscular atrophy have been recognized to occur in association with human immunodeficiency virus infection, to our knowledge, brachial amyotrophic diplegia, a form of segmental motor neuron disease, has not been previously reported. Brachial amyotrophic diplegia results in severe lower motor neuron weakness and atrophy of the upper extremities in the absence of bulbar or lower extremity involvement, pyramidal features, bowel and bladder incontinence, and sensory loss. We describe a human immunodeficiency virus-seropositive man without severe immunosuppression or prior AIDS-defining illnesses who had brachial amyotrophic diplegia. This disorder may represent one end of a spectrum of motor neuron diseases occurring with this retrovirus infection.

Acute lower motor neuron syndrome and spinal cord gray matter hyperintensities in HIV infection

“…Although 2 HIV-positive patients with BAD have been reported to have gray matter spinal cord abnormalities on MRI, the patients described in this report had rapidly evolving neurologic syndromes characterized by distal upper limb weakness heralded by pain. 7 , 9 The clinical, electrophysiologic, and neuroimaging characteristics of the patients we report may be conceptualized as an acute segmental cervical poliomyelopathy. The upper limb pain and weakness experienced by our patients led us to consider the possibility that they might have had a manifestation of brachial plexus neuropathy or neuralgic amyotrophy (i.e., Parsonage-Turner syndrome), as has been described in HIV-infected patients.…”

“… 5 , 6 A rare LMN disorder known as brachial amyotrophic diplegia (BAD) has also been described in HIV-positive patients, including those with undetectable viral loads. 7 – 9 It is characterized by gradually progressive proximal upper extremity weakness and wasting and is also referred to as “flail arm syndrome.” 10 , 11 LMN disease is more common when HIV infection is poorly controlled and symptoms stabilize or reverse after ART initiation, as occurred in our first patient before his subsequent decline 2 years later. 2 …”

Flail arm-like syndrome associated with HIV-1 infection

“…[12] Furthermore, there are several reports describing stabilization/recovery/improvement of ALS symptoms after starting treatment with HAART. [461415] Our patient, who has been receiving HAART since 3.5 years, has also not shown progression in the neurological deficits nor has he developed fresh systemic or neurological symptoms related to HIV-1 infection. This patient is still independent for most activities of daily living and his motor disability has remained the same as it was at the start of HAART.…”

“…Although HIV-1–associated MND may be indistinguishable from classical amyotrophic lateral sclerosis (ALS),[2–4] more frequently it is characterized by a variably progressive lower motor neuron disorder affecting the limb or bulbar muscles. [4–6] The flail arm syndrome, a variant of MND, is characterized by a relatively symmetric involvement of the proximal muscles of both arms, which progresses to severe wasting and functional disability; there is, however, little or no weakness of the leg or bulbar muscles at presentation. [7] The median survival of these patients is reported to be 57 months.…”

"Person in the barrel" syndrome: Unusual heralding presentation of squamous cell carcinoma of the lung

“…[ 9 ] Anterior horn cell may be infected by viruses like enterovirus, HTLV, Japanese encephalitis virus, West Nile virus. [ 10 11 12 ] Similar syndrome of bi-brachial weakness was observed in human immunodeficiency virus (HIV) patients due to destruction of anterior horn cells, either by direct invasion or by cytokines. This weakness may occasionally respond to anti-retroviral therapy.…”