Author(s): Shams PN, Waldman A, Plant GT
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Abstract A 54 year old man is described with signs compatible with ocular myasthenia gravis and an apparent excellent response to pyridostigmine. Subsequent clinical progression and further investigation suggested the presence of an inflammatory brain stem lesion, which responded to corticosteroid therapy. Clinical relapse, including the development of central neurogenic hyperventilation, led to a brain stem biopsy, confirming a diagnosis of B cell lymphoma. This case illustrates the propensity of primary CNS lymphoma (PCNSL) to mimic other conditions. Brain MRI is mandatory in presumed "test negative" ocular myasthenia with atypical clinical findings. Spontaneous regression of PCNSL or response to corticosteroids is common and should not mitigate against the diagnosis. Histopathological confirmation should ideally be made before starting therapy, as this may obscure or delay the correct diagnosis. Although PCNSL is rare, it must be considered in all patients with brain stem syndromes, and in all patients 50 years or older with contrast enhancing focal lesions.
This article was published in J Neurol Neurosurg Psychiatry
and referenced in Journal of Brain Tumors & Neurooncology