Clinical Reasoning: A 57-year-old woman with ataxia and oscillopsia

Abstract

A 57-year-old immunosuppressed renal transplant recipient experienced 2 months of oscillopsia, vertigo, ataxia, and headaches. The headaches were described as constant, bifrontal, dull, and associated with photophobia and nausea but no aura or positional component. She described rotational vertigo, both at rest and provoked by leftward head turn and gait instability that developed gradually over the last 4 weeks. Six months prior to presentation, she had a similar episode of headaches, dizziness, and ataxia that resolved spontaneously over 3 months. This was preceded by a dermatomal vesicular rash on the arm. She had undergone renal transplantation 12 years earlier. Three years ago, she had left C2-3 distribution zoster. She denied infectious or rheumatologic symptoms. Her only current medication was mycophenolate mofetil 360 mg PO BID.Neurologic examination. Visual acuity was 20/2022 OS and 20/20 OD. Extraocular movements were full without nystagmus. All attempts at fixation led to intermittent bursts of ocular flutter. There were also hypometric saccades to the left and hypermetric saccades to the right (video on the Neurology ® Web site at Neurology.org). Dix-Hallpike and other otolith repositioning maneuvers did not elicit vertigo or nystagmus. There was intention tremor in both arms without dysdiadochokinesia or dysmetria. Gait was wide-based and ataxic and the patient was unable to walk tandem without falling to the left. Romberg sign was absent and the remaining neurologic and general examinations were normal.

Infections of the Spine and Spinal Cord

“…Although PCR is more rapidly available, given brief/transient periods of viremia, IgM/IgG may be more sensitive. 73 Transition to IV acyclovir produced rapid resolution of her symptoms. The role of glucocorticoids in zoster myelitis has not been clearly elucidated, although in many cases, glucocorticoids are often offered based on case reports and expert opinion.…”

“…When central nervous system infection with VZV is suspected, CSF VZV PCR should be evaluated, as should CSF VZV IgM/IgG. Although PCR is more rapidly available, given brief/transient periods of viremia, IgM/IgG may be more sensitive 73 . Transition to IV acyclovir produced rapid resolution of her symptoms.…”

“…However, pathologic involvement may be most severe in the dorsal root entry zone, and an infectious/necrotizing vasculitis with demyelination and neuronal inclusions has been described (CASE 4-3). 72,73 Patients with VZV myelitis often develop the characteristic zoster rash before the onset of myelitis, but myelitis can occur without rash as well (zoster sine herpete). Myelitis may be accompanied by encephalitis and/or radiculitis (including Elsberg syndrome; see below in the section titled "Conus Medullaris/Cauda Equina Syndrome"), and manifestations may be severe, particularly in patients who are immunocompromised.…”

“…The typical presentation is acute to subacute asymmetric weakness more often accompanied by spinothalamic tract sensory deficits than dorsal column dysfunction. However, pathologic involvement may be most severe in the dorsal root entry zone, and an infectious/necrotizing vasculitis with demyelination and neuronal inclusions has been described ( case 4-3 ) 72,73 . Patients with VZV myelitis often develop the characteristic zoster rash before the onset of myelitis, but myelitis can occur without rash as well (zoster sine herpete).…”

Emergency Evaluation and Management of Encephalitis and Myelitis in Adults

“…108 VZV can be challenging to diagnose, particularly in more indolent cases, and both VZV PCR and VZV IgG/IgM should be obtained. 118 Treatment consists of IV acyclovir, and glucocorticoids are often considered, especially in the presence of vasculitis.…”