Pearls & Oy-sters: An unusual case of varicella-zoster virus cerebellitis and vasculopathy

Abstract

Pearls & Oy-sters: An unusual case of varicella-zoster virus cerebellitis and vasculopathy PEARLS 1. CNS complications of varicella-zoster virus (VZV) occur mainly in immunocompromised or elderly patients and include meningitis, myelitis, acute encephalitis, vasculopathy, and, rarely, cerebellitis. CASE REPORT A 67-year-old woman presented with unsteadiness and dizziness of gradual onset. Two weeks before presentation, the patient had a painful vesicular rash at the left ear followed within 9 days by acute left peripheral facial palsy; a diagnosis of Ramsay Hunt syndrome (RHS) was made and oral valacyclovir was started. A few days after initiating valacyclovir, she developed gait instability, which gradually worsened. Her medical history disclosed well-controlled diabetes mellitus and hypertension, and therapy included metformin, ramipril, hydrochlorothiazide, and acetylsalicylic acid. On neurologic examination, the patient was unable to stand and walk unassisted; she had a wide-based gait, predominantly left-sided limb ataxia, and bilateral dysdiadochokinesia. On cranial nerve examination, the patient had a left lower motor neuron facial palsy and had pain in all 3 branches of the left trigeminal nerve. Deep tendon reflexes, strength, and sensation were normal. Head CT displayed hypodensity of the left medial pons, and, because of suspected CNS involvement by VZV, the patient was treated with IV acyclovir (10 mg/kg 3 times daily) for 10 days. Brain MRI, performed the second day of therapy, revealed one lesion in the left pons, one in the left midbrain, and one in the right periventricular area. All 3 lesions were hyperintense on T2 and diffusionweighted imaging sequences and hypointense on apparent diffusion coefficient, consistent with recent ischemic lesions ( figure). No other supratentorial or cerebellar lesions were seen. Magnetic resonance angiography (MRA) was normal. Absolute lymphocyte count was 550/mL (normal 0.95-4.40) with inversion of the CD41:CD81 T-cell ratio. CSF analysis showed 40 leukocytes/mL (mainly mononuclear cells), 40 erythrocytes/mL, protein 0.46 g/L (normal 0.15-0.45), and glucose 99 mg/dL (normal 45-80). CSF VZV PCR was positive and anti-VZV IgG antibody was detected in the CSF. PCR tests for other neurotropic viruses were negative. Common causes of stroke including atherosclerosis or dissection were excluded. After 2 days of treatment with IV acyclovir, the patient's symptoms improved and at discharge she had no difficulty in standing and walked with unilateral support. Facial pain was treated successfully with carbamazepine 400 mg/day. At the last follow-up 3 months later, the patient walked without support, retained a mild facial weakness, and denied facial pain after she gradually ceased carbamazepine.DISCUSSION We describe a healthy, immunocompetent adult patient who developed herpes zoster that progressed to cerebellar ataxia and trigeminal neuropathic pain. Brain MRI findings of focal ischemic lesions in the brainstem and right hemisphere are inconsistent with cerebellar invo...

Ramsay Hunt Syndrome Associated with Central Nervous System Involvement in an Adult

“…An additional 3 reports described brainstem involvement [ 8 – 10 ]. Only two of these 5 cases involved immunocompetent adults with PCR confirmation of VZV in the CSF [ 6 , 9 ] Similar to our case, both of these patients were females with one of them having well-controlled diabetes. The coexistence of RHS and VZV encephalitis has also been rarely documented in the English-language literature [ 11 – 13 ].…”

“…Acute cerebellar ataxia following VZV infection is a common neurological complication in children; however, it is extremely rare in adults [ 4 , 5 ]. A PubMed literature search limited to the English language and adults until February 2015 revealed only 2 cases of RHS complicated by cerebellitis [ 6 , 7 ]. An additional 3 reports described brainstem involvement [ 8 – 10 ].…”

Neurological complications of varicella zoster virus reactivation

“…In addition, VZV can cause cerebellitis (22, 23). All of these complications can occur in the absence of rash.…”

Brainstem and Cerebellar Involvement in Ramsay Hunt Syndrome

“…VZV involvement of the geniculate ganglion is known as Ramsay Hunt syndrome (RHS), characterized by ipsilateral facial paralysis, otic pain, and herpetic lesions of the external auditory canal and auricle [3]. To the best of our knowledge, there have been 3 prior cases of RHS complicated exclusively by cerebellitis [1,4,5] and 3 prior cases of RHS complicated exclusively by rhombencephalitis [6][7][8]. While isolated involvement of the brainstem, cerebellum, and spinal cord has been discussed in the literature, no reports to our knowledge have described concurrent brainstem and cerebellar involvement [9].…”