Neurogenic weakness, ataxia and retinitis pigmentosa (NARP) syndrome is a rare maternally inherited mitochondrial disorder. Radiologic findings in NARP syndrome are varied; they include cerebral and cerebellar atrophy, basal ganglia abnormalities, and on rare occasions, leukoencephalopathy. This article describes an extremely rare case of NARP syndrome mimicking mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS).
Ataxia ; Atrophy ; Basal Ganglia ; Leukoencephalopathies ; Magnetic Resonance Imaging ; MELAS Syndrome ; Mitochondrial Diseases ; Retinitis Pigmentosa

Ramsay Hunt syndrome with the complication of encephalitis or meningoencephalitis is rarely reported and uncommon in immunocompetent patients. The radiological manifestations of such cases usually involve the cerebellum and brainstem or exhibit the absence of any abnormality. We report a case of a 78-year-old immunocompetent man hospitalized with Ramsay Hunt syndrome, who later developed meningoencephalitis. The cerebrospinal fluid-study excluded other causes of meningoencephalitis, and the clinical diagnosis indicated varicella zoster virus meningoencephalitis. Magnetic resonance imaging revealed increased signal intensities in the bilateral temporal lobe, midbrain, and pons on T2-weighted imaging, and T2 fluid attenuated inversion recovery and contralateral asymmetric pachymeningeal enhancement. Contrast-enhanced T1-weighted imaging revealed ipsilateral facial nerve enhancement.
Aged ; Brain Stem ; Cerebellum ; Diagnosis ; Encephalitis ; Facial Nerve ; Herpes Zoster Oticus ; Herpesvirus 3, Human ; Humans ; Magnetic Resonance Imaging ; Meningoencephalitis ; Mesencephalon ; Pons ; Temporal Lobe