| History |
A 52-year-old right-handed salesman presented in July 2007 with a 5-year history of slowly progressive imbalance. In 2002 he developed difficulty using his eyes together and then imbalance resulting in frequent backward falls. He reported oscillopsia and episodic vertigo. He later developed fatigue, dysarthria, memory and concentration complaints, weight loss, numbness of his right foot, easy bruising, and neurasthenia. He had a brother with trouble walking. After an extensive evaluation, he was diagnosed with spinocerebellar ataxia. On presentation in 2007 he had normal vision, saccadic pursuit, slow saccades, gaze-evoked rotatory and downbeat nystagmus, abnormal VOR suppression, esotropia with trace bilateral abduction deficits, and intermittent square-wave jerks. His neurologic exam showed dysarthria, a wide-based ataxic gait, unsteady tandem walk, absent ankle reflexes, and decreased peripheral sensation. Past medical history included hypertension, mononucleosis, hepatitis, pericarditis, and squamous cell cancer. Treatment with acetazolamide and memantine were not helpful. Symptoms worsened despite physical and occupational therapy. He became disabled and wheelchair bound. Brainstem evoked responses were abnormal. Nerve conduction studies, swallow evaluation, and neuropsychological testing were normal. Repeat MRI was read as normal except for microvascular disease. He had normal or negative CBC with differential, ESR, CMP, TSH, thyroxine, B1, B12, Vitamin D, Vitamin E, SPEP/IEP, mercury, arsenic, cadmium, UPEP, UA, ANA, B. Henselelae, B. Quintana, Lyme, RPR, FTA-Abs, M. pneumonia, ANCA, Toxoplasma, SSA and SSB, C. burnetii, AFB, M. tuberuculosis antibody, Quantiferon gold, Tropheryma whipplei, HCV, HSV, JCV, Rocky mountain spotted fever, VZV, HIV-1, HIV-2, EBV. He had a low absolute CD4 with low CD4:CD8 ratio, normal anticardiolpin IgG but high anticardiolipin IgM, high parvovirus B19 IgG but normal IgM, and normal CSF studies except high HSV IgG and a total protein of 65. CSF cytology revealed no malignant cells. |
| References |
1. Hajj-Ali RA, Calabrese LH. "Primary angiitis of the central nervous system." Autoimmunity Reviews. 2013;12:463-466. 2. Salvarani C, Brown RD Jr, Hunder GG. "Adult primary central nervous system vasculitis." Lancet. 2012 Aug 25;380(9843):767-77. 3. Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Weigand SD, Miller DV, Giannini C, Meshia JF, Huston J 3 rd , Hundger GG. "Primary central nervous system vasculitis: analysis of 101 patients." Ann Neurol. 2007 Nov;62(5):442-51. 4. Pizzanelli C, Catarsi E, Pellicia V, Cosottini M, Pesaresi I, Puglioli M, Moretti P, Tavoni A. "Primary angiitis of the central nervous system: Report of eight cases from a single Italian center." J Neurolog Sci. 2011;307:69-73. |
