Third Nerve Palsy

Ptosis; Third Nerve PalsyNight sweats Double visionThe patient is a 50 year old man with Type II Diabetes who presented to an outside hospital in September 1996 with ptosis and an inability to open the right eye. Five months prior to admission (PTA), he had flu-like symptoms with mild fever, sinus congestion and diffuse myalgia in the chest, abdomen, legs and the soles of the feet for approximately two weeks. He consulted his physician who diagnosed "a viral syndrome". In addition to fever he described frequent chills and drenching night sweats. He lost 20 pounds in weight over 3 months. Three days PTA, he woke with ptosis of the right eye and when he held the eyelid up he had double vision worse on gaze left. Concomitantly, he experienced decreased auditory acuity, right ear greater than left and gait instability due to impaired balance. He tended to lean to one side or the other. He denied vertigo, tinnitus and ear ache. For several weeks he had also suffered from a dull bifrontal, throbbing headache which intensified three days PTA. The headache woke him from sleep. He denied photophobia, phonophobia or stiff neck. His wife described a change in his behavior over the past several months and felt that he had become more serious and pessimistic, perhaps even depressed. Over the three days leading up to admission, he became somnolent, lethargic and at times "incoherent" and he began to experience difficulties with short-term memory and word-finding. He was admitted to an outside hospital for investigation. Brain CT: Normal. Lumbar Puncture was attempted unsuccessfully. The patient was transferred to the Massachusetts General Hospital. Past History: No history of drug or alcohol abuse Negative for depression and migraine. Family History: Father died of a myocardial infarct Social History: Works as a consultant Served in the Airforce for two years in his early 20's Walks his dog in the woods in the summer Travels frequently in the US but not abroad. General Examination: Temperature 100.3 degrees, pulse regular, BP 150/80 No neck stiffness, lymphadenopathy or skin rash Neurological Examination: Oriented x3 Impaired short-term memory, 0/3 at 3 minutes Naming intact, able to read Speech normal Neuro-ophthalmological Examination: Visual acuity 20/20 Visual field and fundus examination normal Pupils: OD: dilated at 7mm, non-reactive OS: 2 mm, reacted normally to light and near Ocular Motility OD: Ptosis Paresis of all the muscles innervated by the third nerve Cranial nerves 4 and 6 normal No nystagmus Ocular Motility OS: Normal Cranial nerve 8, decreased hearing to a medium snap bilaterally Motor System: Right pronator drift and slowness of dexterous hand/finger movements. Reflexes 2+ bilaterally, right plantar equivocal, left flexor. Sensory System: Normal. Coordination: No finger/nose or heel/shin ataxia. Gait ataxia, unable to tandem walk. Blood studies: Rapid plasma reagin test reactive at 1 to 64 dilution The fluorescent treponemal antibody absorption (FTA-ABS) test reactive 1 to 120 ESR 68 mm/hr HIV antibody non-reactive Serum IgG 20, IgA 490 (both elevated), IgM 52 The serologic diagnosis of syphilis depends on the demonstration of one or two types of antibodies - non-specific or non-treponemal (reagin) antibodies and specific treponemal antibodies. The commonest test for reagin is the venereal disease research laboratory (VDRL) slide test. If positive the reagin tests of the cerebrospinal fluid (CSF) are diagnostic for neurosyphilis. FTA-ABS test is the one in common use for antibodies that are directed specifically against treponemal antigens. Lumbar puncture: Opening pressure 180 mm water CSF protein 142 mg/dl Sugar 64 mg/dl WBC's 755/cubic mm, 20% polys, 56% lymphs IgA 72.9, IgG 23.2 (both elevated), no banding VDRL reactive at 1 to 2 dilution, RPR reactive at 1 to 64 The CSF abnormalities were those that are commonly found in neurosyphilis i.e.: a. 200 to 300 cells per cubic mm, mostly lymphocytes and a few plasma cells and other mononuclear cells. b. Elevation of the total protein, from 40 to 200mg/dl c. An increase in gammaglobulin (IgG) and d. Positive serologic tests e. The glucose content is usually normal. Audiogram: Moderate to severe sensory neural hearing loss. Brain MRI showed irregularity of the horizontal (A1) anterior cerebral artery segment involving the origin of the recurrent artery of Heubner, which appeared occluded. There was slight beading of the basilar artery and the posterior cerebral arteries, right > left. MRA Head and Neck showed: A beaded basilar artery and right posterior cerebral artery greater than left posterior cerebral artery. Diagnosis: Meningovascular syphilis Microinfarct third nerve Bilateral sensory-neural deafness Meningovascular syphilis should always be considered when a young person has one or several cerebrovascular accidents i.e. the sudden development of hemiplegia, aphasia, sensory loss or mental confusion. The commonest time of onset of meningovascular syphilis is six to seven years after the original infection, but it may be as early as 6 months or as late as 10 to 12 years. Treatment: Crystalline penicillin G intravenously 4 million units q.4.h for 14 days. Hospital Course: The third nerve palsy completely recovered. He returned to his normal baseline and energy level but was left with slight impairment of short term memory and mild word finding difficulty.DiplopiaTo see Brain MRI and MRA view Third Nerve PalsyView ID919-2 for details of the anatomy of the third nerve.Syphilis is caused by a slender, spiral, motile organism, the treponemal pallidum. Patients with meningovascular syphilis are usually found at autopsy to have atherothrombotic or embolic infarction rather than meningovascular syphilis. The changes in the latter disorder consist not only of meningeal infiltrates but also inflammation of arteries and fibrosis, which lead to narrowing and finally occlusion. The vascular lesion was first described by Heubner, hence Heubner's arteritis. In some cases of vascular syphilis, there is a meningo-encephalitis as well. Most of the infarctions occur in the distal territories of medium-and small-caliber lenticulostriate branches that arise from the stems of the middle and anterior cerebral arteries. Most characteristic perhaps is an internal capsular lesion, extending to the adjacent basal ganglia. The presence of multiple small but not contiguous lesions adjacent to the lateral ventricles is another common pattern. Small, asymptomatic lesions are often seen in the caudate and lentricular nuclei. (Please see ref 1 for a complete review)Penicillin is the drug of choice for all varieties of neurosyphilis, both asymptomatic and symptomatic. Crystalline penicillin G given intravenously in a dosage of 18 to 24 million units daily (3 to 4 million units every 4 h) for 14 days is recommended. Erythromycin and tetracycline are suitable substitutes in patients who are sensitive to penicillin. The patient should be re-examined every 3 months and the cerebrospinal fluid retested after a six month interval.1. Ropper AH, Brown RH. Non-viral infections of the Nervous System. Chp 32;599-638 In: Principals of Neurology. Adams RD, Victor M (Eds) 5th Edition, McGraw-Hill, Inc. 1993. 2. Katz DA, Berger JR. Neurosyphilis in acquired immunodeficiency syndrome. Arch Neurol 1989; 46:895-898. http://www.ncbi.nlm.nih.gov/pubmed/275753