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Neurosyphilis & the Clinical Motor Speech & Cognitive-Linguistic

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Neurosyphilis & the Clinical Motor Speech & Cognitive-Linguistic By: Amanda Fowler Sarah O’Brien‐Ford What is Syphilis? y From the Greek word “syphlos”, meaning crippled or maimed y A sexually transmitted disease caused by the bacteria Treponema palladium y Referred to as the “Great Imitator” y Prevalence: In the U.S. alone, health officials reported over 36,000 cases in 2006 y Primarily occurs in individuals 20‐39 years of age http://www.merck.com/mmpe/sec14/ch194/ch194i.html What is Neurosyphilis? y An infection of the brain or spinal cord y Occurs in individuals who have untreated syphilis y Caused by the bacteria Treponemapallidum, which causes syphillis y Develops about 10 ‐ 20 years after a person is first infected with syphilis y Not everyone who has syphilis will develop this complication (6.5% of untreated syphilis cases lead to neurosyphilis). http://www.nlm.nih.gov/medlineplus/ency/article/000703.htm#visualContent Symptoms of Neurosyphilis y Abnormal gait •Loss of muscle function y Blindness •Mental decline y Confusion •Paralysis •Poor concentration y Dementia •Seizures y Depression •Stiff neck y Headache •Tremors y Incontinence •Visual disturbances y Inability to walk •Weakness/numbness of the lower extremities y Irritability http://www.ninds.nih.gov/disorders/aids/detail_aids.htm http://www.nlm.nih.gov/medlineplus/ency/article/000703.htm#visualContent Diagnostic Tests & Exams y Confirmatory Signs include: y Muscle atrophy y Muscle contractions y Abnormal reflexes y Blood tests to detect substances produced by the bacteria that cause syphilis y Other tests include: y Fluorescent treponemal antibody absorption (FTA‐ ABS) y Rapid plasma reagin (RPR) y Treponemapallidum particle agglutination assay (TPPA) y * it is important to test the spinal fluid for signs of syphilis Tests to look for problems with the nervous system include… y Cerebral angiogram (uses dye & x‐rays to examine how blood flows through the brain) y Head CT scan y Lumbar puncture (“Spinal tap”) y MRI scan of brain, brainstem, or spinal cord 4 forms of Neurosyphilis y Asymptomatic y General Paresis (Parenchymatous or dementia paralytica) y Meningovascular y TabesDorsalis (Locomotor ataxia) Cognitive‐Linguistic & Motor Speech Symptoms of General Paresis Neurosyphilis y Results when chronic meningoencephalitis causes destruction of cortical parenchyma y Develops 15 to 20 yr after initial infection and typically does not affect patients before they are 40‐50 years old y Behavior progressively deteriorates, sometimes resembles a mental disorder or dementia y Irritability, difficulty concentrating, deterioration of memory, defective judgment, headaches, insomnia, fatigue, and lethargy are common y Seizures, aphasia, and transient hemiparesis are possible. Patients may become emotionally unstable and depressed and have delusions y Tremors of the mouth, tongue, hands, and whole body may occur; other signs include pupillary abnormalities, dysarthria, hyperreflexia, and, in some patients, extensor plantar responses Cognitive‐Linguistic & Motor Speech Symptoms of Tabesdorsalis (locomotor ataxia) y Involves slow, progressive degeneration of the posterior columns and nerve roots y Develops 20 to 30 yr after initial infection y Usually the earliest symptom is an intense, pain in the back and legs that occurs irregularly y Gait ataxia, hyperesthesia, and paresthesia may produce a sensation of walking on foam rubber y Most patients with tabesdorsalis are thin and have characteristic sad facies and Argyll Robertson pupils (pupils that accommodate for near vision but do not respond to light) y Optic atrophy may occur y Hypotonia, hyporeflexia, impaired vibratory and joint position sense, ataxia in the heel‐shin test, absence of deep pain sensation, and Romberg's sign y Tabesdorsalis tends to be intractable even with treatment Medical Treatment of Neurosyphilis: y Penicillin y Alternative = parenteralceftriaxone y Given in various ways: y Injected y Probenecid (a drug that increases uric acid) y Follow‐up y blood tests and lumbar punctures for CSF fluid analysis at 3, 6, 12, and 24 months to make sure the infection is gone Prognosis y Treatable if detected early y However, it is still considered a life‐threatening complication of syphilis y The success of treatment is dependent on the severity level of the neurosyphilis before treatment is given Shann& Wilson, 2003 Motor Speech Symptomatology in patients with Neurosyphilis: y Possible cranial nerves (V, XII) involvement y Flaccid &Ataxic (28%) y dysarthrias, and progressive supranuclear palsy y Other neurological problems that affect speech and voice (Duffy, 2005) Management of Flaccid Dysarthria in Patients with Neurosyphilis: y Due to muscle weakness, unique treatments tend to focus on: y Increasing strength y Compensation for muscle weakness y Postural adjustments and/or prosthetic aids may be used in treatment y Treatments should be aimed at the following components of speech: y respiratory y phonatory y Resonatory y articulatory (Duffy, 2005) Management of Flaccid Dysarthria in Patients with Neurosyphilis Continued: y Symptom: possible reduced loudness y Treatment for increasing respiratory function includes: y Deep inhalation y Controlled exhalation y Inspiratory monitoring y Increased force y Treatment for closing the vocal folds y Possible surgery (i.e. arytenoid adduction surgery) y Voice amplifiers (temporary or permanent) y Palatal lift treatment y Pharyngeal flap surgery (Duffy, 2005) Case Study: y 61 year old man, served abroad in the armed forces y Diagnosis of symptomatic neurosyphilis (Sept. 1997) y Diagnosed with expressive dysphagia and a left sided hemiparesis Symptoms: y “Out of character” behavior y Impaired use of right hand y Marked short‐term memory loss y CSF spaces disproportionate to patient’s age y Frequent falls and delusions Treatment: y Allergic to penicillin; treated with ceftriaxone. Recived1 gram 1x/day for 14 days (Shann, 2003) Neurosyphilis Effects on Vision: y Ophthalmic: Blurred vision, reduced color perception, impaired acuity, visual dimming & photophobia 17% y Pupillarychanges: Argyll Robertson pupils 43% y Optic atrophy 7% (Timmermans, 2004) y A 44 year old man with neurosyphilis reported that his vision was “like looking through tissue paper” in his left eye (Smith et al., 2006, p. 36) y Neurosyphilis: Pupil References y Delli, F.S., Mourellou, O., Chaidemenos. G., Anagnostou, and E., Amaxopoulos, K. (2007). Neurosyphilis: a reality again. Journal of the European Academy of Dermatology &Venereology, 21, 388‐389. y Duffy, J.R. Motor Speech Disorders: Substrates, Differential Diagnosis, and Management. 2nd Edition, Elsevier‐Mosby. 2005. y Musher, D.M. (2008). Neurosyphilis: Diagnosis and response to treatment. Clinical Infectious Diseases, 47, 900‐902. y Shann, S. and Wilson, J. (2003). Treatment of neurosyphilis with ceftriaxone. Sexually Transmitted Infections, 79, 415‐416. y Smith,G.T., Goldmeier, D., and Migdal, C. (2006). Neurosyphilis with optic neuritis: an update. Postgraduate Medical Journal, 82, 36‐39. y Timmermans, M. and Carr, J. (2004). Neurosyphilis in the modern era. Journal of Neurology, Neurosurgery & Psychiatry, 12, 1727‐1730. y http://www.nlm.nih.gov/medlineplus/ency/article/000703.htm#visualContent y http://www.ninds.nih.gov/disorders/aids/detail_aids.htm.
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