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Journal of Neurology Research

Case Report

Volume 4, Number 4, August 2014, pages 121-123

A Case of Neurosyphilis Presenting With Tongue Tremor

Considering the current trend towards an increasing rate of neurosyphilis in several parts of the world, it is essential for the general practitioner to be able to recognize its different clinical presentations, as effective forms of treatment are widely available [1-3]. The diagnosis of neurosyphilis is defined by the presence of positive serum venereal disease research laboratory (VDRL) or fluorescent treponemal antibody - absorption (FTA-Abs) tests, and abnormal cerebrospinal fluid (CSF) analysis (VDRL and pleocytosis). The three common forms of neurosyphilis are asymptomatic, meningeal and vascular. Another rarer presentation is the parenchymatous form, which includes general paresis (dementia paralytica) and tabes dorsalis [1, 2].

Tabes dorsalis, also known as progressive locomotor ataxia, is characterized by progressive ataxia, loss of proprioception, absent deep tendon reflexes, Argyll-Robertson pupils and neuralgic paroxysmal pain. Movement disorders, other than ataxia, represent rare manifestations of neurosyphilis [1, 2].

Here we present a peculiar case of neurosyphilis presenting with tongue tremor and dementia.

A 32-year-old man presented with a 2-year history of progressive aggressiveness, visual and auditory hallucinations, persecutory delusions, anhedonia, apathy, and multi-domain cognitive deficits that included memory, writing, reading and calculation difficulties. On neurological examination, mini-mental status examination (MMSE) score was 17. Argyll-Robertson pupils were absent. A coarse rest and action tongue tremor were observed, associated with generalized bradykinesia. Other features such as rigidity, postural instability or tremor in other body parts were absent. Routine blood tests were normal, except for a reactive VDRL with a 1:16 titer and a positive FTA-Abs test. Brain CT and MRI scans showed signs of generalized atrophy. CSF analysis showed the presence of 69 leukocytes (90% lymphocytes), elevated protein (162 mg/dL), low glucose (47 mg/dL) and reactive VDRL with a 1:64 titer. The patient was treated with penicillin G cristalin 24 million units, intravenously for 14 days, with moderate improvement of cognitive functions. After a 6-month follow-up, neurological examination showed improvement of the tongue tremor. CSF analysis showed 11 leukocytes (95% lymphocytes), protein 42 mg/dL, glucose 58 mg/dL and VDRL 1:2. Another CSF analysis performed after 18 months showed four leukocytes, glucose 64 mg/dL and protein 33 mg/dL, with VRDL 1:2. Cognitive function was also markedly improved with MMSE of 23.

Classically, the clinical syndromes of neurosyphilis that are more widely recognized are general paresis, also known as dementia paralytica, or Lissauer form, and tabes dorsalis or progressive locomotor ataxia [1, 2]. Movement disorders associated with neurosyphilis are typically sensory ataxia, parkinsonism, myoclonus, chorea and dystonia [3]. Other very rare movement disorder syndromes include progressive supranuclear palsy, corticobasal, and cerebellar ataxia/myoclonus syndromes [3, 4]. Tong et al [5] evaluated the spectrum of movement disorders secondary to neurosyphilis. The authors studied seven of the 169 neurosyphilis (with syphilitic meningitis, meningovascular neurosyphilis, general paresis and tabes dorsalis) patients presenting with movement disorders, including four patients with parkinsonism, one each with laryngeal dystonia, corticobasal syndrome and sensory ataxia.

General paresis is characterized by progressive cognitive impairment (evolving to severe dementia), with pyramidal signs, Argyll-Robertson pupils, and psychiatric symptoms (emotional lability, paranoia, delusions of grandeur, hallucinations) and bradykinesia [1, 2, 6]. The case presented here is characteristic of general paresis, a parenchymatous form of neurosyphilis, associated with tongue tremor. In this rare clinical syndrome of neurosyphilis, the more commonly reported movement disorder is a very characteristic coarse tongue tremor, occasionally associated with facial and upper limbs tremor [2, 7, 8]. Silverdale et al [9] studied the spectrum of orolingual tremor and proposed a new syndrome classification. In this review the authors defined other secondary causes of orolingual tremors, including neurosyphilis.

Recently Martinelli et al [7] described a very unique orofacial dyskinesia related to neurosyphilis. This kind of dyskinesia, involving the lips and lower facial muscles, was named the “candy sign” and was considered pathognomonic of neurosyphilis [7]. There are other descriptions of tongue movement disorders in neurosyphilis, such as orofacial chorea, stereotypical dyskinesia, and trombone tremor of the tongue or trombone tongue [6-8, 10] (Table 1).

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Table 1. Tongue Movement Disorders in Acquired Neurosyphilis

Conflict of Interest

The authors have nothing to disclose.

Fund Support

This article is not funded by any source.

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Journal of Neurology Research is published by Elmer Press Inc.