Year : 2007 | Volume
: 28 | Issue : 1 | Page : 32--35
Tabes dorsalis with ocular changes
NA Karibasappa1, S Maheshwarappa2, Rajesh V Muragod1, P Nataraj1,
1 Department of Dermatology and Venereology, J. J. M Medical College, Davangere, Karnataka, India
2 Department of Ophthalmology, J. J. M Medical College, Davangere, Karnataka, India
N A Karibasappa
Department of Dermatology and Venereology, J. J. M Medical College, House No - 3367/2, M.C.C «SQ»B«SQ» Block, Shamnur Road, Davangere - 577 004, Karnataka
A 56-year-old male patient who had history of sexual exposure 28 years back was referred by the Department of General Medicine as he was not responding to treatment for spastic paraplegia and was venereal disease research laboratory (VDRL) reactive. The patient had characteristic clinical features of tabes dorsalis except Charcot«SQ»s arthropathy, dribbling incontinence and visceral crises. Serum VDRL was 1:32 and cerebrospinal fluid (CSF) VDRL was 1:4. CSF analysis showed increased lymphocytes and protein. Though tabes dorsalis is a rarity now compared to the pre-antibiotic era, one may come across few cases presenting with variable manifestations, more so in the era of human immunodeficiency virus infection. Therefore, awareness of the florid manifestations of tabes dorsalis is necessary for early diagnosis and proper treatment of this disabling condition.
|How to cite this article:|
Karibasappa N A, Maheshwarappa S, Muragod RV, Nataraj P. Tabes dorsalis with ocular changes.Indian J Sex Transm Dis 2007;28:32-35
|How to cite this URL:|
Karibasappa N A, Maheshwarappa S, Muragod RV, Nataraj P. Tabes dorsalis with ocular changes. Indian J Sex Transm Dis [serial online] 2007 [cited 2020 Feb 16 ];28:32-35
Available from: http://www.ijstd.org/text.asp?2007/28/1/32/35710
Tabes dorsalis is a parenchymatous neurosyphilis, characterized by slowly progressive degeneration of posterior column, posterior roots and ganglion of the spinal cord. Various clinical studies conducted in India during the last few decades have shown consistent low prevalence of neurosyphilis among sexually transmitted diseases. ,, However, few anecdotal reports of tabes dorsalis continue to appear intermittently in the tertiary care hospitals. ,, It has also been shown that the concurrent infection of human immunodeficiency virus (HIV) and syphilis lead to rapid progression of early syphilis to neurosyphilis. , Therefore, a case of tabes dorsalis is presented with review of relevant literature to remind ourselves of pleomorphic manifestations of tabes dorsalis for proper diagnosis and treatment of patients.
A 56-year-old male, who was a chronic alcoholic and smoker since 33 years, was referred to the Skin Department by the Department of Medicine as his VDRL was reactive and he was not responding to treatment for spastic paraplegia. The patient complained of pain in lower limbs since 10 months and complained of loss of sensation of both lower limbs since 6 months. Detailed history revealed tremors in hand, lightning pain, numbness, loss of sensation below knee and weakness in the lower limbs. The patient did not give history of genital ulcer but gave history of extramarital relationship with a known person before marriage.
On examination, there were no hypopigmented patches and no trophic ulcers or enlarged peripheral nerve trunks. Central nervous system examination revealed normal mental functions and cranial nerves. The temperature and fine-touch sensations were lost, but pain and crude touch were mildly impaired. Muscles of both the lower limbs (below knee) showed weakness (grade III), but there was no wasting. Deep tendon reflexes except for triceps jerk, abdominal reflexes and superficial reflexes were absent with bilateral flexor plantar response. The vibration sense, tactile localization, tactile discrimination and joint position sense were normal below knee on both sides. The Romberg sign was positive and the gait was ataxic and high stepping. Ophthalmologic examination revealed absent light reflex in the right eye, while accommodation reflex was present [Table 1]. On using mydriatic (Tropicamide 0.5% + phenylephrine 5%), the pupil did not dilate in the left eye. On fundus examination, left eye showed pale optic disc, suggestive of early neurosyphilis optic atrophy with Argyll-Robertson pupil.
Complete hemogram showed normocytic hypochromic anemia and raised ESR (48 mm after 1 h). Liver function tests (LFT), renal function tests (RFT) and random blood sugar (RBS) were within normal limits. X-ray chest showed basal lobe consolidation. Radiography of the spine and CT scan of the brain were normal. HIV antibody (ELISA) test was nonreactive for both HIV-1 and HIV-2. Serum VDRL titer was 1:32. Cerebrospinal fluid (CSF) analysis showed clear fluid with total cell count 80 cells/mm 3 , out of which lymphocytes were 65 cells/mm 3 (increased), sugar 64.0 mg/dl, protein 129 mg% (increased), chlorides 118 mmol/L (increased) and CSF VDRL titer 1:4 (suggestive of neurosyphilis).
The patient was treated with aqueous crystalline penicillin 4 million units, 4 hourly, intravenously for 20 days. The patient responded very well to the treatment; tremors almost fully subsided after completion of treatment, and the patient was discharged on request.
In syphilis, involvement of CNS leads to a number of different syndromes, occurring over an extended period ranging from 1 or 2 years to >30 years from the onset of infection. Unlike in syphilitic meningitis and general paresis, where Treponema pallidum could be demonstrated early in CSF, in tabes dorsalis T. Pallidum cannot be demonstrated in CSF or other tissues, suggesting an immunological attack on the spinal cord.  With the exception of tabes dorsalis, clinical syndrome is rarely so clear cut as to permit the diagnosis of neurosyphilis in the face of negative serological findings.  Clinical diagnosis of tabes is more likely in a patient with lightning pains, ataxia, absent tendon reflexes, Argyll-Robertson pupil and positive Romberg sign. In atypical cases, only the results of serologic testing and CSF examination may lead to the correct diagnosis. Other features of tabes dorsalis are bowel and bladder disturbances, gastric crises, impotence, visual failure, pupillary abnormalities, Charcot's arthropathy and perforating ulcers.  Usually sensory symptoms, especially pain, precede ataxia. This pre-ataxic stage lasts for 2 to 5 years. The rate of progression of the disease can be roughly assessed from the duration of pre-ataxic stage. Longer the pre-ataxic stage, slower will be the subsequent deterioration. 
Our patient presented with characteristic features of tabes dorsalis with ocular changes, except gastric crises and Charcot's arthropathy. Pupillary changes have been ascribed to associated ependymitis with subependymal gliosis involving the high reflex tracts in the forebrain. Some workers have also reported changes in the iris itself at the neuromuscular junction, which may account for the irregularities of the pupils. The classical Argyll-Robertson pupils are small (pinpoint) and often irregular in outline; they do not react to light but react normally to convergence. The mydriatic reaction to atropine is rather poor, and the pupil does not dilate with painful skin stimuli.  It must be realized, however, that pupillary abnormalities are not always of this typical pattern. The most usual variants are differences in the size of pupils, fixation to light and accommodation and the retention of a partial light reflex in one eye. 
Though positive serum VDRL often is a clue to presence of neurosyphilis,  reactive VDRL in the spinal fluid is a reliable evidence of past or present neurosyphilis.  In tabes dorsalis, the characteristic changes that occur in CSF are increased lymphocytes (do not exceed 70/mm 3 ), normal or slightly raised protein and leutic type of colloidal gold curve.  Though replaced by more specific and advanced tests like CSF-IgG and CSF-TPHA index, which measure intrathecal antibody synthesis,  the colloidal gold test along with clinical picture and other CSF findings may aid in the diagnosis, but not in determining the activity, of tabes dorsalis.  VDRL test is positive in both CSF and blood in 65%, CSF alone in 10%, blood alone in 5% and negative in both in 20% of cases. The VDRL and FTA-Abs reaction may be negative in CSF in spite of an excess of cells, protein and globulins. A negative reaction in both blood and CSF or even a completely normal CSF is rarely found in a patient with progressive disease. In burnt-out cases of tabes dorsalis, CSF may be normal.  In the present case, the blood VDRL was 1:32 and the CSF VDRL was 1:4 with increased cell count and normal protein levels. In 20-30% of cases of late syphilis, the standard tests for syphilis may be nonreactive; therefore, a treponemal test should be performed.  In resource-limited countries like India, one has to depend on clinical knowledge, blood VDRL, CSF analysis and CSF VDRL for the diagnosis of tabes dorsalis, due to lack of facilities for specific treponemal tests.
Normal LFT, RFT, RBS, radiography of the spine, CT of the brain; normocytic normochromic anemia and the absence of hypopigmented anesthetic patches or enlarged peripheral nerve trunks, in the present case, excluded other possible differential diagnoses like alcoholic neuropathy, diabetes mellitus, spinal trauma, syringomyelia, vitamin B 12 deficiency and leprosy respectively.
With a rise in the prevalence of HIV infection, clinicians will come across patients presenting with more florid manifestations of syphilis of the pre-antibiotic era. Neurosyphilis may present without neurological symptoms in half of HIV-infected patients with serological evidence of syphilis.  There are several reports to show that patients with HIV are more likely to progress to neurosyphilis in the first two years after diagnosis, despite standard treatment. , Though antibiotics cannot reverse the extensive changes,  they may arrest the progression of tabes dorsalis in 50% of the cases.  Therefore, in the era of HIV infection, for the early diagnosis and prompt treatment of tabes dorsalis, alertness to the possibility of, and awareness of, its pleomorphic clinical and laboratory manifestations is crucial and the CSF analysis should be done for the evidence of neurosyphilis;  and it should be treated accordingly with high-dose intravenous penicillin. 
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