Tabes dorsalis

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73 Tabes dorsalis

Instruction

Examine this patient with lightning pains (lightning denotes that the pains are fleeting and does not necessarily mean they are excruciating).

Salient features

History

• ‘Lightening pains’ are electrical shock like sensation in the limbs, throat, stomach or rectum

• High-stepping gait: patient may only hear their feet slapping the ground and feels as if they are walking on cotton-wool (caused by loss of joint position sense)

• Constipation (this is present as a rule).

Examination

• Examine the eyes:

• Bilateral ptosis with frontalis overaction

• Argyll Robertson pupil (irregular, small pupil which reacts sluggishly to light but reacts to accommodation; Case 39)

• Optic atrophy (primary).

• Examine the sensory system:

• Posterior column signs: look for loss of pain sensation but with normal touch and temperature sensation over the nose, cheeks, inner aspects of the arms and legs, a band across the nipple and in the anal area

• Squeeze calf muscles and the Achilles tendon (evokes no pain as deep sensation is lost).

• the ankle jerks (absent) and plantar response (normal)

• the gait for ataxia (steppage gait) and Romberg’s sign

• the knees and hips for Charcot’s joints

• the feet for trophic ulcers.

• Tell the examiner that you would like to:

• ask the patient about bladder sensation (lost), overflow incontinence and impotence (if male)

• check testicular sensation (usually lost early).

Remember: As a rule, the lower limbs are affected before the upper limbs. Very rarely, the upper limbs may be affected first (cervical tabes).

Diagnosis

This patient has posterior column signs and Argyll Robertson pupil (lesion) caused by tabes dorsalis (aetiology), and has severe ataxia (functional status).

Advanced-level questions

What do you know about neurosyphilis?

Without treatment, early syphilis progresses (Fig. 73.1). Tertiary syphilis of the nervous system never develops in those who have received appropriate therapy in the early stages. There are five clinical patterns of neurosyphilis:

• Meningovascular disease, which occurs 3–4 years after primary infection

• Tabes dorsalis, which occurs 10–35 years after primary infection

• Generalized paralysis of the insane (GPI), which occurs 10–15 years after primary infection

• Taboparesis (a combination of tabes dorsalis and GPI)

• Localized gummata.

Fig. 73.1 Approximate time course of the clinical manifestations of early syphilis and neurosyphilis. Width of the areas indicates approximate proportion of patients with the syndrome.

What is the underlying pathology?

It is caused by a combination of neuronal degeneration and/or arterial lesions.

How would you confirm the diagnosis?

The Wasserman reaction, Veneral Disease Reference Laboratory (VDRL), or Treponema pallidum haemagglutination assay (TPHA):

• Meningovascular syphilis: 70% positive, whereas CSF shows 90% positive

• Tabes dorsalis: 75% positive

• GPI: nearly 100% positive.

Note: Other tests include fluorescent treponemal antibody (FTA) and the treponemal immoblization test (TPI).

Mention a few conditions for which the Wasserman reaction may be falsely positive

Rheumatoid arthritis, SLE, chronic active hepatitis, infectious mononucleosis.

How would you treat the active syphilitic infection?

A course of parenteral penicillin.

How would you manage other symptoms of tabes?

• Lightning pains: simple analgesics, carbamazepine

• Sensory ataxia: re-educational exercises to improve limb coordination

• Bladder symptoms: avoid anticholinergics, employ urodynamic studies and intermittent self-catheterization

• Visceral crises: opiate analgesics; in recurrent cases, section of lower thoracic spinal dorsal roots

• Perforating ulcer: well-fitting shoes and regular foot care

• Charcot’s arthropathy: symptomatic treatment, avoid insertion of prostheses as results are poor.

In which group of patients is syphilis now common?

In those with underlying HIV infection.

What is the Jarisch–Herxheimer reaction?

An acute hypersensitivity reaction seen when a patient with syphilis is treated with penicillin. Toxins from the killed spirochaetes cause this reaction, which can be fatal. Steroids are often given during the first few days of penicillin therapy.

How may this patient present to the surgeons?

With acute abdominal pain (lancinating pains).

What are the methods of eliciting deep pain?

• Abadie’s sign is the loss of pain sense in the Achilles tendon

• Biernacki’s sign is the absence of pain on pressure on the ulnar nerve

• Pitres’ sign is loss of pain on pressure on the testes

• Haenel sign is analgesia to pressure on the eyeballs.

Tabes was first recognized by Romberg and Duchenne. Fournier was the first to suspect that it was caused by syphilis.

In the first half of the 19th century, three European physicians—namely Mortiz Romberg, Marshall Hall and Bernadus Brach described the loss of postural control in darkness of patients with severely impaired proprioceptive sensation (Neurology 2000;55:1201–6). Romberg and Brach showed the relationship between this sign and tabes dorsalis (Semin Neurol. 2002;22:409–18).

Alphonse Daudet’s (French novelist) experience of the agonies of tabes dorsalis has been well described.

AP Wassermann (1866–1925), a German physician.

A Jarisch (1850–1902), Professor of Dermatology in Austria.

K Herxheimer (1861–1944), a Jewish German dermatologist who died in a concentration camp.

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