Strachans syndrome

Genetic testing

NCV/EMG

Laboratory

Imaging

Biopsy

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Axonal degeneration with myelin breakdown is seen in the posterior columns of the cervical cord and optic nerves. Sural nerve biopsy shows axonopathy of large diameter fibers.

Anatomy/distribution

Patients report symptoms of sensory neuropathy (painful and burning feet).

Symptoms

Strachan's syndrome is defined by painful neuropathy, amblyopia, and orogen- Clinical syndrome/ ital dermatitis. Patients may also exhibit restless legs and ataxia. signs

Strachan's syndrome occurs from a high carbohydrate diet without vitamins (e.g., sugar cane workers, the Cuban optic and peripheral neuropathy epidemic of 1991, POWs). The patients treated with vitamins during the Cuban outbreak responded well, and thus it is thought that the pathology is due to poly-deficiency of thiamine, niacin, riboflavin, and pyridoxine.

Pathogenesis

Multivitamin replacement with a nutritious diet is effective. Replacement of riboflavin (B2) quickly affects orogenital dermatitis, but has no effect on neurological symptoms.

Therapy

The prognosis is good with early treatment.

Prognosis

Cockerell OC, Ormerod IE (1993) Strachan's syndrome: variation on a theme. J Neurol Reference 240:315-318

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