Strachan’s Disease
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Disease Entity
Strachan’s Disease is a rare neurologic syndrome consisting of optic neuropathy, peripheral neuropathy, and skin and mucous membrane lesions. It is likely caused by nutritional deficiencies.[1][2][3]
History
Dr. Henry Strachan, a medical officer working in Jamaica in 1887, described a constellation of symptoms consisting of optic neuropathy, sensorineural hearing loss, peripheral neuropathy, skin and mucous membrane lesions, and numbness, cramping, and burning pain in the hands and feet.[2][3][4]These symptoms were also described by Madan during the last Cuban war for independence in 1898; as such, efforts have been made to rename this disease Strachan-Madan syndrome.[2] Strachan’s Disease is understood to be related to generalized malnutrition or specific vitamin deficiencies, although the specific vitamins involved remain unclear.[5][6][7]Numerous reports describe these symptoms in malnourished prisoners of war.[1][5][6][7] Strachan’s disease was also described in Cuba in the 1990s, which was linked to group B vitamin deficiencies. [1][8][9]
Symptoms
Strachan described numbness, cramps in the hands and feet, warmth, hyperemia, and severe burning pain in the palms of the hand and soles of the feet, muscle wasting in the hands and feet, vision loss, hearing loss, facial nerve palsies, desquamating skin lesions of the eyelids, lip borders, and nostrils, and monoplegias.[3][4]
Differential Diagnosis
Strachan’s Disease presents similarly to Tropical Ataxic Neuropathy (TAN).[4] Both present with numbness, vision loss, and muscle cramping in the hands and feet. However, Strachan’s Disease may also present with severe burning pain in the hands and feet. Additionally, TAN presents with gait ataxia. While Strachan’s Disease patients may have gait abnormalities, this is due to muscle atrophy rather than sensory gait ataxia.[4]
Specific nutritional neuropathies should be considered in malnourished patients presenting with peripheral neuropathy, vision loss, or sensorineural hearing loss. Deficiencies of thiamine, pyridoxine, nicotinic acid, and riboflavin are known to cause neurologic symptoms.[7]
Another differential diagnosis is HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis (HAM-TSP), a progressive neurological disease caused by Human T-Lymphotrophic Virus 1 (HTLV-1). Clinical features include bilateral or unilateral lower limb weakness, hyperreflexia, spasticity, urinary incontinence, and gait abnormalities.[10] Patients may also present with neuralgias, paresthesias, uveitis, arthritis, polymyositis, and infectious dermatitis.[11] Patients with clinical suspicion of Strachan’s Disease should be screened for HAM-TSP via enzyme-linked immunoassay for detection of HTLV-1 antibodies.[10][11]
Management
Patients should receive a full neurological workup including neuroimaging and CSF analysis to exclude other disease processes including multiple sclerosis (MS).
Treatment consists of dietary management to improve nutrition status and correct specific nutritional deficiencies. Patients should receive early supplementation with B-complex vitamins and vitamin A.[12] Most case reports show symptom resolution with intravenous nutrition therapy. Rarely, patients may have persistent optic neuropathy after treatment.[1][8]
References
- ↑ 1.0 1.1 1.2 1.3 D.S.Mojon, P.K. Mann, H. Baker, N.S. Lincoff, J.G. Odel, M. Hirano, Vitamin status and mitochondrial DNA mutations in patients with incomplete recovery from an epidemic optic neuropathy in Cuba, Neuro-Ophthalmology.16(1996) 355–360.https://doi.org/10.3109/01658109609044640
- ↑ 2.0 2.1 2.2 R. Santiesteban-Freixas, E. Pamias-González, S. Luis-González, C. Serrano-Verdecia, A. González-Quevedo, I. Alfaro-Capdegelle, M. Francisco-Plasencia, J. Suárez-Hernández, [Epidemic neuropathy: proposal and arguments to rename Strachan disease as Strachan and Madan disease]., Rev Neurol. 25 (1997) 1950–6
- ↑ 3.0 3.1 3.2 H. Strachan, Malarial multiple peripheral neuritis., Sajou Ann Universal Med Sci . 1 (1888)
- ↑ 4.0 4.1 4.2 4.3 O.S.A. Oluwole, Persistence of tropical ataxic neuropathy in a Nigerian community, J Neurol Neurosurg Psychiatry. 69 (2000) 96–101. https://doi.org/10.1136/jnnp.69.1.96.
- ↑ 5.0 5.1 O.C. Cockerell, I.E.C. Ormerod, Strachan’s syndrome: variation on a theme., J Neurol. 240 (1993) 315–318.
- ↑ 6.0 6.1 M. FISHER, Residual neuropathological changes in Canadians held prisoners of war by the Japanese; Strachan’s disease., Can Serv Med J. 11 (1955) 157–99
- ↑ 7.0 7.1 7.2 G. v Gill, D.R. Bell, Persisting nutritional neuropathy amongst former war prisoners, J Neurol Neurosurg Psychiatry. 45 (1982).
- ↑ 8.0 8.1 G.C. Roman, Epidemic neuropathy in Cuba: A plea to end the United States economic embargo on a humanitarian basis, Neurology. 44 (1994) 1784–1784. https://doi.org/10.1212/WNL.44.10.1784.
- ↑ Cuban Epidemic Neuropathy: Insights into the Toxic–Nutritional Hypothesis through International Collaboration, MEDICC Rev. 20 (2018) 27. https://doi.org/10.37757/MR2018.V20.N2.6.
- ↑ 10.0 10.1 C.R.M. Bangham, A. Araujo, Y. Yamano, G.P. Taylor, HTLV-1-associated myelopathy/tropical spastic paraparesis, Nat Rev Dis Primers. 1 (2015) 15012. https://doi.org/10.1038/nrdp.2015.12.
- ↑ 11.0 11.1 Charles Clarke, Robin Howard, Martin Rossor, Simon Shorvon, eds., Neurology: A Queen Square Textbook, 2nd Edition, 2nd ed., John Wiley & Sons, 2016.
- ↑ Strachan's Syndrome, Mitch Medical Healthcare. (2022). https://www.mitchmedical.us/clinical-neurology/strachans-syndrome.html
