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Zaphoon

Extremely helpful member
Joined
Aug 2, 2008
Messages
2,857
Reason
DX UMND/PLS
Diagnosis
08/2011
Country
US
State
Missouri
City
Springfield
I found this article to be very interesting. It seems a cause can be found to a motor neuron disease, after all!

KONZO, AN EPIDEMIC UPPER MOTOR NEURON DISEASE STUDIED IN TANZANIAW.
P. HOWLETT1, G. R. BRUBAKER2, N. MLINGI3 and H. ROSLING4

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Author Affiliations

Summary

An epidemic of spastic paraparesis was studied in a drought-affected rural area of Tarime district in northern Tanzania. The uniform clinical findings in 39 cases, aged 4–46 yrs, indicated abrupt symmetric isolated and permanent but not progressive damage to the upper motor neurons. Due to the failure of other food crops, the diet at onset consisted almost exclusively of bitter cassava roots, a drought-tolerant starchy root crop widely cultivated in Africa. The drought increased the natural occurrence of cyanogenic glucosides in the cassava roots, and the processing procedure normally applied in order to remove cyanide before consumption was shortened because of food shortage. The resulting high dietary cyanide exposure was confirmed by very high serum levels of thiocyanate, the detoxification product of cyanide. Tests for HTLV-1 antibodies were negative and no other findings supported an infectious aetiology. The clinical findings and the associations with cassava toxicity are almost identical to those reported from outbreaks of spastic paraparesis in Mozambique and in Zaire, where this disease was first reported under the name ‘konzo’. We thus conclude that konzo constitutes a distinct upper motor neuron disease entity, probably caused by a toxic effect from insufficiently processed cassava under adverse dietary circumstances.
© Oxford University Press
 
very intresting kim,while doing research for h4c i also found another desease with umn involvement.

Adult-onset (adrenomyeloneuropathy, or AMN)

About 30 percent of affected males develop symptoms between the ages 11 and 40. Adult-onset ALD, or AMN, is a less severe and more slowly progressive form of ALD. Signs and symptoms of AMN vary in severity and may include:
Bowel or bladder problems
Gait disturbances — difficulty walking
Loss of muscle tone, muscle spasms and spasticity — stiff or rigid muscles
Peripheral neuropathy — pain, loss of sensation and inability to control muscles
Progressive muscle stiffness, weakness or paralysis, particularly in the lower limbs

Adrenomyeloneuropathy may be difficult to diagnose or misdiagnosed because the symptoms vary and mimic other diseases such as multiple sclerosis.

Nearly 50 percent of women who are carriers of the genetic defect for adrenoleukodystrophy will also develop mild symptoms of AMN during adulthood.

made me think if some of us with pls could be mis-diagnosed.
also i think it could mimic als with the loss of muscle tone with spasticity...........mmmmmmmmm.
 
I'm glad to hear there is such a thing as a NONprogressive umn disease! But I grieve at such terrible effects of famines and droughts. :(
 
Abstract

We present two patients with primary lateral sclerosis-like upper motor neuron disease accompanying subclinical Sjögren's syndrome. Both patients showed progressive spastic quadriparesis, but neither sensory involvement nor detrusor dysfunction was noted. Lower motor neuron signs were detected only in their late follow-up period. Although sicca symptom was nearly absent, salivary labial gland biopsy revealed marked sialoadenitis in both patients. They also displayed a constellation of findings that suggested an autoimmune etiology closely related to Sjögren's syndrome, including germinal center formation in one patient, and markedly elevated levels of anti-nuclear antibody with abnormal sialography in the other. Both patients showed significant neurological improvement after the initial course of intravenous immunoglobulin therapy. We suggest that the evidence for subclinical Sjögren's syndrome should be sought in patients presenting with selective upper motor neuron involvement.
 
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