Presentation
Associated involvement of the arms and, occasionally, cranial nerves may be present in cases with severe spastic paraparesis. [actahort.org]
If present, the latency is prolonged. [ncbi.nlm.nih.gov]
Cassava contains the cyanogenic glucoside linamarin and a small amount of lotaustralin (methyl linamarin) both of which are broken down to cyanohydrins and glucose, catalysed by the enzyme linamarase also present in the root. [biology.anu.edu.au]
The age and sex distributions are presented in Fig. 2. The annual and monthly distributions of the onset of konzo are shown in Fig. 3 and 4, respectively. [ajns.paans.org]
In this issue of Neurology, the report by Siddiqi et. al of Zambia’s first known Konzo outbreak sadly illustrates this new reality.2 Konzo, a permanent upper motor neuron injury that presents with abrupt spastic paresis, was first reported from the Congo [blogs.neurology.org]
Entire Body System
- Disability
Abstract Two Tanzanian patients with konzo were severely disabled by a non-progressive spastic paraparesis, since the sudden onset during an epidemic six years earlier. [jnnp.bmj.com]
The range of disabilities varies from a slightly spastic gait in mild cases to a complete inability to walk in more severely affected cases. [actahort.org]
The handicap remains irreversible and may be exacerbated by further attacks, leading to severe disability. [ajns.paans.org]
Impairments, disabilities and handicap pattern in konzo–a non-progressive spastic para/tetraparesis of acute onset. Disabil Rehabil 2001;23:731-736. [blogs.neurology.org]
Working the land, fetching water and cooking; all these thing I can barely manage due to my disabilities. My husband Luciano (35) had left me, because he said I could no longer be a good wife, yet he came back.’ [biology.anu.edu.au]
- Pain
The remainder (97/152) had disabilities of various other origins: pain in the joints of lower limbs (92), psychosomatic disorder (2), possible disk hernia (1), myositis (1), and foot injury (1). [ajns.paans.org]
Smaller (non-fatal) amounts of cyanide cause acute intoxication with symptoms of dizziness, headache, nausea, stomach pains, vomiting and diarrhoea. [biology.anu.edu.au]
It will therefore be possible to document a ‘post-Konzo’ syndrome with increased pain and weakness due to contractures and joint malalignment in old patients. [ncbi.nlm.nih.gov]
- Weakness
It’s very dangerous for them to be underfed; they are weak and get ill more quickly. I fear that they’ll become paraplegics through konzo as well. [biology.anu.edu.au]
It will therefore be possible to document a ‘post-Konzo’ syndrome with increased pain and weakness due to contractures and joint malalignment in old patients. [ncbi.nlm.nih.gov]
Gastrointestinal
- Nausea
Smaller (non-fatal) amounts of cyanide cause acute intoxication with symptoms of dizziness, headache, nausea, stomach pains, vomiting and diarrhoea. [biology.anu.edu.au]
- Vomiting
Smaller (non-fatal) amounts of cyanide cause acute intoxication with symptoms of dizziness, headache, nausea, stomach pains, vomiting and diarrhoea. [biology.anu.edu.au]
Eyes
- Blurred Vision
Furthermore, 9 patients (16%) experienced blurred vision, 5 (9%) muscle cramps and 3 (5%) a sensation of electrical discharges in the lower limbs at the time of the onset. [ajns.paans.org]
Blurred vision and swallowing difficulties have been occasionally reported. Clinical deficits are usually greater at the onset of the disease, confining the affected subjects to bed. [ncbi.nlm.nih.gov]
- Visual Impairment
Thirteen subjects (23%) with either mild, moderate, or severe konzo reported impaired visual acuity at the onset of the disease. [ajns.paans.org]
This condition encompasses visual impairment, temporal pallor of the optic discs, and defect of visual fields. A pendular nystagmus has been reported in few cases (Mwanza et al., 2003a, b; Mwanza et al., 2005). [ncbi.nlm.nih.gov]
- Visual Impairment
Thirteen subjects (23%) with either mild, moderate, or severe konzo reported impaired visual acuity at the onset of the disease. [ajns.paans.org]
This condition encompasses visual impairment, temporal pallor of the optic discs, and defect of visual fields. A pendular nystagmus has been reported in few cases (Mwanza et al., 2003a, b; Mwanza et al., 2005). [ncbi.nlm.nih.gov]
Neurologic
- Clonus
Ankle clonus was found in 40 cases (70%) and was similarly difficult to elicit in the remainder. Babinski’s sign was found in 34 subjects (60%) and no response in the remainder. [ajns.paans.org]
The ankle clonus is frequently found, as well as pathological reflexes of the upper extremities in severely affected subjects, with a clearly noticeable palmomental reflex. [ncbi.nlm.nih.gov]
- Spastic Paralysis
Lower sulfurtransferase detoxification rates of cyanide in konzo-A tropical spastic paralysis linked to cassava cyanogenic poisoning. Neurotoxicology 2017;59:256-262. [blogs.neurology.org]
The occurrence of konzo is strongly associated with the consumption of improperly processed bitter cassava coupled with malnutrition and environmental stressors such as drought and turbulent times, leading to irreversible spastic paralysis and neurocognitive [nature.com]
- Unable to Walk
Nine subjects (16%) walked with hyperextended knees and on the soles of their feet, thus counteracting (and masking) a tendency to toe walk. Seven subjects (12%) were unable to walk at all. One had spasticity only when running. [ajns.paans.org]
[…] et al., 2011) severe form = subject is unable to walk (Konzo, 1996). [ncbi.nlm.nih.gov]
- Dizziness
Smaller (non-fatal) amounts of cyanide cause acute intoxication with symptoms of dizziness, headache, nausea, stomach pains, vomiting and diarrhoea. [biology.anu.edu.au]
- Flaccid Paralysis
The Konzo outbreak was only incidentally recognized due to ongoing surveillance for polio in Acute Flaccid Paralysis reporting programs. [blogs.neurology.org]
Treatment
Treatment, prognosis and prevention Up to date, there is no effective treatment for Konzo that has been documented. The neurological damages are debilitating, and permanent. [ncbi.nlm.nih.gov]
Prognosis
Treatment, prognosis and prevention Up to date, there is no effective treatment for Konzo that has been documented. The neurological damages are debilitating, and permanent. [ncbi.nlm.nih.gov]
Etiology
Epidemiological evidence from Zaire for a dietary etiology of konzo, an upper motor neuron disease.. Bulletin of the World Health Organization, 69 (5), 581 - 589. [apps.who.int]
Neurological disorders associated with cassava diet: a review of putative etiological mechanisms. Metab Brain Dis 2011; 26:79-85. [medigraphic.com]
Epidemiology
Epidemiological evidence from Zaire for a dietary etiology of konzo, an upper motor neuron disease.. Bulletin of the World Health Organization, 69 (5), 581 - 589. [apps.who.int]
Table 1 Neuro-epidemiology and clinical electrophysiology of Konzo. [ncbi.nlm.nih.gov]
Epidemiological data associate cassava consumption with hypothyroidism and with two very different neurological diseases. [elsevier.es]
Her additional skills in epidemiology, health services research, and tropical medicine are brought to bear during the 6-months annually she spends in Africa where she serves as Director for the Chikankata Epilepsy Care Team in rural Mazabuka, Zambia, [blogs.neurology.org]
Clinical epidemiological observations on an ataxic Syndrome in Western Nigeria. Trop Geogr Med 1964; 16:316-23. Osuntokun BO. An ataxic neuropathy in Nigeria: A clinical, biochemical and electrophysiological study. Brain 1968; 91(2): 215-48. [medigraphic.com]
Prevention
Presently, the key to eradicating Konzo is prevention. Because there is no cure for the neurological damage that Konzo causes, the battle against the disease must focus on prevention. [ncbi.nlm.nih.gov]
A review of health effects of cyanide exposure from cassava and of ways to prevent these effects. A report for UNICEF/African Household Food Security Programme, 2nd revised edition. South Africa; 1988:40. Tshala-Katumbay D. [medigraphic.com]
Konzo was first reported by Dr Trolli, in the then Belgian Congo in 1938, in Popokabaka Health Zone in Bandundu Province and we have prevented konzo for the first time ever in the same Health Zone (see Publications No. 7). [biology.anu.edu.au]
Gretchen’s research programs are aimed at identifying opportunities to prevent or ameliorate the medical and social morbidities of common neurologic conditions in low-income, tropical settings with the ultimate goal of developing successful interventions [blogs.neurology.org]
Improving cassava processing might prevent the disease. [ajns.paans.org]