A neuropathic syndrome of uncertain origin: review of 100 cases

The clinical features of 100 cases of a neuropathic syndrome observed in the University College Hospital of the West Indies over a period of 3½ years are described. The four main features are: (a) upper motor neuron damage (93) (b) damage to the first sensory neuron (c) retrobulbar neuropathy (d) eighth nerve deafness The onset is from the second to the sixth decade and may be sudden or gradual. The sex incidence is equal. Maximum incapacity occurs within a few months and the disease thereafter appears to be stationary. No pathological material has yet been available for study but the nature of the lesion and the possible causes are discussed. The diet of the patients varied from good to very poor but few showed overt signs of vitamin deficiency or mal-nutrition. A dietary factor is possibly responsible but there is no definite evidence of this.

Se describen las caracteristicas clinicas de 100 casos de un sindrome neuropatico observadas en el Hospital del University College durante un plazo de 3½ anos. Las cuatro caracteristicas principles son: (1) dano sufrido en el neuroma motor superior (93) (2) dano sufrido en le primer neuroma sensorio (3) neuropatia retrobulbar (4) sordera del octavo nervio Se registra el acceso de la enferndad entre la segunda y la sexta decada; puede ser repentino o paulatino. La incidencia en los dos sexos es igual. La incapacidad maxima ocurre dentro de unos pocos meses y después de esto la enfermedad parece establecerse en un estado estatica. Todavía no se han conseguido materiales patológicas para un estudio detenido; no obstante se describe la naturaleza de la lesión y las causas que pueden ocasionarla. La dieta de los pacientes variaba de buena a malísima pero pocos manifestaban senales evidentes de deficiencia de vitaminas ni de desnutrición. Puede que sea responsable un agente dietario pero de esto no tenemos evidencia definitiva.

Motor neurone disease in Thailand: the clinical aspects of 77 patients.

This is the first large scale case series of motor neurone disease (MND) in Thailand. Seventy-seven patients were identified between 1978 and 1984 at Siriraj Hospital Medical School, Bangkok, Thailand. Fifty-five patients were male (71.43%) and the mean age of the patients was 51.55 (SD 14.26) years with the range of 17 to 78 years. Clinical classification of MND was categorized as progressive bulbar palsy (PBP), 26 patients (33.77%); amyotrophic lateral sclerosis (ALS), 42 patients (54.54%); and progressive spinal atrophy (PSA), 9 patients (11.69%). The mean age of PBP, ALS and PSA were in the order of 57.61 (SD 12.09), 52.81 (SD 11.18), and 28.11 (SD 9.44) years. Progressive spinal atrophy group was younger than PBP and ALS groups significantly at the P-value less than 0.05 by analysis of variance and Duncan tests. Fifty-three patients (72.60%) were resident in Bangkok and the central part of Thailand. The main presenting symptoms were wasting of the small muscles of both hands, leg weakness, and speech and/or swallowing difficulties. These symptoms were found in 62 patients (81.58%). Nearly half of the patients (48.68%) came to our care within six months of onset, 22.8 per cent presented with asymmetry of motor wasting, while limb and trunk fasciculation was seen in 73.61 per cent. Dysarthria, dysphagia and tongue fasciculation were recorded as 51.32, 48.68, 60.53 per cent respectively. Exaggerated deep tendon reflexes were noted as 65.79 and 80.26 per cent over the upper and lower limbs, while Babinski sign was elicited in only 23.3 per cent of the patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Epidemiological evidence from Zaire for a dietary etiology of konzo, an upper motor neuron disease

A clear association between seasonal outbreaks of a paralytic disease called konzo and toxic effects from consumption of insufficiently processed bitter cassava roots has been demonstrated in Bandundu region, Zaire. A community-based survey of 6764 inhabitants identified 110 live and 24 dead konzo-affected persons with a history of isolated non-progressive spastic paraparesis of abrupt onset. The start of these annual outbreaks of konzo in 1974 coincided with the completion of a new tarmac road to the capital, which facilitated the transport of cassava and made it the main cash crop. The extensive cassava sales encouraged the consumption by the peasant families of roots that had not been adequately processed; frequent acute cyanide intoxications resulted when the naturally occurring cyanogens in the roots were eaten. The disease mainly appeared in the dry season when there was high consumption of insufficiently processed cassava and the diet lacked supplementary foods with sulfur-containing amino acids which promote cyanide detoxification. These results, which confirm the earlier findings in East Africa, show that, owing to the high cyanide and low sulfur dietary intake, there is an increased risk of konzo outbreaks in cassava-growing areas during periods of adverse agro-economic changes

Ashort review of some neurological disorders in Benghazi

The Epidemiology of some of the neurogical diseases in Benghazi, north-eastern Libya, has been summarized from previously published studies