Aetiology of Proximal Weakness among Adult Sudanese patients

Objective: To determine the aetiology of proximal myopathy among adult Sudanese patients seen in Elshaab Teaching Hospital. Methods: This is a descriptive cross sectional hospital based study conducted in Elshaab Teaching Hospital, during the period from January 2004 – September 2005. 100 adult Sudanese patients with proximal myopathy were reviewed, detailed history and proper clinical examination was done by the authors. Results: The most frequent cause of proximal myopathy was found to be muscular dystrophy whic accounted for 30% of the cases, followed by myasthenia gravis 20%, polymyositis and dermatomyositis 14%, Guillain Barre 8%, diabetes mellitus 5%, connective tissue diseases 5%, thyrotoxicosis 3%, chronic renal failure 3%, malignancy 2%, drugs (steroids alcohol 2%. Spinal muscular atrophy, hypokalaemia, and hypocalcaemia each accounted for 1%. Conclusion: The study showed that the incidence of proximal myopathy is more common among females. Proximal muscle weakness involved the lower

mal Weakness among Adult Sudanese patients ltahir, Affrah A/Rahman Mustafa, A.Sidig, M.O.EH.Gadour iology of proximal myopathy among adult b Teaching Hospital.ve cross sectional hospital based study Hospital, during the period from January adult Sudanese patients with proximal ed history and proper clinical examination e of proximal myopathy was found to be muscular dystrophy whic ases, followed by myasthenia gravis 20%, polymyositis and Barre 8%, diabetes mellitus 5%, connective tissue diseases 5%, failure 3%, malignancy 2%, drugs (steroids ophy, hypokalaemia, and hypocalcaemia each accounted for 1%.
that the incidence of proximal myopathy is more common among ess involved the lower limbs more than the upper limbs.1].ss was found in 4%, vulsions in only 1%, rash, polyuria and each in 5% of patients.was shown in table2.Myasthenia gravis and limb girdle muscular dystrophy were the most frequent myopatheis among our patients.Backer disease, diabetes mellitus and connective tissue disease affect 5% each while Facio-scopuls-humeral dystrophy, Spinal muscular atrophy, Hypokalaemia and Hypocalcaemia were found each in only 1% of the patients.Rest of the diagnoses was shown in table 3.

DISCUSSIO.:
The study showed that the incidence of proximal myopathy is more common among female, this is probably due to the fact that diseases like myasthenia gravis, thyroid diseases, polymyositis and dermatomyositis, which constituted 37% of all cases with proximal myopathy in the study, are more common among female.Almost 95% of patients with myasthenia gravis, thyrotoxicosis, dermatomyositis and dermatomyositis were females; this is similar to the results reported elsewhere [3][4][5] .The peak incidence was observed in individual aged 16 -45 accounting for 62%, which is the most active sector of the community, this goes with what was mentioned in the literature 6 .Patients with proximal myopathy showed chronic onset of presentation in 79%, this may be because most of the causes of proximal myopathy like muscular dystrophy, and myasthenia gravis have chronic and progressive course [7][8] .Some diseases presenting with proximal myopathy like myasthenia gravis and thyroid diseases have increased incidence among relatives of the patients 6,9 .This is supported by the finding of family history of similar condition in 18% of our patients.Unlike what was found in UK and USA, most frequent causes of proximal myopathy in our population were muscular dystrophy followed by myasthenia gravis, polymyositis and dermatomyositis 10,11 .Wide variety of clinical presentations of proximal myopathy were encountered including frequent falls [66%] which was similar to what was mentioned in the literature 12 .In consistent with other reports proximal muscle weakness involving the pelvic girdle was seen in 86%, where as that involving the shoulder girdle was seen in 70% 1,2,10 .Myopathy with muscle pain seen in all patients with polymyositis and dermatomyositis was similar to a study done by Dalakes and Narayana 13,14 .The classical presentation of limb girdle and Fascioscabulohumeral muscular Dystrophy which was seen in 10% and 19% of our patients respectively differs from what was reported by Panegyres who found a lesser percentage skeletal deformities and winging of scapulae.This can be explained by the fact that limb girdle and Fascioscabulohumeral muscular Dystrophy constitute 20% of our patients 15 .Proximal myopathy attributed to abnormal gate in 66%, while 20% were completely bed ridden due to severe weakness.This contrasts the findings reported by others 15 .However, it can be explained by the late presentation in our patients.Similar to earlier reports, CPK was found to be high in 66% of the study group including all patients with Duchenne and Beaker muscular dystrophy 1,12 .The higher characteristic EMG features seen in our patients with proximal myopathy [92%] when compared with other's [55%], is probably due to the late presentation of our patients 4 .Similar to Christopher findings, muscle biopsy supported the diagnosis of muscular dystrophies in 46% of the patients 5 .

CO.CLUSIO.:
The study showed that the incidence of proximal myopathy is more common among females.Proximal myopathy has a wide range of causes and had involved the lower limbs more than the upper ones.Muscular dystrophy, myasthenia gravis and limb-girdle muscular dystrophy had dominated the clinical presentation.

Table 1 :
Relationship between sex and age in 100 Sudanese patients with proximal myopathy.

Table 2 :
Some of the symptoms of the patients:

Table 3 :
Causes of proximal myopathy among 100 Sudanese patients.
The study showed that 79% of our patients had chronic symptoms.Family history of proximal myopathy was detected in 19% of the studied group.The vast majority of the patients had normal sensations and coordination[table 4]

Table 4 :
Upper and lower limbs examination findings: