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 which 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 and chloroquine) 2%, 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 limbs more than the upper limbs. Keywords: myopathy, Guillain Barre, dermatomyositis, polymyositis, hypokalaemia, atrophy.Sudan Journal of Medical Sciences  Vol. 3 (1) 2008: pp. 17-2

Eltahir, AE

Gadour, MO

Hussein, A

Mustafa, A

Sidig, A

English

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      © Sudan JMS Vol. 3, No. 1, Mar. 2008                          17   Aetiology of Proximal Weakness among Adult Sudanese patients  Abbasher Hussein, A.E. Eltahir, Affrah A/Rahman Mustafa, A.Sidig, M.O.EH. Gadour  Abstract 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 which  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 and chloroquine) 2%, 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 limbs more than the upper limbs.   Key words: myopathy, Guillain Barre, dermatomyositis, polymyositis, hypokalaemia, atrophy.  yopathy is a disease of striated muscle. Clinically the patient generally experiences weakness or complete paralysis of the affected muscle. Acute inflammatory myopathies can be part of the manifestations of infections1. Acute inflammatory polyneuropathy, Guillain Barre Syndrome, porphyria, diphtheria usually have sub acute onsets. Myasthenia Gravis, severe polymyositis and dermatomyositis must be considered in the differential diagnosis of subacute weakness of muscles. Weakness from endocrine disorders and certain muscle toxins may also develop sub acutely. Proximal weakness evolving over months may be a part of manifestations of polymyositis, dermatomyositis or endocrinopathy2. When the course has extended for a year or more, muscular dystrophies, spinal muscular atrophy, and myasthenia gravis has to be suspected. OBJECTIVE: To study the etiology of proximal myopathy among adult Sudanese patients attending neurology department in EL Shaab Teaching Hospital Khartoum Sudan in the period from January 2005 to December 2005. Abbashar Hussein Associated Prof, Faculty of Medicine, University of Khartoum, Sudan, P. O. Box 102 Khartoum, Sudan, Tel. +249-9123456722. E-mail: abbashar59@yahoo.com METHODOLOGY: This is a descriptive cross sectional hospital based study .It was conducted at Elshaab Teaching Hospital, Khartoum Sudan which is a 240 bedded hospital with cardiology, respiratory, neurology and an intensive care units. The study population included adult Sudanese patients with proximal weakness referred to the hospital from different parts of Sudan. One hundred patients were included in the study. Detailed history and proper clinical examination was done. Urine analysis, complete haemogram, blood urea, serum potassium, CPK, muscle biopsy, thyroid function test, F.B.S, P.P.B.S., Tensilon test and EMG were done.  DATA ANALYSIS: Data were analyzed using Statistical Package for Social Science (SPSS). The results were expressed as tables.   RESULTS: Out of 100 patients included in the study 60% were females. The majority of the patients were younger than 45 years [Table 1]. Facial muscle weakness was found in 4%, dizziness in 6%, convulsions in only 1%, whereas fever, skin rash, polyuria and polydepsia were found each in 5% of patients. Rest of the symptoms was shown in table2. M Hussein A et al.                                               Aetiology of Proximal Weakness among Adult Sudanese patients  © Sudan JMS Vol. 3, No. 1, Mar. 2008                                          18       Table 1:  Relationship between sex and age in 100 Sudanese patients with proximal  myopathy.  Age  Female  Male  Frequency   % 16 – 25 16 14 30 30 26-  35 09 08 17 17 36 - 45 13 02 15 15 46 – 55 10 07 17 17 56 – 65 07 06 13 13 > 66 05 03 08 08 Total  60 40 100 100                                                            Table 2: Some of the symptoms of the patients:   Symptoms   Frequency Percentage Visual disturbance and diplopia [each]  09 09 Dropping of upper eyelid  13 13 Difficult mastication and swallowing [each] 22 22 Nasal regurgitation 12 12 Abnormal speech  14 14 Frequent falls  66 66 Not able to get up from sitting  86 86 Use his hand to climb up himself when getting up  29 29 Not able to comb hair 85 85 Not able to lift objects  78 78 Not able to climb stairs 80 80 Muscle pain  14 14 Joint pain  13 13 Weight loss  80 80 Loss of appetite  25 25 Constipation  24 24  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.  Table 3: Causes of proximal myopathy among 100 Sudanese patients.  The diagnosis  Frequency Percentage  Duchenne  04 04 Limbgirdlemusculardystrophy  19 19 Motor neuron disease  04 04 Myasthenia gravis 20 20 Guillain Barre syndrome  08 08 Polymyositis and dematomyositis [each]  7 07 thyrotoxicosis and CRF [each] 03 03 Malignancy, drugs and alcohol [each]. 02 02    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]  Hussein A et al.                                                             Aetiology of Proximal Weakness among Adult Sudanese patients        © Sudan JMS Vol. 3, No. 1, Mar. 2008                                        19    Table 4: Upper and lower limbs examination findings:  Clinical finding  Frequency in upper limbs Frequency in lower limbs Fasiculation and tremors [each] 03 03 Wasting  15 15 hypertrophy of deltoid muscle  09 09 hypotonia  38 36 Hypertonia  02 00 Hyperreflex  02 02 Hyporeflex  34 34 normal sensation  95 93 Normal coordination  85 69  Associated skeletal deformities (Kyphosis, Scoliosis, and Kyphoscilosis) were seen in 10% of the patients, where as 19% had winging scapulae due to weakness of serratous anterior, pectoralis minor, trapezius muscle resulting in instability of scapular and shoulder joint.  Creatine kinase was high in 66%, RBS> 126 in 6% while potassium and calcium were low in 1% of the patients. Thyroid function test consistent with thyrotoxicosis was seen in 3% and tensilon test was positive in 20%. Hgh CSF protein was found in 4%. EMG showed myopathic changes in 92%, muscle biopsy revealed an evidence of myopathy in 46%, The frequency of diagnosis according to classification of muscle diseases showed that 30% of our patients had genetic disorders, 20% neuromuscular (myasthenia), 22% inflammatory disorders, 16% metabolic disorders, 5% autoimmune and 7% had other causes. DISCUSSION:                                                                                                                                                                                                   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 elsewhere3-5. 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 literature6. 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 course7-8. Some diseases presenting with proximal myopathy like myasthenia gravis and thyroid diseases have increased incidence among relatives of the patients6, 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 dermatomyositis10,11. Wide variety of clinical presentations of proximal myopathy were encountered including frequent falls [66%] which was similar to what was mentioned in the literature12. In consistent with other reports proximal muscle weakness involving the pelvic girdle was seenin 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 Narayana13,14. 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 patients15. Proximal Hussein A et al.                                                             Aetiology of Proximal Weakness among Adult Sudanese patients        © Sudan JMS Vol. 3, No. 1, Mar. 2008                                        20   myopathy attributed to abnormal gate in 66%, while 20% were completely bed ridden due to severe weakness. This contrasts the findings reported by others15. 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 dystrophy1,12. 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 patients4. Similar to Christopher findings, muscle biopsy supported the diagnosis of muscular dystrophies in 46% of the patients5.   CONCLUSION: 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.    References: 1. Bushby K , Gardner , Medwin D et al. The clinical genetic and dystrophin characteristics of Becker muscular dystrophy. J Neurol 1993; 240: 105 – 115. 2. Kaufmann J , Hunzel M N , Genth E et al. The clinical spectrum of dermatomyositis. Disch Deimatol  2005 ; 3(3) : 181-94 3. Dewey CW, Bailey CS, Shelton GD et al. Clinical forms of acquired myasthenia gravis: 25 cases. J Vet int Med 1997; II: 50-57. 4. Reed AM, Mason T. Recent advance in Juvenile dermatomyositis, Cur Rheumatol Rep 2005 ;7 (2) : 94 – 8 . 5. Christopher SL, Plotz P. Adult inflammatory myopathies , Best Pract Res clin Rheumatol  2004 ; 18(3) : 331 - 44 . 6. Drachman DB. Myastheria gravis N Engl J Med 1994; 330:1797-1810. 7.  Nisbet JA, E Astwood JB, Colston KW. Detection of Osteomalacia in British – Asian, a comparison of clinical score with biochemical Measurements. Clin sci 1990; 78:383 – 390. 8. Leif M, Erick FE, Peder C. Effect of thyroid on bone and mineral metabolism. Endocrinol Netab Clin 1990; 19:55 – 63. 9. Werneck LC, Cunha FM, Scola RH. Myasthenia gravis: a retrospective study comparing thymectomy to conservative treatment. Acta Neurol Scand 2000;101:41–6  10. Eurgene PC, Henry GH, Daniel HM et al. Osteomalacia in thyrotoxicosis. Metablism 1964; 13:161-171. 11. Renuka D, Rao SD, Murthy KSR. A profile of nutritional osteomalacia in Hyderabad city. J Assoc Physicians India 1982; 30: 215 – 217. 12. Angelinic , Beggs Alt  , Hoffman ED et al. Enormous dystrophin in parient with Becker muscular dystrophy. Neurology 1990; 40: 808 – 812.  13. Dalakes MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003; 362: 971-82. 14. Narayana S AS , Akhtar M , Kumar N et al. Polymysitis –a review and follow up study of 24 cases , J Assoc Physicians India 1993 ; 41(6) : 354-6 . 15. Panegyres PK , Mastaglia FL ; Kahulas BA.  Limb girdle syndromes , clinical , Morpholoical and Electrophysiological Studies , J Neurol Sci 1990; 1995(2): 201- 18 .                  

Aetiology of Proximal Weakness among Adult Sudanese patients

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Aetiology of Proximal Weakness among Adult Sudanese patients

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