The patterns of clinical presentations of cerebellar syndromes among adult Sudanese patients
DOI:
https://doi.org/10.52981/sjms.v4i2.1001Keywords:
ataxia,, dysmetria,, disdiadochokenesis,, decomposition,, nystagmus,, dysarthria.Abstract
Cerebellar syndromes are one of the commonest neurological diseases.
Objectives: To study the patterns of clinical presentations of cerebellar syndromes and to identify the possible causes.
Methods: This is a prospective hospital based, cross-sectional study. One hundred adult Sudanese patients with cerebellar syndromes were included in the study during the period from January 2006– January 2007.
Results: The most common age group affected was 18 – 25 years. Male to female ratio was 1.5: 1 unsteadiness on walking was the most common symptom (83%). Gait-ataxia was the most common sign (83%). Cerebrovascular disease was the most common aetiology (25%).
Conclusion: Cerebellar syndromes are not rare in Sudan. However, they were diagnosed more commonly at the central regions of the country probably because of more awareness of patients and better facilities
for diagnosis. The age of onset, the male predominance, the presentation and clinical findings were not different from reported literature. This also goes for the common causes apart from alcohol which is a strikingly rare as a cause in this study and could be accounted for the implementation of Elshariya (Islamic laws) Laws in Sudan.
References
1. Ferrier D, Turner WA. A record of experimentsillustrative of the symptomatology and degenerationsfollowing lesions of the cerebellum and its pedunclesand related structures in monkeys. Phil Trans R SocLond B 1893; 185:719–728.
2. Russell JS. Experimental researches into thefunctions of the cerebellum. Phil Trans R Soc Lond B1894; 185: 819–831.
3. Brown S. On hereditary ataxia, with a series oftwenty-one cases. Brain 1892; 15:250–282.
4. Holmes G.“ Clinical symptoms of cerebellar diseaseand their interpretation , the croonian lecture 111”.Lancet 1922; 2: 59 – 65.
5. Michael J, Zigmond-Flyed E, Bloom J. Cerebellumanatomy, phylogenetic development and function. In:Michael J, Zigmond-Flyed E, Bloom J (ed),Fundamental neuroscience. Academic Press, 1999. P860, 973-979.
6. Adams CR, Ziegler DK, Lin JT. Cerebellumsyndrome. In: Adams CR, Ziegler DK, Lin JT (edit),Principle of neurology, 6th, Oxford, 1997. P. 90, 1313.
7. Christophar G, Goetz MD. Cerebellar ataxia. In:Christophar G Goetz MD (edit), Textbook of clinicalneurology, 2nd, Saunders, Pennsylvania - USA, 2003; P299-315.
8. Bradley WG, Daroff RB, Fenichel GM. Cerebellardisorder. In: Bradley WG, Daroff RB, Fenichel GM(edit.), Neurology in Clinical practice, 4th,Philadelphia: Butterworth Heinemann USA; 2004. P287-290.
9. Holmes G .“ The cerebellum of man”. Brain 1939;62: 11 – 30.
10. Holmes G. The symptoms of acute cerebellarinjuries due to gunshot wounds. Brain 1917; 40:461-480.
11. Middleton FA. “The cerebellum: an overview,”.TINS. 1998; 21: 367 - 369.
12. Fine EJ. “The history of development of cerebellarexamination”; Semin Neurol 2002; 22 : 375 – 384 .
13. Arthar C, Guyton-John E. The cerebellum and itsmotor function. In: Arthar C, Guyton-John E (ed),Textbook of medical physiology, 10th, SaundersPennsylvania, 2000, p 617- 656.
14. Richard S, Snell MD. Anatomy and Function ofthe cerebellum. In: Richard S, Snell MD (edit),Clinical Neuro-anatomy, 4th, Lippincott Williams andWilk publication, London, 1999, P 222-229.
15. Marr D. A theory of cerebellar cortex. J Physiol1969; 202:437470.
16. Paulin MG. The role of the cerebellum in motorcontrol and perception. Brain 1993; 41: 39-40.
17. Quin NP. Subcortical structures, the thalamus,cerebellum and basal ganglia. In: Weatherall DJ,Leding ham JG, Waredl DA (edt), Oxford textbook ofMedicine, 3rd, Oxford, 1998, P 967-969.
18. Botterwell EH. Functional localization in thecerebellum in primates II. Lesions of midline structures(Vermis) and deep nuclei. J Comp Neurol 1938; 69:47-62.
19. Botterwell EH. Functional localization in thecerebellum of prmates III. Lesion of the hemishere(Neo cerebellum). J Comp. Neurol 1938; 69: 47-72.
20. Iain Willkinson, Graham Lennox. Cerebellardisorders .In: Iain Willkinson,Graham Lennox (edit),
essential Neurology, 4th, Blackwell scientificpublications Malda USA; 2005. P 76-99.
21. Rammanan KW. Efficacy and safety of modifinal(Provigel) for treatment of fatigue in MS a two
continues phase 2 study. J Neurol NeurosurgPsychiatry 2002; 72: 179-183.
22. Roos KL. Encephilits. Neurol Clin 1999; 17:813-834.
23. Mamidi A. CNS infections in individuals withHIV infections. J Neuro Viral 2002; 8: 158-167
24. Johnson R. Arboviruses. In: Johnson R (edit), viral
infections of the NS, 2nd, Philadelphia: Lippincott;1999. P 119-124.
25. Canpell GL. West Nile virus. Lancet infect Dis2002; 2: 519-529.
26. Pattern J. Cerebellar disorders. In: John pattern
Neurological differential diagnosis, 2nd, London:Springer; 1999. P 203-212.
27. Koeppen AH. The hereditary ataxias. J NeuropatholExp Neurol 1998 Jun; 57(6): 531-543.
28. Werdelin L. Hereditary ataxias: Occurrence andclinical features. Acta Neurol Scand. 1986; 73:1-12.
29. Wetterau JR. Absence of microsomal triglyceridetransfer protein in individuals withabetalipoproteinemia. Science 1992; 258: 999-1001.
30. Sharp D. Cloning and gene defects in microsomaltriglyceride transfer protein associated withabetalipoproteinaemia. Nature 1993; 365: 65- 69.
31. Gotoda T. Adult-onset spinocerebellar dysfunctioncaused by a mutation in the gene for the alphatocopherol-transfer protein. N Engl J Med 1995; 333:1313-1318.
32. Robert C, Collins M. Mitochondrialencephalopathy. In: Robert C, Collins M (edit),Neurology review series, London: Saunders Pub;1997. P 91-97.
33. Petty RK. The clinical features of mitochondrialmyopathy. Brain. 1986; 109: 915-38.
34. Ouahchi K. Ataxia with isolated vitamin Edeficiency is caused by mutations in the alphatocopheroltransfer protein. Nat Genet1995;9: 141-145.
35. Cavalier L. Ataxia with isolated vitamin Edeficiency: heterogeneity of mutations and phenotypicvariability in a large number of families. Am J HumGenet 1998; 62: 301-310.
2. Russell JS. Experimental researches into thefunctions of the cerebellum. Phil Trans R Soc Lond B1894; 185: 819–831.
3. Brown S. On hereditary ataxia, with a series oftwenty-one cases. Brain 1892; 15:250–282.
4. Holmes G.“ Clinical symptoms of cerebellar diseaseand their interpretation , the croonian lecture 111”.Lancet 1922; 2: 59 – 65.
5. Michael J, Zigmond-Flyed E, Bloom J. Cerebellumanatomy, phylogenetic development and function. In:Michael J, Zigmond-Flyed E, Bloom J (ed),Fundamental neuroscience. Academic Press, 1999. P860, 973-979.
6. Adams CR, Ziegler DK, Lin JT. Cerebellumsyndrome. In: Adams CR, Ziegler DK, Lin JT (edit),Principle of neurology, 6th, Oxford, 1997. P. 90, 1313.
7. Christophar G, Goetz MD. Cerebellar ataxia. In:Christophar G Goetz MD (edit), Textbook of clinicalneurology, 2nd, Saunders, Pennsylvania - USA, 2003; P299-315.
8. Bradley WG, Daroff RB, Fenichel GM. Cerebellardisorder. In: Bradley WG, Daroff RB, Fenichel GM(edit.), Neurology in Clinical practice, 4th,Philadelphia: Butterworth Heinemann USA; 2004. P287-290.
9. Holmes G .“ The cerebellum of man”. Brain 1939;62: 11 – 30.
10. Holmes G. The symptoms of acute cerebellarinjuries due to gunshot wounds. Brain 1917; 40:461-480.
11. Middleton FA. “The cerebellum: an overview,”.TINS. 1998; 21: 367 - 369.
12. Fine EJ. “The history of development of cerebellarexamination”; Semin Neurol 2002; 22 : 375 – 384 .
13. Arthar C, Guyton-John E. The cerebellum and itsmotor function. In: Arthar C, Guyton-John E (ed),Textbook of medical physiology, 10th, SaundersPennsylvania, 2000, p 617- 656.
14. Richard S, Snell MD. Anatomy and Function ofthe cerebellum. In: Richard S, Snell MD (edit),Clinical Neuro-anatomy, 4th, Lippincott Williams andWilk publication, London, 1999, P 222-229.
15. Marr D. A theory of cerebellar cortex. J Physiol1969; 202:437470.
16. Paulin MG. The role of the cerebellum in motorcontrol and perception. Brain 1993; 41: 39-40.
17. Quin NP. Subcortical structures, the thalamus,cerebellum and basal ganglia. In: Weatherall DJ,Leding ham JG, Waredl DA (edt), Oxford textbook ofMedicine, 3rd, Oxford, 1998, P 967-969.
18. Botterwell EH. Functional localization in thecerebellum in primates II. Lesions of midline structures(Vermis) and deep nuclei. J Comp Neurol 1938; 69:47-62.
19. Botterwell EH. Functional localization in thecerebellum of prmates III. Lesion of the hemishere(Neo cerebellum). J Comp. Neurol 1938; 69: 47-72.
20. Iain Willkinson, Graham Lennox. Cerebellardisorders .In: Iain Willkinson,Graham Lennox (edit),
essential Neurology, 4th, Blackwell scientificpublications Malda USA; 2005. P 76-99.
21. Rammanan KW. Efficacy and safety of modifinal(Provigel) for treatment of fatigue in MS a two
continues phase 2 study. J Neurol NeurosurgPsychiatry 2002; 72: 179-183.
22. Roos KL. Encephilits. Neurol Clin 1999; 17:813-834.
23. Mamidi A. CNS infections in individuals withHIV infections. J Neuro Viral 2002; 8: 158-167
24. Johnson R. Arboviruses. In: Johnson R (edit), viral
infections of the NS, 2nd, Philadelphia: Lippincott;1999. P 119-124.
25. Canpell GL. West Nile virus. Lancet infect Dis2002; 2: 519-529.
26. Pattern J. Cerebellar disorders. In: John pattern
Neurological differential diagnosis, 2nd, London:Springer; 1999. P 203-212.
27. Koeppen AH. The hereditary ataxias. J NeuropatholExp Neurol 1998 Jun; 57(6): 531-543.
28. Werdelin L. Hereditary ataxias: Occurrence andclinical features. Acta Neurol Scand. 1986; 73:1-12.
29. Wetterau JR. Absence of microsomal triglyceridetransfer protein in individuals withabetalipoproteinemia. Science 1992; 258: 999-1001.
30. Sharp D. Cloning and gene defects in microsomaltriglyceride transfer protein associated withabetalipoproteinaemia. Nature 1993; 365: 65- 69.
31. Gotoda T. Adult-onset spinocerebellar dysfunctioncaused by a mutation in the gene for the alphatocopherol-transfer protein. N Engl J Med 1995; 333:1313-1318.
32. Robert C, Collins M. Mitochondrialencephalopathy. In: Robert C, Collins M (edit),Neurology review series, London: Saunders Pub;1997. P 91-97.
33. Petty RK. The clinical features of mitochondrialmyopathy. Brain. 1986; 109: 915-38.
34. Ouahchi K. Ataxia with isolated vitamin Edeficiency is caused by mutations in the alphatocopheroltransfer protein. Nat Genet1995;9: 141-145.
35. Cavalier L. Ataxia with isolated vitamin Edeficiency: heterogeneity of mutations and phenotypicvariability in a large number of families. Am J HumGenet 1998; 62: 301-310.
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2021-08-18
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