MYELOSIS FUNICULARIS AS A RESULT OF VITAMIN B12/SECONDARY DEFICIENCY IN A 9-YEAR-OLD GIRL

 

Ilona Kopyta, Ewa Jamroz, Elzbieta Marszal

Upper Silesian Mother and Child Health Centre, Neurology Department, ul. Medykow 16 40-752 Katowice

 

A nine year old girl with no family history or adverse pregnancy or natal outcome, who underwent a resection of the end section of the small bowel as a result of atresia in the first twenty four hours of her life, was admitted to the Clinic on account of spastic paresis of the lower extremities. Apathy and tiredness appeared the most striking features on the physical examination of the child; likewise the pale colour of the integument and a scar on the abdomen integuments - a result of the resection. Neurological examination revealed pyramidal syndrome from the lower extremities, disturbed sense of balance which intensified with eyes closed, and deep sensibility disorders. Megaloblastic anaemia was revealed on further examinations, while the bone marrow image showed normoblastic erythropoesis with the presence of megaloid cells, extremaly low levels of vitamin B12 in the blood serum and a regular level of folic acid; vitamin B12 absorption test showed considerable impairment in the process; inflammation of the esophagus and duodenum mucosa, which was confirmed in a histopathologic examination. Furthermore, irregulator somatosensory result of induced potentials and a conductivity test revealed some characteristics of axonal demyelination neuropathy in the lower extremities.

MRI of the head and spinal cord was correct. Diagnosis excluded methylomalonic acidosis, multiple sclerosis and other causes of ataxia in children. The overall clinical image, that is megaloblastic anaemia, the characteristics of the myelosis funicularis and interview data - resection of the bowel as well as other examinations confirm that the observed disorders resulted from a deficiency in the absorbtion of vitamin B12. An appropriate regimen duly implemented (intramuscular vitamin B12) improved the neurological condition and hematologic parameters.

CONCLUSIONS: It is imperative that the hematologic parameters and the neurologic conditions should be monitored in the group of children who underwent the resection of the ileum in infancy. If vitamin B12 deficiency symptoms occur, appropriate treatment ought to be duly implemented.