Info

Guide To Beating Hypoglycemia

The Best Ways to Treat Hypoglycemia

Get Instant Access

* v * /

Fig. 3.9. Pattern of thiamine deficiency, which is very similar to that of Leigh syndrome (cf. Fig. 3.8). An important difference is that in thiamine deficiency the mamillary bodies are involved in most cases, whereas in Leigh syndrome they are not

Fig. 3.9. Pattern of thiamine deficiency, which is very similar to that of Leigh syndrome (cf. Fig. 3.8). An important difference is that in thiamine deficiency the mamillary bodies are involved in most cases, whereas in Leigh syndrome they are not

Leigh Syndrome

in Leigh syndrome (Fig. 3.8) and the pattern of thiamine deficiency (Fig. 3.9) are very similar, although the lesions in the mamillary bodies, typical for thiamine deficiency, are consistently lacking in Leigh syndrome, but most different causes of energy failure lead to very different patterns of abnormalities (Figs. 3.1,3.2,3.4-3.13). The biochemical explanation for the differences in selectively involved structures is unclear, and apparently our present models are too simplistic.

Apart from mitochondrially mediated disorders, other conditions may involve the basal ganglia predominantly or selectively. This is the case in many inborn errors of metabolism other than mitochon-driopathies, in hepatic failure (Fig. 3.14), and in classic Creutzfeldt-Jakob disease (Fig. 3.15). The explanations must be different from those so far proposed.

In glutaric aciduria type I there is typically involvement of the putamen and caudate nucleus, but not of the cortical layers and Purkinje cells. The neo-

Glutaric Aciduria Type

Fig. 3.10. Typical involvement of the parieto-occipital region in a neonate with hypoglycemia; in the worst cases this leads to multicystic encephalopathy restricted to this region. Selective vulnerability of this region for hypoglycemia disappears with age, indicating the influence of morphologic and biochemical maturation of the brain on the pattern of resulting damage

Fig. 3.10. Typical involvement of the parieto-occipital region in a neonate with hypoglycemia; in the worst cases this leads to multicystic encephalopathy restricted to this region. Selective vulnerability of this region for hypoglycemia disappears with age, indicating the influence of morphologic and biochemical maturation of the brain on the pattern of resulting damage

Was this article helpful?

0 0
Hearing Aids Inside Out

Hearing Aids Inside Out

Have you recently experienced hearing loss? Most probably you need hearing aids, but don't know much about them. To learn everything you need to know about hearing aids, read the eBook, Hearing Aids Inside Out. The book comprises 113 pages of excellent content utterly free of technical jargon, written in simple language, and in a flowing style that can easily be read and understood by all.

Get My Free Ebook


Post a comment