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We're talking putaminal or

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corpus striatal necrosis,

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which is kind of an end stage or late

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manifestation of metabolic and genetic

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disorders that affect metabolism,

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oxygenation and ATP production.

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A nice example of this,

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unfortunately, is Leigh's disease,

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the most common phenotypic mitochondrial

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disorder of childhood.

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In this case,

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pyruvate carboxylase deficiency is at fault.

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The patient has choreoathetosis.

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There is symmetric involvement of

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the basal ganglia, some putamen,

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but in this case, also the external and internal

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portions of the globus pallidus are involved.

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And you might say, well,

0:47

the corpus striatum consists of the caudate

0:50

nucleus and the basal ganglia putamen.

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Well, the putamen inner portion is involved,

0:56

but look at the caudate nucleus,

0:59

you might blow right by it.

1:01

The caudate nucleus body is necrotic with these

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round foci of CSF-like signal surrounded by gliosis,

1:09

best seen in this coronal projection.

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Now, Leigh's disease is an uncommon disorder.

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One in 40,000 patients often have brain stem

1:18

signs, ataxia, pyramidal disorders.

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They may have lactate elevation,

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but usually they present when they have some type

1:24

of minor infection that throws them over the edge.

1:27

Maybe they have an upper respiratory tract

1:29

infection and then they start to present with

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diffuse metabolic abnormalities,

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including brainstem signs, which is very scary.

1:37

Consistently involved are the basal ganglia, putamen,

1:40

and to a lesser extent,

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globus pallidus and caudate,

1:43

thalami may be bilaterally involved the

1:46

substantia nigra and the brainstem nuclei,

1:49

although in this case,

1:50

the brainstem nuclei were spared.

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Leigh's disease,

1:53

a nice example of what happens to the corpus

1:56

striatum, the putamen and the caudate nucleus.

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In this case, also with some globus pallidus

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involvement from a general metabolic systemic

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heredofamilial disorder.