-transparent.png){kind=logo}
A 16-month-old boy was hospitalized because of a 1-day history of severe ketoacidosis with lethargy, hypotonia, vomiting, and important dyspnoea. Organic acid assay by gas chromatography-mass spectrometry confirmed the diagnosis of methylmalonic acidaemia (MMA). On the sixteenth day, he developed an acute extrapyramidal disorder. The CT scan of the brain disclosed bilaterally symmetric lucency of basal ganglia. He died at 17 months of age. Post-mortem neuropathological examination, showed severe necrosis with spongiosis, cystic cavitation and numerous lipid-laden macrophages of the globi pallidi, and mild spongiosis of subthalamic nuclei, mammillary bodies, portion of internal capsule adjacent to globus pallidus, superior cerebellar peduncles and tegmentum of brainstem. Pallidal infarction, a focal ischaemic lesion, demonstrates that ischaemia/energy depletion may be important in the etiology of the neuropathology of MMA.
A Larnaout, and
M A Mongalgi, and
N Kaabachi, and
D Khiari, and
A Debbabi, and
A Mebazza, and
M Ben Hamida, and
F Hentati
September 1986,
Annals of neurology,
A Larnaout, and
M A Mongalgi, and
N Kaabachi, and
D Khiari, and
A Debbabi, and
A Mebazza, and
M Ben Hamida, and
F Hentati
June 2014,
Movement disorders : official journal of the Movement Disorder Society,
A Larnaout, and
M A Mongalgi, and
N Kaabachi, and
D Khiari, and
A Debbabi, and
A Mebazza, and
M Ben Hamida, and
F Hentati
November 1993,
No to hattatsu = Brain and development,
A Larnaout, and
M A Mongalgi, and
N Kaabachi, and
D Khiari, and
A Debbabi, and
A Mebazza, and
M Ben Hamida, and
F Hentati
February 2021,
BMJ case reports,
A Larnaout, and
M A Mongalgi, and
N Kaabachi, and
D Khiari, and
A Debbabi, and
A Mebazza, and
M Ben Hamida, and
F Hentati
January 2012,
The Journal of the Louisiana State Medical Society : official organ of the Louisiana State Medical Society,
A Larnaout, and
M A Mongalgi, and
N Kaabachi, and
D Khiari, and
A Debbabi, and
A Mebazza, and
M Ben Hamida, and
F Hentati
April 1978,
Nursing times,
A Larnaout, and
M A Mongalgi, and
N Kaabachi, and
D Khiari, and
A Debbabi, and
A Mebazza, and
M Ben Hamida, and
F Hentati
January 2015,
AJNR. American journal of neuroradiology,
A Larnaout, and
M A Mongalgi, and
N Kaabachi, and
D Khiari, and
A Debbabi, and
A Mebazza, and
M Ben Hamida, and
F Hentati
July 1984,
Annals of neurology,
A Larnaout, and
M A Mongalgi, and
N Kaabachi, and
D Khiari, and
A Debbabi, and
A Mebazza, and
M Ben Hamida, and
F Hentati
January 1993,
Journal of computer assisted tomography,
A Larnaout, and
M A Mongalgi, and
N Kaabachi, and
D Khiari, and
A Debbabi, and
A Mebazza, and
M Ben Hamida, and
F Hentati
January 2011,
JBR-BTR : organe de la Societe royale belge de radiologie (SRBR) = orgaan van de Koninklijke Belgische Vereniging voor Radiologie (KBVR),
