Help needed for a case

Antonio Bardi a.bardi at COMUNE.LIVORNO.IT
Sat Mar 8 16:09:12 EST 1997


WHOEVER MIGHT BE INTERESTED IS KINDLY REQUESTED TO CONTACT 
US URGENTLY WITH HIS ADVICE, CONCERNING THE TREATMENT OF 
THE FOLLOWING CASE:

PIPPAN ROBERTO - Age:51 - Profession: former fireman - Living in Leghorn - Italy

Because of a heavy deterioration of his vital and intellectual capabilities,
his case was 
framed within the manifestations of a progressive, primitive, subcortical
gliosis, as it 
was recently described, that is variations of the frontal cerebral
involution (Pick's 
second type).
The patient was on various occasions admitted to the National Institute
"Carlo Besta" 
in Milan with a frontal symptoms complex, progressive mental degeneration, 
dysarthria, motility troubles and subcortical cortico-atrophy set in 1994
and still 
worsening.
Detailed neuro-radiological scrutiny as well as magnetic resonance revealed
an altered 
resounding area localized in a central mesencephalic location, without mass
effect but 
characterized by two small contiguous roundish impregnations.
In 1995, in the above institute, an evident apathic behaviour was noted, by
far not 
corresponding to his earlier extremely active and extrovert personality.
The patient is suffering from diabetes since he was 24 and is carrier of a 
polyneuropathy complex fully proved with neurophysiological documentation.
Presently the subject is extremely under-weighted, neither aware/vigil nor co-
operating, with heavily impoverished spontaneous speech turning to
dysarthria when 
stimulated. Temporal and spatial orientation seem preserved.
He is apparently retaining ideation and denomination, but has motorial
troubles and 
poor limbs and body co-ordination together with heavy apathy, volatile,
light-headed 
judgement - some episodes of verbal aggressivity - defective short-term memory, 
whilst the middle- term one sounds still operating;
He is affected by Dysarthria and disphagy - slow wide-legged walk with slight 
bilateral stepping, traces of paraparesis.
Are also observed grave left upper limb rigidity with abducted arm posture,
flexed 
forearm and hand fingers. doubtful traces of  plastic hypertones.
Heavy decrease of muscular trophism affecting face, neck, shoulder & pelvis
girdles, 
upper & lower limbs (the left upper one more than the other ) and the
interosseal hands 
bilateral muscles. Muscular sthenia.
HISTOLOGICAL TEST ( The right frontal pole, atrophic, was removed as a whole)
DESCRIPTION OF SPECIMENS: The white subcortical material is looking pale on 
colouring for myelin and is characterized by heavy gliosis with astrocytes
having 
abundant cytoplasm and a felt of intensely GFAP-positive cells
prolongations. Among  
U-fibres there are areas where the gliosis is more evident and reaches the
deeper layers 
of the cerebral cortex, though the process is mostly sparing it so far.
Deposits of A-Beta proteins or PRP are not noted as well as neuro-fibrillar 
degenerations or other hyperphosphorilated tau neuronal inclusions.
HISTOLOGICAL DIAGNOSIS: subcortical gliosis
Please note: the report is not consistent with diagnosis of Alzheimer and
Creutzfeldt-
Jacob desease as well as prions induced Encephalopathy, Pick's desease
(Pick's corpus 
phenotype), Lewy's corpus dementia.
The patient is presently an inmate of the high intensity care department at
Leghorn 
Hospital, tracheotomized with respirator and PEG.

ANTONIO BARDI, via Calatafimi 38, 57126 Livorno 
e-mail:      a.bardi at comune.livorno.it




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