Multiple Sclerosis Journal - October 2017 - 1559

AA Zambon, G Cecchetti et al.
Table 1. Neuropsychological battery performed during the hospitalization.
Test

Raw score

Adjusted score

Cut-off

MMSE
CPM
Digit Span Forward
Digit Span Backward
15 Words Learning
Immediate/Delayed Recall
Rey Recall
DCT
FAB
Semantic Fluency
Phonemic Fluency
10 Point Clock Test
Rey's Figure Copy
Token Test
Ideative Apraxia R
Ideomotor Apraxia R/L

20/30
19
5
3
17/0

-
17
4.5
2.52
9.5/-

24
19
4.26
2.65
28.53/4.69

4
20
5
15
1
2
7
22
15
3/2

9.46
30
13.4
23.58
16
8
28.87
26.25
13
14

9.25
3.5
11.3
-
-
5
19.5
-
-

MMSE: MiniMental State Examination; CPM: Raven's Colored Progressive Matrices; DCT: Dot Counting Test; FAB: Frontal
Assessment Battery.

dysfunction, clumsiness, and mild pyramidal signs
(brisk reflexes and increased limb tone) were observed.
The presence of schizoid personality disorder was also
reported. EEG was normal. SPECT showed a
decreased cerebral blood flow (CBF) in the left temporal lobe, while brain MRI showed a diffused bilateral
white-matter involvement and severe atrophy. He was
therefore directed to our tertiary referral center with
the suspect of a metabolic disorder.
Neurological evaluation at arrival showed slowed
ideation, fatuous and hypocritical attitude, gaze
apraxia, mild pyramidal signs (increased spastic tone
of four limbs, diffused brisk reflexes, Achilles clonus,
and partial extensor plantar response), and mild limb
ataxia with reduced vibratory and kinesthetic sensation of lower limbs. Moreover, the patient showed difficulties in walking on tiptoes and running, and a mild
swaying at Romberg maneuver. Due to the severity of
cognitive impairment, an extensive neuropsychological assessment usually performed in patients affected
by dementia was performed, resulting in a moderate
multi-domain cognitive impairment (MiniMental
State Examination (MMSE) = 20/30) mostly affecting
short-term memory, verbal fluency, executive functions, and abstract reasoning (Table 1).
Serological investigations for infectious (syphilis,
Lyme disease, brucellosis, and HIV) and autoimmune
(lupus and vasculitis) diseases were negative. Sensory
and motor nerve conduction velocities were normal.

journals.sagepub.com/home/msj

EEG organization was normal except for occasional
left temporal slow wave abnormalities. Visual, auditory, and somatosensory-evoked potentials showed
markedly increased central latency of major components with the absence of tibial nerve cortical
responses bilaterally. Very long chain fatty acids
(VLFCA) were normal.
The patient performed 1.5 T brain MRI that showed
diffused atrophy. On T2-weighted images, multiple,
multifocal, and confluent lesions in the periventricular and juxta-cortical regions, the corona radiata, the
external capsules, the corpus callosum, the middle
cerebellar peduncles, and the pons were detected.
Non-enhancing T1-hypointense lesions (black holes)
of the corona radiata were present. Spinal cord MRI
showed three different dorsal lesions with focal atrophy. No gadolinium enhancement was observed
(Figure 1).
Cerebrospinal fluid (CSF) analysis showed a slight
increase in proteins (66 mg/dL), normal glucose, and
one cell. Oligoclonal bands (OBs) were present and
IgG-index was 0.65. Moreover, a reduction of betaamyloid was detected (423 ng/L; normal values > 500).
MRI findings, CSF analysis, and multi-modal-evoked
potentials were consistent with an inflammatory
demyelinating disorder of the central nervous system.
According to the 2010 Revision of the McDonald
Criteria,4,5 diagnosis of primary progressive multiple

1559

https://journals.sagepub.com/home/msj

Table of Contents for the Digital Edition of Multiple Sclerosis Journal - October 2017