Ioannis Mavridis, M.D., Sophia Anagnostopoulou, M.D., Ph.D., Assoc.
Professor
Department of Anatomy, University of Athens School of Medicine
Corresponding author:
Ioannis N. Mavridis, M.D.
Department of Anatomy, Medical School, University of Athens,
Mikras Assias str. 75, Goudi, 11527 Athens, Greece
Tel.: 0030-210 74 62 404
Fax: 0030-210 74 62 398
e-mail: pap-van@otenet.gr
Ioannis Mavridis, M.D., Sophia Anagnostopoulou, M.D., Ph.D., Assoc.
Professor
Department of Anatomy, University of Athens School of Medicine
Corresponding author:
Ioannis N. Mavridis, M.D.
Department of Anatomy, Medical School, University of Athens,
Mikras Assias str. 75, Goudi, 11527 Athens, Greece
Tel.: 0030-210 74 62 404
Fax: 0030-210 74 62 398
e-mail: pap-van@otenet.gr
Dear Sir/Madam,
We read the article by Mok et al (2011) on cortical and
frontal atrophy associated with cognitive impairment in age-related
confluent white-matter lesion (WML).[1] They interestingly reported
that cognitive impairment in patients with confluent WML is
mediated by global and frontal cortical atrophy and that the
mechanisms whereby WML induces cognitive impairment are
uncertain.[1] They also mentioned that factors such as infarct
load and location, microbleed, global atrophy, central atrophy,
regional brain atrophy and Alzheimer's pathology may also affect
cognition.[1] They concluded that cognitive impairment in confluent
WML is probably mediated by frontal and global cortical atrophy.
We agree with the authors and we would like to comment on the
relation between regional brain atrophy and cognitive impairment.
We experienced a case of a 94-years-old female who had suffered
a stroke (a few years prior to her death) which caused focal
brain atrophy and was followed by the clinical expression of a
psychotic syndrome. The brain atrophy was found during a
cadaveric study in the dissection room of our Department. During
our macroscopic pathological investigation we noticed a fossa,
surrounded by a few petechiae, on the external surface of the
right parietal lobe, at the posterior end of the Sylvian
fissure. At this area, the cerebral gyri were excessively
atrophic and the fossa so deep that almost reached the external
wall of the lateral ventricle. The minimum gyri thickness was
approximately 1 mm, after removing the meninges. The tissue of
the atrophic gyri was soft like a chewing gum, despite the rest
gyri of the same brain. Checking out the arteries of the Willis
circle, we found a 25 mm long red fussiform thrombus occluding
the right middle cerebral artery (MCA). It was beginning from
the bifurcation of the right internal carotid artery and
extending within the MCA, occluding it completely. The microscopic
pathological investigation of the atrophic gyri revealed
degenerative lesions with microcycts, edema and many PAS(+)
amyloidal bodies. Oxymoronically, clear microscopic findings of an
infarct were absent.
Persson et al (1989) supported that focal ischemic changes
produced by MCA occlusion constitute a dynamic process in which
several pathophysiologic events occur over an extended period.[2]
Tamura et al (1991) reported that delayed neuropathologic changes
following cerebral ischemia have attracted widespread attention and
their results demonstrate the significance of remote changes over
a long period of time following focal brain injury. This
phenomenon could be important in understanding the pathophysiologic
changes during the chronic phase of cerebral infarction.[3] We
believe that, in our case, the dynamic pathophysiologic process
followed MCA occlusion, combined with delayed neuropathologic
changes during the chronic phase of the stroke, resulted to the
regional atrophy and the cognitive impairment (regarded as a
'psychotic syndrome').
Finally, we support that major cerebrovascular accidents can
rarely cause regional brain atrophy. Perhaps the microvascular
condition of an old brain could partially help in understanding
not only the pathogenesis of this unusual phenomenon, but also
the developed cognitive impairment. Moreover, the pathophysiologic
mechanism which resulted to the observed atrophy could be also
involved in the clinical expression of cognitive impairment. We
hope that future studies will illuminate such paths of the human
brain physiology and pathophysiology, which are still remaining
dark and mysterious and therefore challenging and admirable.
References
1. Mok V, Wong KK, Xiong Y, et al. Cortical and frontal
atrophy are associated with cognitive impairment in age-related
confluent white-matter lesion. J Neurol Neurosurg Psychiatry
2011;82:52-7.
2. Persson L, Hardemark HG, Bolander HG, et al. Neurologic and
neuropathologic outcome after middle cerebral artery occlusion in
rats. Stroke 1989;20:641-5.
3. Tamura A, Tahira Y, Nagashima H, et al. Thalamic atrophy
following cerebral infarction in the territory of the middle
cerebral artery. Stroke 1991;22:615-8.
Kengo Maeda,
Department of Neurology,
National Hospital Organization Shiga Hospital
255 Gochi, Higashi-oumi, Shiga 527-8505, Japan
Tel: +81-748-22-3030
Fax: +81-748-23-3383
E-mail: maeda-kengo@shiga-hosp.jp
Dear Editor,
I have read a postscript 'Brainstem and spinal cord motor neuron
involvement with optineurin inclusions in proximal-dominant hereditary
motor and sensory neuropathy' (HMSN-P)1 by Fujita et al with a great
interest. HMSN-P is very rare and is found in only limited area (Two areas
in Japan and Brazil). They discovered optineurin-positive inclusions as
well as lower motor neuron involvement in the brainstem and spinal cord.
They proposed a new classification of HMSN-P into the subgroup of
amyotrophic lateral sclerosis (ALS). Also, they described the pathological
changes in the lateral columns with neuronal loss in the precentral gyrus.
This finding might also have encouraged them to consider the new
classification. After I reported three Brazilian brothers with HMSN-P who
had Okinawan ancestry2, I went to Brazil to see their siblings and another
HMSN-P family3 and saw nine Brazilian HMSN-P patients in all. Moreover, I
have seen six HMSN-P of Kansai (Shiga)-type in my hospital (V:3, V:11,
V:16, and V:17 of pedigree 1, IV:8 and V:4 of pedigree 2)4. But, none of
them showed clear pyramidal tract sign. A report of Shiga-type HMSN-P
written in Japanese did not mention the involvement of pyramidal tract5.
There was no description of the involvement of the corticospinal tract in
the original paper by Takashima et al6. When the lower motor neurons and
large sensory neurons in the dorsal root ganglion are severely damaged,
pyramidal tract signs might be masked. That might be why we did not find
any pyramidal tract signs in HMSN-P patients. I think that involvement of
the pyramidal tract in HMSN-P is much more important than the presence of
optineurin-positive inclusions in the lower motor neurons. But, we should
be careful, because their postscript1 is a single case report. Moreover,
the patient had multiple cerebral infarctions. These infarctions might
explain the bilateral Babinski sign and pathological changes in the
lateral columns. They did not show the density of Bets cells of the
precentral gyrus or changes of the myelinated fibres in the posterior
limbs of the internal capsule. Although their pathological study is quite
important in the understanding of the pathomechanisms of HMSN-P or
familial ALS, I think that it might be too early to propose the new
classification of HMSN-P into the subgroup of ALS.
References
1)Fujita K, Yoshida M, Sako W, et al. Brainstem and spinal cord motor
neuron involvement with optineurin inclusions in proximal-dominant
hereditary motor and sensory neuropathy. J Neurol Neurosurg Psychiatry
2011;82:1402-3.
2)Maeda K, Sugiura M, Kato H, et al. Hereditary motor and sensory
neuropathy (proximal dominant form, HMSN-P) among Brazilians of Japanese
ancestry. Clin Neurol Neurosurg 2007;109:830-2.
3)Patroclo CB, Lino AMM, Marchiori PE, et al. Autosomal dominant HMSN with
proximal involvement: New Brazilian cases. Arq Neuropsiquatr 2009;67:892-
6.
4)Maeda K, Kaji R, Yasuno K, et al. Refinement of a locus for autosomal
dominant hereditary motor and sensory neuropathy with proximal dominancy
(HMSN-P) and genetic heterogeneity. J Hum Genet 2007;52:907-14.
5)Takahashi M, Mitsui Y, Yorifuji S, et al. Clinical report of hereditary
motor and sensory neuropathy with proximal dominance in Shiga prefecture.
Rinsho Shikeigaku 2007;47:571-6.
6)Takashima H, Nakagawa M, Nakahara K, et al. A new type of hereditary
motor and sensory neuropathy linked to chromosome 3. Ann Neurol
1997;41:771-80.
Ioannis Mavridis, M.D., Sophia Anagnostopoulou, M.D., Ph.D., Assoc. Professor
Department of Anatomy, University of Athens School of Medicine
Corresponding author: Ioannis N. Mavridis, M.D. Department of Anatomy, Medical School, University of Athens, Mikras Assias str. 75, Goudi, 11527 Athens, Greece Tel.: 0030-210 74 62 404 Fax: 0030-210 74 62 398 e-mail: pap-van@otenet.gr
Dear Sir/Madam,...
Pyramidal tract involvement in HMSN-P?
Kengo Maeda, Department of Neurology, National Hospital Organization Shiga Hospital 255 Gochi, Higashi-oumi, Shiga 527-8505, Japan Tel: +81-748-22-3030 Fax: +81-748-23-3383 E-mail: maeda-kengo@shiga-hosp.jp
Dear Editor,
I have read a postscript 'Brainstem and spinal cord motor neuron involvement with optineurin inclusions in proximal-dominant hereditary mo...
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