CC BY
24DIFFERENTIAL DIAGNOSING WITHMRIPATTERNS OF CNS LESION IN NMOSD AND MS
Daminova Khilola Maratovna
MD in Tashkent Medical Academy Shokhimardonov Shokhijakhon Shokhimrdonovich
Master student in Tashkent Medical Academy
Opticomyelitis or Devik's disease is an autoimmune progressive demyelinating disease of the central nervous system, with a predominant lesion of the optic nerves and spinal cord, which can lead to both loss of vision and death.
One of the common causes of mortality in this disease is the difficulty associated with the differential diagnosis of opticomyelitis with multiple sclerosis. A distinctive difficulty in diagnosis is the clinical cases of opticomyelitis in which periventricular asymptomatic foci in the brain can be detected. Their clinic and laboratory data can be very similar, but they need to be differentiated among themselves in the selection of therapy, since the drugs used for multiple sclerosis are not only ineffective in patients with opticomyelitis, but can even cause an increase in the frequency of exacerbations.
Due to the reasons for the high disability of patients (blindness: in at least one eye in 50% of patients with recurrent and 25% of patients with a monophasic form of the disease) and mortality, the optimization of clinical trial methods is still an urgent issue.
To date, there are already the main diagnostic methods for Opticomyelitis developed by the international team for the diagnosis of Opticomyelitis. That is why the integration of these methods into the realities of Uzbekistan is an extremely important task.
Key words: NMOSD, MS, MRI, CNS lesion.
ДИФФЕРЕНЦИАЛЬНАЯ ДИАГНОСТИКА БОЛЬНЫХ С РС И ОПТИКОМИЕЛИТОМ ПРИ ПОМОЩИ МРТ
ИССЛЕДОВАНИЯ
Даминова Хилола Маратовна
доцент кафедры нервных болезней Ташкентская Медицинская Академия Шохимардонов Шохижахон Шохимардонович
магистр кафедры неврологии Ташкентская Медицинская Академия
Оптикомиелит или болезнь Девика является аутоиммунным прогрессирующим демиелинизирующим заболеванием центральной нервной системы, с преимущественным поражением зрительных нервов и спинного мозга, которое может привести как к потерею зрения так и к летальному исходу.
Одной из частых причин летальности данного заболевания является трудности связанные с проведением дифференциальной диагностики оптикомиелта с рассеянным склерозом. Отличительную трудность при диагностике составляют клинические случаи оптикомиелита при которых можно обнаружить перивентрикулярные асимптомные очаги в головном мозге. Их клиника и лабораторные данные могут быть очень похожими, но их нужно дифференцировать между собой при подборе терапии, поскольку препараты, применяемые при рассеянном склерозе не только неэффективны у больных c оптикомиелитом, но даже могут стать причиной повышения частоты обострений.
По причины высокой инвалидизации больных (слепота: по крайнеи мере на один глаз у 50% пациентов с рецидивирующеи и у 25% пациентов с монофазнои формои заболевания) и летальности исхода, оптимизация методов клинического исследования является все еще актуальным вопросом.
На сегодняшний день уже имеются основные способы диагностики Оптикомиелита разработанные международной группой по диагностике Оптикомиелита. Именно поэтому интеграция данный методов в реалии Узбекистана является крайне важной задачей.
Ключевые слова: Оптикомиелит, РС, МРТ, очаги в ЦНС.
DOI: 10.24411/2181-0443/2020-10016
Demyelinating diseases of the central nervous system (CNS) are the second most frequent cause of disability in young people among neurological diseases (Confavreux, 2006; Frost, 2008). Since the able-bodied population suffers, demyelinating diseases are
not only a medical, but also a socio-economic problem, to the solution of which considerable resources are attracted, both in Uzbekistan and around the world (Spirin, 2015). Optoneuromyelitis is a fairly rare disease among Europeans, while in the countries of Southeast Asia, the incidence of OHM among all demyelinating diseases reaches 20-30% (Kira, 2003). However, the improvement of diagnosis, the development of criteria for the diagnosis of optoneuromyelitis makes us re-evaluate the relevance of this problem in modern neurology. Despite the successes achieved in studying the pathogenesis and semiotics of demyelinating diseases, the number of erroneous diagnoses of PC reaches 30%, especially in isolated syndromes (optic neuritis, acute transversal myelopathy, pseudotumor PC), as well as rare variants of Marburg and Balo (Jarius, 2012 ) Multiple sclerosis, as well as isolated syndromes: myelitis and optic neuritis, present the greatest difficulties in the differential diagnosis of optoneuromyelitis (Melnikov, 2012). Occurrence data currently in Uzbekistan optoneuromyelitis (NMO) and associated disorders are not submitted; individual cases described optic neuromyelitis, characterized by a combination of optical neuritis and longitudinal spread myelitis, a more severe course and worse prognosis than multiple sclerosis in the CIS countries (Totolyan, 2009). In addition, cases of detection of optical neuritis and myelitis in case of systemic autoimmune diseases manifesting from neurological manifestations have been described (Pizova, 2012), there is no clear data on this issue in the Russian population, and there is no concept of the spectrum of optoneuromyelitis disorders. The study of the diagnostic significance of MRI is of great importance for the differential diagnosis of demyelinating diseases of the central nervous system and determine the tactics of a particular patient. Ultimately, a strong recognition of the spectrum of MRI brain findings in NMOSD is critical in accurate diagnosis. Several brain lesion patterns are highly suggestive of NMOSD, whereas others may serve as red lags. Lesions involving the diencephalon, periependymal tissues of the lateral or 4th ventricle, corpus callosum, or corticospinal tract (CST) are necessary to support the diagnosis of NMOSD. In this pictorial review, we present the spectrum of typical NMOSD brain lesions in NMOSD from a single institutional series of subjects diagnosed based on 2015 consensus criteria.
Clinical and demographic characteristics. Thirty cases of NMOSD diagnosed based on agreed 2015 criteria were reviewed. The average age of the patients was 32 years (range 27-49 years). The median age at the time of diagnosis was 24.7 years (range 10-39 years). 21 (70%) were female. Of the 30 patients, 17 (56%) initially had OH, 9 (30%) had transverse myelitis, and 4 (13%) had symptoms almost simultaneously associated with both.
Imaging characteristics. The agreed criteria of 2015 recognize the relatively high incidence of brain MRI abnormalities in patients with NMOSD, with a frequency of 50 to 85% based on the revised diagnostic criteria of 2006 and 51 to 89% in seropositive patients with NMOSD. In 24 (86%) cases, violations of MRI of the brain were detected. It is assumed that the differences in frequency between existing studies are partly due to the fact that brain damage is becoming more common with the duration of the disease. Summary of typical patterns of brain damage in NMOSD according to Wingerchuk Wang are given in Table-1, and the prevalence of these brain lesions in our series is presented in Table-2.
Typical MRI patterns of brain damage in NMOSD
Table 1
1. Diencephalon lesions involving the thalamus and hypothalamus adjacent to third ventricle.
2. Cerebellar and dorsal brainstem lesions adjacent to fourth ventricle._
3. Dorsal medulla lesions, particularly the area postrema._
4. Long, contiguous CST lesions._
5. Hemispheric deep or subcortical cerebral white matter lesions._
6. Periventricular white matter lesions adjacent to lateral ventricle, including corpus callosum.
CST, corticospinal tract; NMOSD, neuromyelitis opticaspectrum disorder.
Table 2
Overview of brain lesions in 30 patients with NMOSD based on 2015 diagnostic
criteria
Imaging finding Frequency
Periependymal lesions :
Diencephalon 3/30
Dorsal brainstem/cerebellum 11/30
Lateral periventricular 14/30
Absent 9/30
Enhancement
Cloud-like 5/30
Nodular 1/30
Pencil-thin 1/30
Ring 0/30
Leptomeningeal 3/30
Absent 10/30
Diffusivity:
Increased 11/30
No change 20/30
Other:
CST lesions 3/30
Cerebral hemispheric lesions 5/30
Figure -1
Diencephalic lesions surrounding (A.a) the third ventricles and cerebral aqueduct, (A.b) which include thalamus, hypothalamus, and (A.c) anterior border of
the midbrain
Figure - 2
(B.a) Dorsal brain - stem lesion adjacent to the fourth ventricle, (B.b) linear medullary lesion that is contiguous with cervical cord lesion, (B.c) edematous and extensive dorsal brainstem lesion involving the cerebellar peduncle
Supratentorial white matter lesions: These lesions are commonly asymptomatic. In our patients incidence rang was 64%, but longitudinal studies demonstrating asymptomatic white matter lesions accumulating in 60% of NMOSD patients(4).
Diencephalic lesions surrounding third ventricle and cerebral aqueduct: Lesions within these regions correspond to areas of high AQP-4 expression.(5) Diencephalic involvement of peripenidma was noted in 3 of 30 cases in our study. Similar CNS involvement can be defined as atypical and exceptional for MS and relatively characteristic, but not pathognomonic for NMOSD (Figure - 1).
Dorsal brainstem lesions surrounding fourth ventricle: Peripendimal lesions of the regions around spinal cord and cerebellum adjacent to the fourth ventricle, especially in the area are the most specific findings in brain MRI for NMOSD. Overall, these lesions were found in 11 of 30 cases in our study (Figure - 2).
Corticospinal tract lesions: CST involvement is not uncommon and found in 22 to 44% of patients.(l) It is interesting that only in 3 cases it was possible to determine the participation of CST on an MRI. The outbreak can be single-sided or double-sided, and an extended and continuous configuration is typical of NMOSD. For example, lesions can spread from the white matter of the brain through the hind limb of the inner capsule and cerebral peduncles to the heads. This longitudinal configuration is analogous to the LETM observed in the spinal cord.
Hemispheric cerebral white matter lesions: Lesions of the white matter of the brain in the hemisphere may indicate a possible diagnosis of NMOSD, but they are difficult to distinguish from atypical lesions in MS when they are presented as an isolated opening. In our study, we observed 5 cases out of 30 patients. Damages, usually greater than 3 cm in diameter, can be unilateral or bilateral and include subcortical white matter or deep white matter.
Lesions surrounding lateral ventricles: Periependymal lesions surrounding the lateral ventricles have been reported to be less commonly found than in those in the third and fourth ventricles, with studies observing them in 12 to 40% of patients.(5) In our research we have found 14 of 30 cases with such lesion on MRI.
Enhancing lesions: Presence of brain lesion enhancement has been reported in 9 to 36% of patients. (4) Although the relationship between amplification and acute relapse was not well understood, it was sometimes reported that linear perpendimal amplification disappeared after acute relapse, according to our patients, see Table - 2.
Diffusivity: The presence and extent of changes in weighted images is not well described. A diffusional tensor image can reveal changes in our occultism with conventional MRI and demonstrate a higher average diffusivity in the regions of both white and gray matter. In our diffusion weighted visualization series, 11 out of 28 cases showed
increased diffusivity.
Conclusions: The discovery of AQP4-Ab, the subsequent expansion of the clinical phenotype of NMOSD, the ability to diagnose NMOSD in the absence of damage to the optic nerve or spinal cord, and the growing recognition of brain involvement as one of the hallmarks of NMOSD, in general, made it possible to obtain images with MRI in a more important role in diagnosis. Typical patterns include long lesions of CST, lesions of the white matter of the brain in the hemisphere and periependimal lesions in the diencephalon, dorsal brain stem, white matter adjacent to the lateral ventricles. Accurate recognition of the MRI spectrum of the brain with NMOSD is important for accurate diagnosis, especially when differentiated from MS. This study confirms that early MRI diagnosing results among patients with NMOSD in Uzbekistan usually demonstrate involvement of the brainstem and hemispheres periventricular white matter. Future studies of the relationship between imaging and remission may allow imaging to play a more important role in assessing response to treatment.
REFERENCES:
1. Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. e spectrum of neuromyelitis optica. Lancet Neurol 2007; 6: 805-15. doi: https://doi.org/ 10.1016/S1474-4422(07)70216-8
2. Pittock SJ, Lennon VA, Krecke K, Wingerchuk DM, Lucchinetti CF, Weinshenker BG. Brain abnormalities in neuromyelitis optica. Arch Neurol 2006; 63: 390-6. doi: https://doi.org/10.1001/archneur. 63.3.390
3. Kim HJ, Paul F, Lana-Peixoto MA, Tenembaum S, Asgari N, Palace J, et al. MRI characteristics of neuromyelitis optica spectrum disorder: an international update. Neurology 2015; 84: 1165-73. doi: https://doi.org/10.1212/WNL. 0000000000001367
4. Pittock SJ, Weinshenker BG, Lucchinetti CF, Wingerchuk DM, Corboy JR, Lennon VA. Neuromyelitis optica brain lesions localized at sites of high aquaporin 4 expression. Arch Neurol 2006; 63: 964-8. doi: https://doi.org/ 10.1001/archneur.63.7.964
5. Kim W, Park MS, Lee SH, Kim SH, Jung IJ, Takahashi T, et al. Characteristic brain magnetic resonance imaging abnormalities in central nervous system aquaporin-4 autoimmunity. Mult Scler 2010; 16: 1229-36. doi: https://doi.org/10.1177/ 1352458510376640
