Imaging aspect of neuromyelitis optica: a case report and review of the literature

The case report presents a neuromyelitis optica in a 19 years old male. Brain and spinal cord MRI showed bilateral optic neuropathy, multiphasic demyelinating process involving the cervical and thoracic spinal cord. Cerebrospinal fluid showed negative NMO Ig G. We will describe the radiological aspect of neuromyelitis optica with a review of the literature.

Tumefactive demyelinating CNS lesion in a 60-year-old woman with familial Mediterranean fever

SummaryWe here report on a 60-year-old woman with familial Mediterranean fever (FMF) who developed cognitive impairment 16 years after initial diagnosis. On MRI, a new extensive white matter lesion in the right frontal lobe with mild local mass effect but without contrast enhancement was detectable and classified as a tumefactive lesion. Additional MR spectroscopy showed markedly increased choline levels accompanied by a significant lactate peak, highly suggestive of a low-florid demyelinating process. Although diffuse central nervous system (CNS) lesions have been described in single FMF cases, tumefactive lesions have not been observed in FMF patients without concomitant multiple sclerosis. In summary, this case highlights rare differential diagnoses of atypical, inflammatory CNS lesions and the clinical utility of MR spectroscopy.

ASSESSMENT OF THE DEMYELINATING PROCESS ACTIVITY IN PATIENTS WITH HERPESVIRAL MENINGITIS AND MENINGOENCEPHALITIS BASED ON THE LEVEL OF MYELIN BASIC PROTEIN (MBP) IN THE CEREBROSPINAL FLUID

The aim: To study the peculiarities of demyelination by detection of changes in the levels of myelin basic protein (MBP) in CSF of patients with acute herpesviral meningitis (M) and meningoencephalitis (ME). Materials and methods: A total of 136 CSF samples from 68 patients with herpesviral M and ME were collected. The control group consisted of patients with acute respiratory infection and meningismus. MBP level in CSF was identified at the admission and after 10-12 days of treatment. Analysis of MBP concentrations in CSF was performed using an enzyme immunoassay. Results: Examination of patients on the first day of hospitalization showed the presence of a significant increase of MBP in the CSF in all patients with viral M/ME compared with the indicators of the comparison group (р<0.01). In all groups of patients with ME, the level of MBP in CSF was significantly higher than the indicators of comparison group and M groups of the suitable etiology of the disease (p<0.01). In patients with lethal outcome, the MBP level was significantly higher (p<0.01) than in all meningitis groups, but we did not find a significant difference with the patients with ME (p>0.05). Conclusions: The increase of MBP level identified in patients with acute M/ME confirms the presence of the demyelinating process that occurs in all patients, but it is more pronounced in patients with ME.

IDENTIFYING POST-PARTUM OPTIC NEURITIS IN RURAL AREAS: A CASE REPORT

Background: Optic neuritis is an inflammatory demyelinating process that causes acute impairment of vision that may appear in one or both eyes, either simultaneously or successively. Case: A 22-year-old woman admitted to Neurology Clinic with chief complaint of sudden vision loss in both eyes simultaneously in the last two days. Additional complaints like headache and dizziness also occur. The patient was in her 7th day of post-partum period when this happened. There was no problem during pregnancy nor delivery. The initial visual acuity was 1/300 in both eyes. The patient then admitted and treated with high-dose corticosteroid. After treatment, the patient went home with visual acuity of more than 6/60. Discussion: This patient may have B6, B12, and folic acid deficiency that can cause demyelinating process of optic nerve. It is also possible as an early manifestation of Multiple Sklerosis, an autoimmune process that arises during post-partum period. Conclusion: Pregnancy has a protective effect to autoimmune disease, whereas post-partum period is immunologically more vulnerable to autoimmune disease. Keywords: optic neuritis, post-partum, sudden vision loss

Levamisole-induced leukoencephalopathy

A medical case of an acute demyelinating process in cerebrum caused by taking levamisole is presented. The issues of pathogenesis and differential diagnosis of levamisole-induced leukoencephalopathy with acute disseminated encephalomyelitis, multiple sclerosis, progressive multifocal leukoencephalopathy, and cerebral lymphoma are discussed.

Neuromotor impairment, hearing loss and blindness in a preclinical mouse model of Charcot Marie-Tooth disorder

Schwann cells produce myelin sheath around peripheral nerve axons. Myelination is critical for rapid propagation of action potentials, as illustrated by the large number of acquired and hereditary peripheral neuropathies, such as diabetic neuropathy or Charcot-Marie-Tooth (CMT) diseases, that are commonly associated with a process of demyelination. Peripheral neuropathy is a major complication of diabetes, and the pathomechanism of the disease remains poorly studied. Here, we studied the progressive demyelinating process, hearing impairment and blindness observed in the CMT1A mouse model C3. Our results confirm that these mice represent a robust and validated model to study the peripheral neuropathy induced by CMT disorder allowing to determine the efficacy of new pharmacological candidates targeting demyelinating diseases such as CMT1A disorder.

More on differential diagnosis of demyelinating and hereditary diseases

Демиелинизирующие заболевания являются гетерогенной группой, включающей рассеянный склероз (РС), острый рассеянный энцефаломиелит, оптиконевромиелит (ОНМ), идиопатический поперечный миелит, оптический неврит. Дифференциально-диагностический поиск при демиелинизирующих заболеваниях включает в себя в том числе и наследственные/нейродегенеративные заболевания. Сходство некоторых митохондриальных болезней (МБ) с РС не случайно, поскольку в настоящее время этиопатогенез РС рассматривается не только как аутоиммунная демиелинизация, но и как нейродегенерация, одним из важных патогенетических механизмов которой является митохондриальная дисфункция. В статье проведен краткий анализ пациентов с нарушением зрительных функций и нетипичной для РС клинической картиной. Критерием отбора больных был первичный клинический эпизод, вызванный предположительно воспалительным демиелинизирующим процессом и жалобами на снижение зрительных функций. Demyelinating diseases are heterogeneous group including multiple sclerosis (MS), acute multiple encephalomyelitis, opticoneuromyelitis (ONM), idiopathic transverse myelitis, optical neuritis. The spectrum of differential diagnostic screening in this rare autoimmune disease is variable, including hereditary/neurodegenerative diseases. The similarity of some mitochondrial diseases (MD) with MS is not accidental, since currently etiopathogenesis of MS is considered not only as autoimmune demyelination, but also as neurodegeneration, one of the important pathogenetic mechanisms of which is mitochondrial dysfunction. The article provides a brief analysis of patients with impaired visual functions and atypical clinical-diagnostic picture for MS. The selection criterion for patients was a primary clinical episode caused by a presumably inflammatory demyelinating process and complaints of decreased visual functions.