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Clinical Neuroscience

JULY 30, 2020

Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

TOLVAJ Balázs, HAHN Katalin, NAGY Zsuzsanna, VADVÁRI Árpád, CSOMOR Judit, GELPI Ellen, ILLÉS Zsolt, GARZULY Ferenc

Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.

Clinical Neuroscience

JULY 30, 2018

Atypical type of Hirayama disease: Onset of proximal upper extremity

AYAS Özözen Zeynep, ASIL Kıyasettin

Hirayama disease is a rare, benign motor neuron disease. It has been proposed that the dura mater’s posterior wall lacks sufficient elasticity in the lower cervical region and this causes the tense dura part to displace anteriorly upon flexion. The disease is described as involving unilateral upper extremity with a distal-onset. We reported weakness and atrophy of the proximal part of an extremity in a 45-year-old man who is diagnosed with Hirayama disease. Proximal onset is a rare type of Hirayama Disease. Clinicians must be alert of proximal involvement and the diagnosis should be confirmed with electrophysiological and flexion MRI studies.

Clinical Neuroscience

JANUARY 30, 2018

Insights into the structure and function of the hippocampal formation: Relevance to Parkinson’s disease

GYÖRFI Orsolya, NAGY Helga, BOKOR Magdolna, KÉRI Szabolcs

The link between the hippocampus and declarative memory dysfunctions following the removal of the medial temporal lobe opened unexplored fields in neuroscience. In the first part of our review, we summarized current theoretical frameworks discussing the role of hippocampus in learning and memory. Several theories are highlighted suggesting that the hippocampus is responsible for assembling stimulus elements into a unitary representation that later can be utilized to simulate future events. The hippocampal formation has been implicated in a growing number of disorders, from neurodegenerative diseases to atypical cognitive ageing and depression. Recent neuroimaging studies provided new opportunities to study in detail the hippocampal formation’s role in higher levels of the nervous system. We will present data regarding the regional specialization of the hippocampus in experimental models developed for healthy and neurological conditions with a special focus on Parkinson’s disease. Combined evidence from neuroimaging studies suggested that hippocampal volume is reduced in non-demented, newly diagnosed patients with Parkinson’s disease, which is associated with impaired memory performance. These findings proposed that, beyond the well-known striatal dopamine loss, impaired hippocampal synaptic plasticity may contribute to cognitive and affective impairments in early Parkinson’s disease.

Clinical Neuroscience

MAY 25, 2014

[LADA type diabetes, celiac diasease, cerebellar ataxia and stiff person syndrome. A rare association of autoimmune disorders]

SOÓS Zsuzsanna, SALAMON Mónika, ERDEI Katalin, KASZÁS Nóra, FOLYOVICH András, SZŰCS Anna, BARCS Gábor, ARÁNYI Zsuzsanna, SKALICZKI József, VADASDI Károly, WINKLER Gábor

[Celiac disease - in its typical form - is a chronic immunemediated enteropathy with typical clinical symptoms that develops against gliadin content of cereal grains, and is often associated with other autoimmune diseases. In cases of atypical manifestation classic symptoms may be absent or mild, and extra-intestinal symptoms or associated syndromes dominate clinical picture. The authors present a longitudinal follow-up of such a case. A 63-years old woman was diagnosed with epilepsy at the age of 19, and with progressive limb ataxia at the age of 36, which was initially thought to be caused by cerebellar atrophy, later probably by stiff person syndrome. At the age 59, her diabetes mellitus manifested with type 2 diabetic phenotype, but based on GAD positivity later was reclassified as type 1 diabetes. Only the last check-up discovered the celiac disease, retrospectively explaining the entire disease course and neurological symptoms. By presenting this case, the authors would like to draw attention to the fact that one should think of the possibility of celiac disease when cerebellar ataxia, progressive neurological symptoms and diabetes are present at the same time. An early diagnosis may help to delay the progression of disease and help better treatment.]

Lege Artis Medicinae

APRIL 20, 2014

[Diagnostic challenges in our adolescent patient with lymphoma]

MAGYARI Ferenc, RAJNAI Hajnalka, BARNA Sándor, MILTÉNYI Zsófia, VÁRÓCZY László, CSOMOR Judit, UDVARDY Miklós, ILLÉS Árpád

[OBJECTIVES - Unclassifiable B-cell lymphoma, which shows intermediate features typical for both diffuse, large B-cell lymphoma (DLBCL) and classical Hodgkin lymphoma (HL) is a novel category of diffuse, large B-cell lymphomas (DLBCL/HL), described in the WHO classification in 2008. This rare type of lymphomas presents peculiar clinical, morphological and immunophenotypical patterns, previously called gray-zone lymphomas. CASE REPORT - In December 2011 a 17- year old boy was diagnosed with mixedcellularity subtype of classical HL on the basis of left inguinal lymph node biopsy. Staging examinations revealed a IV/BXS (abdominal bulky) stage disease with unfavourable prognosis. Because of the unusally extended disease (nodal-extranodalbulky), a histological revision was performed. After a half course of ABVD chemotherapy the patient’s symptoms disappeared and the sizes of the involved lymph nodes decreased. On the basis of the histological revision, the diagnosis was changed to DLBCL/HL, so the treatment was modified to R-CHOP-14 regimen. After 3 cycles of R-CHOP-14 a complete metabolic remission (CMR) was achieved, which was confirmed by a 18FDG-PET/CT scan. Staging examinations after further 4 cycles of RCHOP- 14 therapy showed that the patient was still in CMR, but a PET-negative large mass (7×3 centimeter) still remained visible in the abdominal region. Considering this residual tumour and the agressive subtype of lymphoma the patient was referred for an autologous haemopoietic stem cell transplantation (AHSCT). After 2 cycles of R-DHAP regimen, successful CD34- positive stem cell collection was performed in August 2012. In September 2012, a RLAM-BEAM conditioning was performed followed by AHSCT. Posttransplantation 18FDG-PET/CT scan revealed further morphological regression, no symptom of the underlying disease appeared and the patient is in complete remission for 15 months. CONCLUSIONS - This case exemplifies that in case of atypical clinical findings and unusual progress of the disease it might be worthwile to re-evaluate the case and the (histological) diagnosis, which requires a close cooperation between the clinician and the pathologist.]

LAM KID

MAY 30, 2013

[A magnézium és csonthatásai]

BAJNOK Éva

[Since 1932, a number of animal studies have demonstrated the correlation of hypomagnesaemia and hypocalcaemia, and the variety of skeletal abnormalities resulting from low magnesium (Mg) intake. Several studies have shown that patients with osteoporosis have a decreased serum magnesium level, which is related to decreased bone mineral content and increased bone fragility. Mg has multiple physiological effects, thus it is not surprising that dozens of hypomagnesaemia-related diseases and symptoms have been reported. Adequate Mg concentration is necessary for the secretion of parathormone and its effect on target organs, activation of vitamin D in the kidney, the maintenance of calcium homeostasis, bone mineralisation and regeneration. Mild hypomagnesaemia is associated with general, atypical symptoms, whereas severe Mg deficiency is a life-threatening condition. Its concentration should be measured in serum and urine. Mg metabolism is determined by its absorption from the intestines and reabsorption in the kidneys. Recently revealed details of these processes give some insights into the mechanisms underlying a number of Mg deficient conditions related to genetic or medical reasons. Mg supplementation may be indicated for patient populations with the highest risk of hypomagnesaemia. For supplementation, the recommended total Mg dose is 350 mg, first in higher doses, several times per day for a longer period, complemented with Ca and K supplementation. Overdosing can only occur in patients with impaired renal function, which necessitates careful monitoring. Adequate Mg supplementation is an inexpensive, safe and effective preventive and therapeutic option for many diseases.]

Clinical Neuroscience

MAY 30, 2013

[Benign-onset acute disseminated encephalomyelitis: A report on two cases]

DEGIRMENCI Eylem, ERDOGAN Cagdas, OGUZHANOGLU Attila, BIR Sinan Levent

[The signs and symptoms of acute disseminated encephalomyelitis are heterogeneous and dependent on the location and severity of the inflammatory process. The meningoencephalitic presentation may include meningism, impaired consciousness (occasionally leading to coma), seizures and confusion, or behavioral disturbances. Multifocal neurological features include a combination of optic neuritis, visual field defects, cranial neuropathy, sensorimotor impairment, ataxia, aphasia, and involuntary movements. One definition of acute disseminated encephalomyelitis is “an initial clinical event with a presumed inflammatory and demyelinating cause, with acute or sub-acute onset affecting multifocal areas of the central nervous system”. Patients with acute disseminated encephalomyelitis frequently suffer from seizures, disturbances of consciousness, fever, and headaches, and occasionally there are focal signs and symptoms. Here, we report on two cases who presented with different symptoms, but the clinical findings that the patients showed were benign.]

Clinical Neuroscience

JANUARY 30, 2012

[Depression in neuropsychiatric diseases]

HIDASI Zoltán, SALACZ Pál, CSIBRI Éva

[Depression is frequently observed together with neurological disorders. Moreover this connection is bidirectional in the case of several neurological disorders, as depression can be either a comorbide syndrome or also a risk factor of them. Neurobiological background of depression involves neuroanatomical structures, their interconnected networks, disturbances of neurotransmitters, neurohormonal, neuroimmunological and neurotrophic changes, genetic background. Disfunction of these systems also plays a role in the pathogenesis of comorbid depression of neurological disorders. Interactions and clinical aspects of biological factors involved in the pathogenesis of depression in dementias, Parkinson’s disease, cerebrovascular disorders and epilepsy are discussed further. Depression as a result of neurobiological factors responsible for both neurological and psychiatric consequencies of these disorders, are often atypical as a clinical manifestation, however chracteristic for the particular neurological disorder. Evaluation of the biological backgound and clinical features of depression in neurological disorders makes the complex neuropsychiatric approach of these disorders possible.]