Central nervous system involvement in CD4+/CD56+ hematodermic neoplasm: a report of two cases.

CD4+/CD56+ hematodermic neoplasm, formerly known as blastic NK-cell lymphoma, is an uncommon, aggressive non-Hodgkin's lymphoma with cutaneous, lymph node, and bone marrow involvement at presentation. The disease is characterized by early leukemic phase; however, central nervous system involvement is rarely reported. Herein we describe two cases of CD4+/CD56+ hematodermic neoplasm with meningeal manifestation. Microscopic analysis and flow cytometry of cerebrospinal fluid proved to be diagnostic; however, imaging studies were not informative. These observations call attention to the possibility of central nervous system involvement, which could be more common than expected previously. Authors recommend routine cerebrospinal fluid analysis and prophylactic intrathecal chemotherapy in patients with this highly aggressive disease.

Simultaneous central and peripheral nervous system involvement in systemic lupus erythematosus.

Systemic lupus erythematosus is a frequent autoimmune disease, affecting several organs, including the brain, spinal cord and nerves. Cerebral vasculitis, transverse myelitis and polyneuropathy are the most common neurological manifestations. We report a case of a 46 years old woman who suffered incomplete transverse myelitis at the age of 44. After 2 years the second relapse presented with arthralgias, painful paraesthesias and weakness of the lower limbs. Neurological signs suggested involvement of the central and the peripheral nervous system. Based upon clinical and laboratory findings systemic lupus erythematosus was diagnosed. Magnetic resonance imaging revealed two hyperintense lesions on T2 weighted scans within the cervical spinal cord. The brain scan was normal. Protein content was slightly elevated in the cerebrospinal fluid, with normal cell count. Electrophysiological examinations diagnosed a subacute sensory-motor axonal polyneuropathy. On methylprednisolone treatment her condition improved. Simultaneous development of central and peripheral lesions of the nervous system in cases with systemic lupus erythematosus may lead to a challenge to establish the diagnosis.

One-and-a-half syndrome--two cases.

One-and-a-half syndrome is characterized by combination of the clinical features of unilateral horizontal gaze palsy and internuclear ophthalmoplegia. The common symptoms are double vision and oscillopsia. The lesion is located in the paramedian pontine reticular formation, involving the centre of horizontal gaze and medial longitudinal fasciculus. More extensive brainstem damage may result in additional neurological signs. The most frequent underlying diseases are vascular insults, multiple sclerosis, and brainstem tumor. We present two cases of one-and-a-half syndrome. Both patients had lacunar infarction in the paramedian pontine tegmentum, revealed by MRI. The first patient had isolated eye movement disorder, while the second had additional nuclear-type facial paresis. In the first case brainstem evoked potentials indicated brainstem damage, in the second patient it was normal. Ocular symptoms improved within some days in both patients.

[Aids-related toxoplasma-encephalitis presenting with acute psychotic episode]. / Akut psychosis mint az AIDS-hez társuló toxoplasma-encephalitis elso tünete.

The most frequent neurological manifestations of the Acquired Immunodeficiency Syndrome-(AIDS) are Cerebral Toxoplasmosis, Primary Central Nervous System Lymphoma (PCNSL), Progressive Multifocal Leukoencephalopathy (PML) and AIDS-encephalitis (AIDS-dementia complex, multinucleated giant cell encephalitis, HIV-encephalopathy). Neurological complications usually occur in the advanced stages of the disease, and they are uncommon in the beginning as presenting illness, but may result in life-threatening condition or in death. Rarely the disease presents as a neuropsychiatric illness in an undiagnosed AIDS patient, delaying a proper diagnosis. We present the case of a 34 years old patient treated for AIDS-related Toxoplasma-encephalitis in our department. His illness started as an acute psychosis followed by rapid mental and somatic decline, leading to death in three months. His HIV-seropositivity was not known at his admission, and the extraneural manifestations were slight. The diagnosis was established by serology, imaging methods and histopathological investigation. After presenting the medical history and results of autopsy studies of the patient we discuss the problems of the differential diagnosis, especially regarding the findings of the imaging methods.

[Clinical analysis of patients with peripheral facial palsy]. / Perifériás facialis paresis klinikánk betegei között.

UNLABELLED: Peripheral facial palsy is one of the most frequent neurological symptoms. In two thirds of the cases the cause is unknown, this is called "idiopathic peripheral facial palsy or Bell's palsy", but several different diseases have to be considered in the differential diagnosis. In this paper we reviewed the case histories of 110 patients treated for "peripheral facial palsy" in the Department of Neurology, Semmelweis University, Budapest in a five year period, 2000-2004. We studied the age, gender distribution, seasonal occurrence, comorbidities, sidedness, symptoms, circumstances of referral to the hospital, the initial diagnoses and therapeutic options. We also discuss the probable causes and consequences of diagnostic failures. RESULTS: the proportion of males and females was equal. There was no considerable difference between sexes regarding age-distribution. Of the 110 patients 106 was diagnosed with idiopathic Bell's palsy, three cases with otic herpes zoster and one patient with Lyme disease. In our material, peripheral facial palsy was significantly more frequent in the cold period of late autumn, winter, and early spring. Diabetes mellitus and hypertension were more frequent than in the general population. 74% of the patients were admitted within two days from the onset of the symptoms. In 37% preliminary diagnosis was unavailable. In 15% cerebrovascular insult was the first, incorrect diagnosis, the correct diagnosis of "Bell's palsy" was provided only in 16%. The probable causes of diagnostic failures may be the misleading symptoms and accompanying conditions. We examined the different therapies applied and reviewed the literature in this topic. We conclude that intravenous corticosteroid treatment in the early stage of the disease is the therapy of choice.

[Cerebral sinus thrombosis and ulcerative colitis: two cases]. / Agyi sinusthrombosis és colitis ulcerosa--két eset ismertetése.

Inflammatory bowel diseases (IBD)--ulcerative colitis and Crohn's disease--are associated with increased risk for thrombotic complications both in the arterial and venous system. Cerebral sinus thrombosis is a rare but potentially fatal consequence of these diseases. Modern imaging methods made this uncommon complication of IBD more frequently recognized. The link between IBDs and thrombosis has been extensively studied. Inherited coagulation disorders (APC resistance, antithrombin III and protein-S deficiency), acquired diseases (antiphospholipid syndrome), and the frequent use of corticosteroids were suspected. Two cases of ulcerative colitis associated with cerebral sinus thrombosis successfully treated are reported. The connection between IBD and thrombotic complications and the therapeutic risks are discussed as well.