Primary malignant meningeal melanomatosis: a clinical, radiological and pathologic case study

A 40 year-old woman with subacute headache and visual impairment was admitted. Neurological examination revealed meningismus, diminished visual acuity, bilateral sixth cranial nerve palsy, and papillary edema. Dermatologic examination was normal. The brain CT scan showed hydrocephalus and hyperdense edging around fissures and sulci. The CSF study showed an increased protein level, with persistently negative microbiologic and cytological studies. Prior to Gd-DPTA injection, the brain T1-WI MRI revealed leptomeningeal hyperintensity. A dark subpial substance became evident at cerebral biopsy. The histopatological diagnosis was diffuse leptomeningeal melanomatosis. This case report highlights the diagnostic value of the brain MRI findings in primary leptomeningeal melanomatosis, a rare pathologic condition diagnosed in most published cases only after necropsy. Meningeal T1-WI hyperintensity prior to contrast injection is not caused by sarcoidosis or meningeal carcinomatosis, lymphomatosis or gliomatosis.     

Primary intracerebral lymphoma. Clinicopathological confrontation in a case study

A case of a primary intracerebral lymphoma, presenting with posterior uveitis, is reported together with its stepwise diagnostic approach. Various aspects of the diagnostic and therapeutic problems with these malignant tumours are discussed. The importance of indirect techniques for achievement of the diagnosis is emphasized.     

Primary sciatic nerve lymphoma: a case report and review of the literature

A patient with primary B cell non-Hodgkin's lymphoma of the sciatic nerve is described. He presented with neuropathic symptoms in the left leg, initially diagnosed as tarsal tunnel syndrome. Magnetic resonance imaging (MRI) identified the abnormality in the sciatic nerve. A fascicular biopsy of the sciatic nerve showed a diffuse large B cell non-Hodgkin's lymphoma. The patient was treated with chemotherapy and rituximab (anti-CD20 monoclonal antibody). Four months later he was in remission, and remains so 48 months from presentation. Primary lymphoma of single peripheral nerves may be a unique subtype of extranodal lymphoma, which usually follows an aggressive course and has a variable response to current therapeutic strategies. MRI is useful, alongside electrophysiological studies, in patients with atypical peripheral nerve symptoms.     

Primary central nervous system lymphoma in acquired immune deficiency syndrome: a clinical and pathological study

Twenty cases of primary lymphoma of the central nervous system associated with acquired immune deficiency syndrome were seen over a period of four years and were studied clinically and pathologically. Biopsy established the diagnosis in 11 cases, and autopsy confirmed it in 9. Multicentricity was demonstrated in all cases for which there was adequate autopsy material. Both large-cell immunoblastic and small noncleaved lymphomas were seen, and marker studies in 5 patients established that the lymphomas were of B-cell origin. Neurological symptoms and signs, cerebrospinal fluid characteristics, and radiographic appearance were reviewed. The clinical and radiographic picture is nonspecific and histological confirmation is essential for diagnosis. Although the tumor appears to be radiosensitive, prognosis is extremely poor, with an average survival of less than two months.     

Primary central nervous system lymphoma: a retrospective study

Eleven patients with primary central nervous system lymphoma (PCNSL) proven with biopsy between January 1991 and September 1995 were analysed according to the location of lesions, pathological studies, treatment and outcome. The ages of these patients ranged from 25 to 74 years with an average of 51.4 years. The male to female sex ratio was 6 to 5. The most frequent sites of lesions were frontal lobes. One patient had only leptomeningeal involvement. All intracranial PCNSL in this report arose from B cells; the leptomeningeal PCNSL case was the only one which arose from T cells. All patients received chemotherapy with or without radiotherapy, except two patients who did not receive any treatment. The results of treatment were generally poor. The survival time of patients who died ranged from 2.5 months to 45 months with a median of about 12.5 months. If a combined treatment is to be considered, the possibility of complications such as leucoencephalopathy should be taken in account.     

Primary CNS lymphoma in AIDS. A clinical and pathological case report

A case is reported of a primary central nervous system lymphoma in a heterosexual patient affected by acquired immunodeficiency syndrome. Because of the rarity and the aggressive diagnostic procedures, pre-mortem diagnosis is seldom established. This radiosensitive tumor, however, often contains monoclonal immunoglobulin productive cells and shows, at CT-scan, a multicentric arrangement. On this basis, a careful analysis of the cerebrospinal fluid and CT-scan may offer a possible mean of earlier diagnosis and improve the survival of these patients.     

Post-stroke pain case study: clinical characteristics, therapeutic options and long-term follow-up

Central post-stroke pain (CPSP) is a syndrome characterized by sensory disturbances and neuropathic pain. In 40%−60% of CPSP patients, the onset of central pain following a stroke occurs more than 1 month after the stroke, creating a source of diagnostic uncertainty or significant delay in treatment since healthcare providers familiar with CPSP may no longer be caring for the patient when the symptoms occur. In addition to chronic pain, the presence of somatosensory abnormalities is the most important diagnostic corollary of CPSP. Neuropathic or central pain has been estimated to occur in up to 8% of patients after a stroke, and about 18% of stroke patients with a somatosensory disturbance will develop CPSP. Although largely a matter of conjecture, it is generally agreed that damage to spinothalamic sensory pathways plays a significant role in the pathogenesis of CPSP. A comprehensive examination of the patient for sensory deficits is essential before treatment can be initiated. Functional disturbances such as depression, anxiety and sleep disturbances are significant comorbid conditions associated with CPSP; the physician should incorporate an assessment of these potential comorbidities into the examination. Treatment options for CPSP are limited; at present, amitriptyline is the drug of first choice. Other drugs including antidepressants, anticonvulsants, antiarrhythmics, opioids and N -methyl- d -aspartate antagonists may provide relief for some patients who do not respond to amitriptyline. Included in this review is a case study outlining the challenges of managing the patient with CPSP.     

Primary central nervous system lymphoma: a case of long survival

Despite combined treatment (surgery, radio—and chemotherapy) primary central nervous system lymphoma (PCNSL) has generally a poor prognosis. Long surviving cases (more than 5 years) are exceptional. We report a case of a patient completely symptom-free 79 months after craniotomy. Histological diagnosis was diffuse centroblastic-centrocytic malignant lymphoma. Immunohistological study of the lesion was performed. Repeated CT scans reveal no tumor recurrence and screenings for extraneural disease remain negative. The prognostic value of tumor histology is emphasized.

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Sommario Nonostante il trattamento combinato la prognosi del linfoma primario del SNC è infausta a breve termine. La sopravvivenza oltre 5 anni è eccezionale. Gli AA. riportano un caso di linfoma frontale di tipo centroblastico-centrocitico diffuso che, a distanza di 79 mesi dall'intervento, appare clinicamente guarito, con esami neuroradiologici negativi. Viene posto in rilievo il rapporto tra tipo istologico e prognosi.

     

A sporadic case of benign myoclonus epilepsy of adult onset: its clinical aspects and electroencephalographic, polygraphic study

A sporadic case of myoclonus epilepsy of adult onset with a benign course is presented. Its clinical implications were discussed symptomatologically and nosologically. EEG findings consisted of the slowing of basic activity, characteristic polyspike and wave discharges or other paroxysmal patterns and photomyoclonic response (PMR). The PMR was well evoked by flashes of 6-21 Hz and of yellow or red color. Clinical similarities of this case to those of hereditary myoclonus epilepsy without progressive dementia and cerebellar ataxia, and pathognomonic characteristics of electrophysiological findings indicated that the myoclonic epilepsy syndrome might share basic properties, which are state-dependent, but independent of underlying neuropathology or heredity. During sleep, paroxysmal discharges with spike(s) significantly decreased in slow-wave sleep and almost recovered in REM sleep. This finding suggests that the brain stem may be responsible for the epileptogeneity in this case.     

Clinically suspected fibrocartilaginous embolism: clinical characteristics,treatments, and outcomes

Objective: To study the frequency, demographics, clinical characteristics, and outcomes of patients with an antemortem diagnosis of fibrocartilaginous embolism (FCE), a rare cause of spinal cord and cerebral infarction because of the presumed embolization of nucleus pulposus material into the vascular circulation. Methods: We retrospectively reviewed the institutional experience of patients who received an antemortem diagnosis of FCE by their treating physician at the Mayo Clinic (Rochester, MN, USA) from 1997 to 2009. All patients underwent laboratory, radiological, and clinical exclusion of other possible and related diagnoses. Results: Of 164 patients with acute spinal cord infarction seen during the study timeframe, 9 (5.5%; 95% CI 2.5, 10.2%) met inclusion criteria for high likelihood of FCE (6 men, 3 women; median age 46 years old, range 21–64). All patients were severely affected (median modified Rankin Scale 4, median Barthel index 45; mean time to evaluation 57 days). One patient (1/9) experienced concomitant cerebral infarction. No patients had noticeable improvement from steroid treatment. Conclusion: The diagnosis of FCE in life is common at this referral center, accounting for 5.5% of all cases of acute spinal cord infarction seen. Although FCE is a postmortem diagnosis, we propose clinical criteria for FCE in life to better characterize the relatively high number of patients with unexplained ischaemic myelopathy.     

Status epilepticus in pregnancy: Etiology,management, and clinical outcomes

BackgroundStatus epilepticus (SE) in pregnancy carries significant risk to both mother and fetus. There is limited literature available on SE occurring in pregnancy world-over, with majority being from obstetric centers.MethodsAll women who developed SE related to pregnancy (gestation, labor, or puerperium) between January 2000 and December 2016 were included in the study. Data were collected from our SE registry, maintained, and archived in the institute. The variables influencing the maternal and fetal outcome were compared using Student's t-test for continuous variables and Fisher's exact test for discrete variables.ResultsDuring the 16-year study period, a total of 348 SE events were recorded in 294 patients. Among these, there were 138 women, of which 17 had SE related to pregnancy. The etiology of SE was remote symptomatic in two and acute symptomatic in 15 patients. The various causes detected after initial evaluation for acute symptomatic SE were eclampsia (n = 4), posterior reversible encephalopathy syndrome due to various causes other than eclampsia (n = 6), cortical venous thrombosis (n = 3), subarachnoid hemorrhage (n = 1), and NMDA receptor antibody-mediated encephalitis (n = 1).13 of 17 women with SE (76%) had good outcome. Majority of the fetuses had good outcomes, i.e., Category 1 (n = 9, 57%). Duration of intensive care unit stay (p = 0.029) and Status Epilepticus Severity Score (p = 0.0324) at admission, were found to be significantly associated with poor outcomes.ConclusionIn any patient presenting with SE occurring in pregnancy, though eclampsia is presumed to be the most common overall cause; it is relevant to consider other etiologies such as posterior reversible encephalopathy syndrome, cortical venous thrombosis, and autoimmune encephalitis especially in cases presenting with refractory SE. Posterior reversible encephalopathy may occur in pregnancy due to diverse etiologies other than eclampsia.     

Primary CNS lymphoma: clinical presentation, pathological classification, molecular pathogenesis and treatment

Primary CNS lymphomas (PCNSL) represent malignant non-Hodgkin's B cell lymphomas, which are confined to the central nervous system. They show a dramatic increase in frequency in the immunocompromised as well as in the immunocompetent population. Recent studies have identified germinal center B cells as the cellular origin of PCNSL; however, the details of their molecular pathogenesis still remain to be elucidated. Treatment recommendations are not clearly established. Radiotherapy (RT) is efficient in terms of tumor response, but not curative. Median survival after RT alone is about 1 year. According to the results of uncontrolled studies the combination of RT and chemotherapy based on high-dose methotrexate (HD-MTX) is most efficient in terms of survival rates. However, long-term neurotoxicity overshadows treatment efficacy, especially in patients over 60 years of age. The authors favor the systematic evaluation of chemotherapy alone with protocols including HD MTX, because unicenter results are promising in terms of both survival as well as quality of life in long term survivors.     

Comparison of characteristics,management and outcomes in hospital-onset and community-onset stroke: a multi-centre registry-based cohort study of acute stroke

Neurological Sciences - Hospital-onset stroke (HOS) is associated with poorer outcomes than community-onset stroke (COS). Previous studies have variably documented patient characteristics and...     

Primary osteoma of the orbit with atypical facial pain: case report and literature review

Osteoma is a benign, slowly growing tumor that mainly occurs in the bones and cavities of the middle third of the face, representing the most frequent benign tumor of the paranasal sinuses. It rarely originates primarily from the orbit. Most of these lesions develop in the fourth to fifth decades of life, and are more commonly encountered in males. In the English literature, there are so far three reported cases of primary osteoma of the orbit that originated from the sphenoid bone. Here we present another case of a primary osteoma of the orbit presenting with atypical facial pain and discuss the relevant literature.     

Intradiploic meningioma of the orbit: a case report

We report a case of a 12-year-old male child who presented with a gradual onset exopthalmos involving the left eye. The plain radiographs of the skull showed hyperostosis of the left orbital roof. The computerized tomography (CT) scan revealed an intradiploic orbital roof tumor with expansion of both the tables of the orbital roof. The tumor was completely excised by an extradural route using a basal frontal craniotomy. The histopathological diagnosis of the tumor was a psammomatous meningioma. A split calvarial frontal bone graft was used to reconstruct the orbital roof. The pathogenesis, radiological features and surgical technique involved in the management of intradiploic orbital roof meningioma are discussed and the relevant literature is reviewed.     

Melanotic paraganglioma of the orbit: a case report

Summary A paraganglioma of the orbit in a 21-year-old woman is presented, containing oculo-cutaneous melanin in many tumor cells, occasionally adjacent to neurosecretory granules, and in macrophages. This tumor expands the list of neuroectodermal tumors with potential melaninization.     

Trochlear pain: clinical characteristics and treatment outcomes

Trochlear pain is frequently overlooked as published data regarding the clinical characteristic and current treatment are limited. The aim of this study is to evaluate this information from our experiences with trochlear pain. Medical records of 43 patients with trochlear pain from HRH Princess Maha Chakri Sirindhorn Medical Center between November 2010 and April 2017 were reviewed. Most patients were female (88%), with a median age of 51 years. Common characteristic symptoms of trochlear pain were acute, episodic, dull or pressure-like, periorbital pain, often radiating to the forehead, and aggravated by eye movements, especially reading. The causes of trochlear pain were idiopathic or primary trochlear headache (n = 33, 77%) and trochleitis (n = 10, 23%). Treatments included oral NSAIDs or dexamethasone injection into the trochlear region. At a median follow-up of 11 months (range 0–64), 67% of the patients reported complete remission using oral medication. Local steroid injection is useful in non-responding patients to oral therapy with an overall remission of 86%. Successful treatment outcome was achieved in most patients.