Spinal cord compression resulting from Burkitt's lymphoma in children

We report seven cases of spinal cord compression resulting from Burkitt lymphoma in boys aged 15 years and below. This became manifest clinically as acute or rapidly progressive spinal cord compression. All the patients showed total paraplegia with a sensory loss at thoracic level and sphincter disturbances. Four patients were operated on, the operation permitting exeresis of an epidural tumor. In three cases surgery was followed by chemotherapy. One patient was treated exclusively with chemotherapy. Owing to the effectiveness of chemotherapy, surgery should be considered only in cases of rapid deterioration or for diagnostic purposes.     

Primary malignant lymphoma of the brain: An immunohistochemical study of eight cases using a panel of monoclonal and heterologous antibodies

Summary Frozen sections of eight primary malignant lymphomas of the brain were examined by the immunohistochemical methods using a panel of monoclonal and heterologous antibodies to B lymphocyte (immunoglobulins, BA-1, Leu-12 and HLA-DR), T lymphocyte (OKT-11 and Leu-1) and monocyte (OKM-1) surface markers. Paraffin sections were also used in the examination of cytoplasmic immunoglobulins. Surface and/or cytoplasmic immunoglobulins (Ig) were observed in seven cases,four of which were shown to be distinctly monoclonal and the other three less so. The remaining 1 case showed no distinct staining for Ig. BA-1, Leu-12 and HLA-DR stainings were positive in four, four and five cases, respectively. The marker phenotypes of (BA-1, Leu-12, HLA-DR) were shown to be (+,+,+) in one lymphoma, (+,-,-) in three, (-,+,+,)in three, and (-,-,+) in one. Thus, it was demonstrated that the present lymphoma cases showed a marked immunological heterogeneity, and it was shown that all of them including the Ig-negative case revealed one or more of these three additional B cell markers, indicating B cell lineage of these cases. Examination of T cell and monocyte markers revealed positive staining in normal or reactive lymphoid cells distributed around blood vessels or sporadically in tumor tissues, but not in lymphoma cells. Epstein-Barr virus (EBV)-associated nuclear antigen was not demonstrated in the seven cases examined, making it unlikely that these lymphomas were related with EBV infection.     

Bilateral symmetrical enhancing brainstem lesions: an unusual presentation of primary CNS lymphoma

We report a patient with a progressive brainstem syndrome, who on magnetic resonance imaging had large bilateral, symmetrical, contrast-enhancing, infratentorial space-occupying lesions. Biopsy of one of the lesions revealed this unusual appearance to be due to a primary central nervous system (CNS) lymphoma of B-cell type. Symmetry of lesions may be a clue to the diagnosis, perhaps reflecting the mechanism by which CNS lymphomas spread.     

Pneumocephalus: an unusual cause

A 60 year old male had an open thoracotomy for bronchogenic carcinoma. On the twelfth hospital day he became obtunded and complained of headache. Radiographs revealed intracranial air. It was thought that the pneumocephalus in this patient was most likely secondary to a tension pneumothorax continuously forcing air through a dural tear sustained at the time of initial surgery. The causes of pneumocephalus are reviewed and no similar case report has been found in the literature.     

Giant prolactinomas in adolescence: an uncommon cause of blindness

Introduction Prolactinomas in childhood and adolescence are rare. However, in male patients in particular they may become extremely large and invasive, resulting in visual impairment without necessarily producing endocrine symptoms. Case reports We report on two adolescent males who presented with deteriorating vision over a long period of time and who had optic atrophy on examination. Magnetic resonance imaging identified tumours with extensive anterior skull base invasion and suprasellar extension. Prolactin levels in both patients were markedly elevated, and a diagnosis of prolactinoma was made. Bromocriptine treatment was started resulting in lowered prolactin levels, improved vision and tumour shrinkage on imaging. Conclusion A male child or adolescent presenting with diminished vision and found to have suprasellar or anterior skull base tumour should have their prolactin levels checked to rule out a prolactinoma, which can be successfully managed with medical therapy.     

Ascaris lumbricoides: an unusual cause of shunt infection

Two cases of delayed shunt infection attributable to the abdominal complications of Ascaris lumbricoides infestation are reported. The first child presented with a peritoneal shunt catheter protruding through the anus and the second patient was found to have two live worms around the cephalic shunt tubing. Both had enteric shunt infections which responded well to therapy. The epidemiology and treatment of ascariasis are briefly discussed.     

Pallister-Killian syndrome: an unusual cause of epileptic spasms

Pallister-Killian syndrome (PKS) is a rare, sporadic, genetic disorder characterized by dysmorphic features, learning disability, and epilepsy. It is caused by a mosaic supernumerary isochromosome 12p (i[12p]). The i(12p) is rarely found in peripheral blood but it is present in skin fibroblasts. Recognition is essential for cytogenetic diagnosis. We describe a male aged 2 years 6 months and a female aged 11 years with PKS and epileptic spasms (ES). This type of seizure is not unusual in patients with brain malformations and with severe developmental delay, but it is sometimes difficult to recognize without video-electroencephalogram studies and could be mistaken for other types of seizure or behavioural manifestations. In these two patients with PKS, spasms had late onset, persisted beyond infancy, and were drug resistant. Clinicians should be aware of this possibility in PKS, which appears to be a rare cause of ES.     

Paradoxical embolism: an unusual cause of cerebral ischemia

Paradoxical embolism (PE) is an uncommonly diagnosed cause of unexplained ischemic cerebral event. Its diagnosis is based on established criteria and the failure to identify an arterial or cardiac embolic source. The association between patent foramen ovale (PFO) and cryptogenic stroke has repeatedly been demonstrated in clinical studies. Transesophageal contrast echocardiography is the best diagnostic test. Surgical or endovascular PFO closure is recommended for the long-term prevention of PE.     

Total recuperation in a case of sudden total paraplegia due to an aneurysmal bone cyst of the thoracic spine

A 3-year-old girl developed sudden complete paraplegia because of an aneurysmal bone cyst of the fourth thoracic vertebra. We performed a two-step surgical procedure resulting in complete neurological recuperation and no cyst recurrence within 2.5-year follow-up. Although the literature shows the prognosis of such a condition to be poor, this case reveals the positive effect of rapid surgical decompression and of postponing assessment by magnetic resonance imaging until before the second surgical stage. Furthermore, this case is an example of cure with neither interbody fusion nor postoperative radiotherapy, despite the total involvement of the vertebra by the lesion.     

Acute paraplegia due to schistosomiasis: an uncommon cause in developed countries

Journal of NeuroVirology - We present a case of a young African migrant from Guinea-Conakry presented to a French emergency department with burning pain in both feet for 2 days. The...     

Symptomatic palatal myoclonus: an unusual cause of respiratory difficulty

A 67-year-old man presented with dysphagia and difficulty breathing. Physical examination revealed palatal myoclonus. In this patient, the respiratory difficulty was caused by the fragmentation of breathing. Electromyographic examination of the cricothyroid muscle demonstrated rhythmic myoclonic jerks. Magnetic Resonance Imaging (MRI) yielded a pontine midline and right sided tegmental infarct. The patient responded to sodium valproate.     

Neurenteric cyst: an unusual cause of third nerve palsy

Introduction  

Neurenteric cysts are a rare cause of central nervous system compression. Pediatric intracranial cysts are even less common.     

Organic psychoses in childhood: report of an unusual case

A psychosis in a 5 years old girl is reported. By the 2nd month of her illness there was clear mental regression, with disorders of contact, motricity, speech, instinctive behavior and pragmatism. Neurologic and electroencephalographic abnormalities, together with the psychiatric symptoms, lead to the diagnostic of organic psychosis. High doses of neuroleptics were used and there was progressive remission of the symptoms, beginning at the 3rd month of disease. The etiologic diagnosis (being more probable a demyelinizing encephalitis) and the therapeutic effect of the neuroleptics are discussed.