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We report a case of a 70 year old lady who presented to the accident and emergency department with a three day history of left sided otalgia, drowsiness and confusion. On admission her temperature was 39 degrees C, glascow coma scale was 14/15 and otoscopy revealed a left sided otitis media. There was no sign of CSF otorrhoea or mastoiditis. Neuro-otological examination was normal. High resolution CT scan of temporal bones and brain showed evidence of pneumocephalus near the left cerebello-pontine angle and posterior to the left petrous bone. No focal parenchymal pathology, intra-cranial bleed or defect in the tegmen was noted. The patient responded very well to conservative management in the form of high dose intravenous antibiotics. We would like to emphasise that this complication of otitis media should be borne in mind when an elderly patient presents with otalgia and confusion.
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PMID:Otogenic pneumocephalus: case report and review of the literature. 1763 67

Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma arising from and confined to the neuraxis. It represents about 3-4% of all primitive brain neoplasms and is mainly located in deep supratentorial regions. A ventricular involvement is quite frequent, but in the vast majority of cases it is secondary to an extension from a subependymal location. Amongst the primary ventricular forms occasionally reported, a choroid plexus origin is extremely rare; to date, only three cases have been described in the English literature. We report the case of a 71-year-old left-handed woman admitted to our department after a progressive 2-week history of confusion, motor speech disturbances and left hemiparesis. A brain MRI scan displayed an intraventricular lesion located in the right atrium, about 2.5cm in its major axis, homogeneously enhancing after gadolinium administration, surrounded by edema in the homolateral deep hemispheric region; the main diagnostic hypothesis was meningioma. She underwent a parieto-occipital craniotomy and a navigation-assisted posterior parietal transcortical approach, achieving complete removal of the intraventricular lesion originating from the choroid plexus. The histological diagnosis was of high-grade diffuse large B-cell lymphoma. Both an MRI scan of the entire craniospinal axis and a thoracic and abdominal CT scan showed no other neoplastic lesions; a bone marrow biopsy was normal; a final diagnosis of primary central nervous system lymphoma was made. CSF cytological analysis did not show any signs of lymphoma dissemination. Adjuvant chemotherapy with methotrexate was administered, and at the last follow-up (26 months) our patient is clinically and radiologically disease-free. Primary choroid plexus lymphoma is a very rare tumor that should be included in the differential diagnosis of intraventricular neoplasms. It usually occurs in the lateral ventricles (with a predilection for the atrium) of adult people (>50 years of age), apparently with a male prevalence. Generally, the radiological picture mimics that of a meningioma, despite the fact that meningiomas usually reach a greater volume before clinical onset. Surgery is almost always necessary to obtain an adequate tissue sample and frequently leads to a radical removal of the lesion. Low-grade marginal zone B-cell, T-cell and high-grade diffuse large B-cell (present case) forms have been described. With a proper adjuvant therapy a relatively good outcome is observed.
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PMID:Primary high-grade B-cell lymphoma of the choroid plexus. 1792 35

The findings of a neurological evaluation in 85 patients with confirmed, acute, dengue virus infection are described. Signs of central nervous system involvement were present in 18 patients (21.2%). The most frequent neurological symptom was mental confusion. The frequency of neurological involvement did not differ between patients with primary and secondary dengue infection, and the prevalence of central nervous system involvement in dengue fever and dengue hemorrhagic fever also did not differ significantly. The presence of CNS involvement did not influence the prognosis of dengue infection. Dengue viral CSF RNA was found in 7 of 13 patients submitted to a spinal tap, the CSF viral load being less than 1000 copies/ml. PCR was negative in serum samples obtained from three patients on the same day as the CSF samples, suggesting that the dengue virus actively enters the CNS and that the presence of the virus in the CNS does not result from passive crossing of the blood-brain barrier.
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PMID:Involvement of the central nervous system in patients with dengue virus infection. 1795 98

A 37-year-old woman developed memory disturbance and seizures preceded by headache and high fever. Physical examination revealed persistent high fever, confusion, neck stiffness, anterograde and retrograde amnesia and disorientation. CSF showed pleocytosis and normal glucose level, and negative herpes simplex virus DNA on PCR. The fluid attenuated inversion recovery (FLAIR) MRI of the brain demonstrated nearly symmetric high signal intensity areas in the bilateral mesial temporal lobes. The tentative clinical diagnosis was non-herpetic acute limbic encephalitis (NHALE), and administration of methylprednisolone improved her conditions. Laboratory tests revealed mild hypothyroidism with high titers of serum antibodies against TPO and thyroglobulin, consistent with Hashimoto's disease. In addition, antibodies against amino terminal of alpha-enolase in the serum and those against glutamate receptor (GluR) epsilon2 in the serum and CSF were positive. A final diagnosis of Hashimoto's encephalopathy associated with GluRepsilon2 antibody was made. The present case suggests that NHALE-like clinical manifestation can be produced by autoimmune-mediated encephalopathies.
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PMID:[Non-herpetic acute limbic encephalitis-like manifestation in a case of Hashimoto's encephalopathy with positive autoantibodies against ionotropic glutamate receptor epsilon2]. 1809 94

We present a case with seizure, confusion, hypesthesia and paraplegia after intrathecal injection of fluorescein. A 41-year-old man was admitted to our institution for the management of the CSF leakage. Intrathecal injection of fluorescein was performed and he complained of severe pain and numbness in the lower extremities at the end of the injection. Four hours later, he exhibited confusion, paraparesis and two episodes of generalized seizures. Two days later, he showed paraplegia and all sensory modalities below the T12 level were absent. Spine magnetic resonance imaging revealed myelopathic change in the lower thoracic spinal cord. There was no improvement of weakness and sensory deficits in lower extremity even 14 days after fluorescein injection. We speculated that thoracic myelopathy was associated with the intrathecal injection of fluorescein. In spite of its rarity, the complication after intrathecal injection of fluorescein could be serious. Thus, obtaining an informed consent with discussion with patient before the procedure is mandatory.
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PMID:A case of myelopathy after intrathecal injection of fluorescein. 1909 98

Symptomatic nervous system leukemic infiltration is rarely observed in CLL. Various clinical manifestations including headache, confusion, cranial nerve palsies, focal central deficits and peripheral neuropathies have been seldom reported, occurring in less than 1% of patients. We report herein 2 CLL patients with unusual clinical presentations of nervous system invasion. They presented multiple progressive peripheral deficits due to meningoradiculitis. In both, CSF immunophenotyping analysis identified a majority of T cells (>90%), and less than 10% of B-CLL cells expressing CD5, CD19 and CD20. Our analyses revealed the transformation of CLL into an aggressive B-cell lymphoma in one case (Richter's syndrome). A post mortem study showed massive infiltration of cranial nerves and spinal roots by large B lymphomatous cells. In the other case, CNS oriented chemotherapy led to remission and total neurological recovery. In practice, the etiological diagnosis of neurological deficits in CLL patients is difficult. CSF analysis may be useful, requiring viral PCR, repeated cytological studies and immunophenotyping analysis. Although rare, leptomeningeal leukemic localization has to be discussed, even in the absence of overt Richter syndrome, and may require an early therapeutic test.
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PMID:Multifocal deficits due to leukemic meningoradiculitis in chronic lymphocytic leukemia. 1910 Sep 98

Leptomeningeal (LM) dissemination arises rarely in patients with low-grade gliomas. Few cases of LM dissemination of spinal cord low-grade gliomas have been reported in children. Here, we report a case of an intramedullary spinal cord low-grade glioma occurring in an adult presenting by LM dissemination. A 57-year-old man presented with a history of headaches and visual acuity deterioration. Neurological examination revealed mental confusion, decreasing left limb strength and left positive Babinski sign. Lumbar puncture showed low CSF glucose, high CSF protein and no tumor cells. Spinal MRI revealed an intramedullary spinal lesion with a diffuse leptomeningeal enhancement. A spinal meningeal biopsy showed arachnoiditis with isolated atypical cells which were cytokeratin immunopositive. A differential diagnosis was proposed between arachnoiditis close to an infectious process or close to meningeal carcinomatosis. An intramedullary biopsy was programmed but the patient died 1 month after biopsy. The post-mortem examination showed thickened leptomeninges and an ill-circumscribed intramedullary grey lesion. Histological examination revealed a low-grade glial neoplasm composed of monomorphous spindle "piloid" cells with diffuse dissemination to leptomeninges. Glial fibrillary acidic protein immunostaining showed scattered immunoreactive cells. Clinical and pathological features of this case are unusual. Pathogenesis and prognosis of low-grade gliomas with LM dissemination are poorly understood. This case, like cases occurring in children, cannot easily be classified in the present WHO system of classification of CNS tumors. Spinal low-grade gliomas with LM dissemination may represent a possible distinctive clinico-pathological entity.
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PMID:Leptomeningeal dissemination of low-grade intramedullary gliomas: about one case and review. 1912 71

Encephalitis caused by Toxoplasma gondii is the most common cause of central nervous system damage in patients with acquired immunodeficiency syndrome (AIDS). Toxoplasma may infect any of the brain cells, thus leading to non-specific neurotoxoplasmosis clinical manifestations including focused or non-focused signs and symptoms of central nervous system malfunction. Clinical development ranges from insidious display during weeks to experiencing acute general confusion or ultimately fatal onset. Cerebral toxoplasmosis occurs in advanced stages of immunodeficiency, and the absence of anti-toxoplasmosis antibodies by the immunofluorescence method does not allow us to rule out its diagnosis. As specific therapy begins, diagnosis confirmation is sought through clinical and radiological response. There are few accurate diagnosis methods to confirm such cases. We present a method for T. gondii DNA detection by real time PCR-Multiplex. Fifty-one patients were evaluated; 16 patients had AIDS and a presumptive diagnosis for toxoplasmosis, 23 patients were HIV-positive with further morbidities except neurotoxoplasmosis, and 12 subjects were HIV-negative control patients. Real time PCR-Multiplex was applied to these patients' cephalorachidian liquid with a specific T. gondii genome sequence from the 529bp fragment. This test is usually carried out within four hours. Test sensitivity, specificity, positive predictive value, and negative predictive value were calculated according to applicable tables. Toxoplasma gondii assay by real time Multiplex of cephalorachidian fluid was positive for 11 out of 16 patients with AIDS and a presumptive diagnosis for cerebral toxoplasmosis, while none of the 35 control patients displayed such a result. Therefore, this method allowed us to achieve 68.8% sensitivity, 100% specificity, 100% positive predictive value, and 87.8% negative predictive value. Real time PCR on CSF allowed high specificity and good sensitivity among patients who presumably had cerebral toxoplasmosis. Since this is a low invasive method, it could be included in the diagnosis algorithm of patients with AIDS and central nervous system damage.
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PMID:Neurotoxoplasmosis diagnosis for HIV-1 patients by real-time PCR of cerebrospinal fluid. 1957 25

We report the case of a patient who was admitted to the hospital because of language and mental confusion. His initial lumbar puncture revealed 193 leukocytes per mm3 mostly lymphocytes (95%), no red blood cells, high protein content (1.20 g/L) and normal glucose level. The antibiotic therapy by amoxicilline and aciclovir during 6 days led to complete clinical recovery in a week. A CT scan of the cerebrum showed no abnormalities, nor did chest radiography. Twelve days after discharge, the patient was rehospitalized because of a meningitis syndrome. On lumbar puncture, the CSF analysis revealed 280 leukocytes/mm3, 56% lymphocytes, 10% monocytes and 34% polymorphonuclear cells. CSF chemistry showed a protein level of 3.54 g/L, and a glucose level depressed at 0.9 mmol/L. Because of the clinical symptoms and CSF abnormalities, the patient received aciclovir, amoxicilline vancomycine, isoniazide, rifampicine, pyrazinamide and ethambutol. Screening for infections gave negative results until the 37th day, when the diagnosis of tuberculous meningitis was confirmed by the isolation of Mycobacterium tuberculosis in the repetitive CSF. Antituberculous therapy was expanded. According the Reiber diagrams, intrathecal IgG synthesis was negative at day 25, day 37, month 4, month 9, month 17. Intrathecal IgM synthesis was elevated at day 12 and day 25 and intrathecal IgA synthesis at day 25. Improvement of the patient's conditions by tuberculosis treatment was obtained in 17 months. Cerebrospinal fluid analysis has been the basis for the diagnosis and follow-up of tuberculous meningitidis.
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PMID:[Contribution of CSF analysis to diagnosis and follow-up of tuberculous meningitis]. 2014 81

Cranial pachymeningitis is a poorly understood syndrome, defined by leptomeningeal thickening and typical gadolinium enhanced MRI. The heterogeneous clinical and aetiological features of five patients with both focal and diffuse pachymeningitis are presented. The initial symptoms included headache (n=3), sensory Jackson seizures (n=1), hemiparesis (n=1), episodes of short lasting hemiataxia (n=1), hemihypaesthesia (n=1), aphasia (n=1) and confusion (n=2). MRI scans revealed focal (n=3) or diffuse (n=2) leptomeningeal gadolinium enhancement and cortical swelling (n=4). In addition, one case presented with a subarachnoid and a second with an intracerebral haemorraghe. CSF findings were variable and showed clear lymphomonocytic pleocytosis in 3/5 cases. Infectious diseases were extensively excluded in all cases. Leptomeningeal biopsies of two cases revealed perivascular inflammation, indicating central nervous system vasculitis. In the cases presented, pachymeningitis was caused by primary central nervous system vasculitis (n=2) and rheumatoid arthritis (n=2). In one case, the cause remained unclear.
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PMID:Cranial pachymeningitis: a rare neurological syndrome with heterogeneous aetiology. 2018 66


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