Lymphocytes B population profile in a case of multiple sclerosis presenting with sudden sensorineural hearing loss caused by a demyelinating pontine lesion.

Sudden sensorineural hearing loss (SSHL) is a rare manifestation of multiple sclerosis, typically appearing in the early stages of the disease, especially in female subjects. SSHL is produced by the involvement of auditory tract, vestibulocochlear nerve and possibly cochlear structures and rarely due to a single lesion. The authors report the case of a young woman in which the onset of multiple sclerosis presented with SSHL caused by a pontine lesion. Oligoclonal bands in the cerebrospinal fluid (CSF) were absent at the disease onset and appeared during disease progression. Immunophenotyping of cells showed low cellularity of CD19+ cells in the CSF and expression of CD38+ on the majority of CD19+, CD20+ B cells in the peripheral blood, suggesting that many of them were mature B lymphocytes.

Progressive multiple cranial nerve palsies as the presenting symptom of meningeal carcinomatosis from occult colon adenocarcinoma.

We herein describe a rare case of meningeal carcinomatosis in a 77-year-old woman who had bilateral deafness as the initial symptom of a previously undetected colon cancer malignancy. Meningeal carcinomatosis should be considered in the differential diagnosis in cases of abrupt-onset sensorineural deafness. Both MRI scans and cerebrospinal fluid evaluation are necessary diagnostic tools, and should be used in conjunction as each of these procedures could have false-negative results. This should apply even when there is no known primary malignancy.

Serial cerebrospinal fluid neurofilament concentrations in bacterial meningitis.

Neurofilament (NF) is one of the major cytoskeleton proteins of neurons. We investigated the concentrations of the heavy subunit of NF (NF-H) in cerebrospinal fluid (CSF) as biomarkers of neuronal injury in bacterial meningitis. Concentrations of NF-H in CSF of 26 children with bacterial meningitis and in 16 control subjects were measured by ELISA. The CSF NF-H levels were elevated in 22 of the 26 children (85%) with bacterial meningitis. The peak CSF NF-H level occurred at a median period of 10.5 days after onset of illness (range, 1 to 35 days). The peak CSF NF-H levels of the patients with neurological sequelae (n=4) were significantly higher than those without sequelae (n=22) (7.06 vs. 2.46 ng/mL as median, p=0.048). There was no significant difference in CSF NF-H levels between patients with and without severe neurological sequelae up to day 14 of illness, but the CSF NF-H levels in patients with sequelae were significantly higher than in those without sequelae after day 14 of illness (2.04 vs. 1.19 ng/mL as median, p=0.024). We suggest that neuronal injury occurs in bacterial meningitis regardless of the presence or absence of neurological sequelae.

The spectrum of Susac's syndrome.

We report a series of four patients with Susac's syndrome, which is characterized by the triad of visual loss due to branch retinal artery occlusions, sensorineural hearing loss due to cochlear involvement, and encephalopathy due to cerebral microangiopathy. However, as we describe in this series, the clinical triad may not be apparent for years, resulting in delays in diagnosis. We also report the variable cerebrospinal fluid and brain magnetic resonance imaging findings, and treatment using a combination of steroids and intravenous immunoglobulin, followed by mycophenolate mofetil.

[Superficial siderosis of the CNS. Case report and literature review]. / Superfizielle Siderose des ZNS. Fallbericht und Literaturübersicht.

Superficial siderosis of the central nervous system is caused by destructive deposition of haemosiderin in the leptomeninges and subpial layers of the brain and spinal cord. This deposition is the result of continuous or recurrent, often clinically silent, haemorrhage in the subarachnoid space, eventually without an evident bleeding source. Cerebellar ataxia, progressive bilateral sensorineural hearing loss, pyramidal tract signs, and dementia are the major clinical findings. The diagnosis is supported in vivo by the characteristic symptom constellation,xanthochromic cerebrospinal fluid,and typical MRI findings which show on the surface of the brainstem, cerebellum, cortex, and spinal cord. Early recognition of this rare entity may be of relevance for the further course and prognosis.

Serum and cerebrospinal fluid pathology in patients with sudden sensorineural hearing loss.

The serum and cerebrospinal fluid (CSF) pathology of patients with sudden sensorineural hearing loss (SHL), both seropositive and seronegative to Borrelia burgdorferi (Bb), was prospectively studied. Nineteen consecutive patients were included and trends between the degree of hearing recovery and serum/CSF pathology and given therapy were examined. The pilot study showed a high prevalence (68%) of pathology in serum and CSF in patients with SHL. In 54% of the patients, elevated levels of CSF proteins and/or pathological CSF cell counts were present without positive antibodies to Bb. Positive levels of antibodies against Bb or pathological proteins in CSF were associated with better hearing recovery (means of 47.2 and 51.7%, respectively). The audiometric configuration "high frequency sloping" hearing impairment was associated with the lowest degree of hearing recovery. Patients with SHL and positive serology to Bb who received antibiotic treatment (oral tetracycline), with or without steroids, had the best hearing recovery in this study (61.7 and 48.4%, respectively). In conclusion, we found a high prevalence of serum and CSF pathology in a consecutive group of patients with SHL. Early appropriate antibiotic treatment may prevent the development of major late complications of Lyme disease/borreliosis. We also find it justified to perform more general serological analyses, including CSF analysis, in patients with SHL. A more liberal approach to testing and development of test protocols for SHL patients will increase our knowledge in this field.

Could antioxidant therapy reduce the incidence of deafness following bacterial meningitis?

Sensorineural hearing loss following acute bacterial meningitis could be caused by hydroxyl radicals generated by the inflammatory response. Obstruction of cerebrospinal fluid circulation through the tela choroidae of the choroid plexuses, with subsequent rupture of the tela choroidae, would expose the auditory nerve to selective radical damage. Acute administration of lipophilic antioxidants might provide the auditory nerve with increased protection.