Dissociation of caloric and head impulse tests: a marker of Meniere's disease.

A retrospective analysis of the horizontal video head impulse test (vHIT) results and caloric testing results was undertaken on 644 patients who attended a neuro-otology outpatient facility. Presenting symptoms included spontaneous vertigo, positional vertigo, imbalance or chronic subjective dizziness. For 570 patients, the results of vHIT and caloric testing were concordant. Both tests were normal in 500 subjects with an average vHIT gain = 0.92 ± 0.09 (L); 0.98 ± 0.10 (R) and canal paresis (CP) = 7.88 ± 6.12; (range 0-28%). 54 had concordant asymmetries, average ipsilesional vHIT gain = 0.56 ± 0.15, average contralesional vHIT gain = 0.88 ± 0.12. CP = 68.02 ± 24.38 (range 31-100%). 16 subjects had bilateral vestibular hypofunction with average vHIT gains of 0.42 ± 0.20 (L); 0.41 ± 0.19 (R), peak slow phase velocity (SPV) on warm caloric testing = 2.68 ± 2.08, range 0-6°/s (L) and 3.75 ± 3.43 range, 0-10°/s (R). 36 patients showed a dissociation of results between the two tests. In these subjects, the vHIT gain was normal (0.93 ± 0.06 left and 0.98 ± 0.07 right) and the caloric test showed a CP > 30% (48 ± 13.8%). Their final diagnoses included clinically definite Meniere's disease (MD) (n = 27), vestibular schwannoma (VS) (n = 2) vestibular migraine (VM) (n = 1), vestibular neuritis (VN) (n = 5) and unknown (n = 1). No patient with abnormal HSCC gain on vHIT had a normal caloric result. The caloric test complements the vHIT in the assessment of vestibular disorders and is most useful in suspected endolymphatic hydrops. Asymmetric caloric function in the presence of normal horizontal head impulse tests is most commonly associated with Meniere's disease and may function as a diagnostic marker.

Mal de Debarquement Syndrome: A Retrospective Online Questionnaire on the Influences of Gonadal Hormones in Relation to Onset and Symptom Fluctuation.

INTRODUCTION: Mal de Debarquement Syndrome (MdDS) is a condition characterized by a persistent perception of self-motion, in most cases triggered from exposure to passive motion (e.g., boat travel, a car ride, flights). Patients whose onset was triggered in this way are categorized as Motion-Triggered (MT) subtype or onset group. However, the same syndrome can occur spontaneously or after non-motion events, such as childbirth, high stress, surgery, etc. Patients who were triggered in this way are categorized as being of the Spontaneous/Other (SO) subtype or onset group. The underlying pathophysiology of MdDS is unknown and there has been some speculation that the two onset groups are separate entities. However, despite the differences in onset between the subtypes, symptoms are parallel and a significant female predominance has been shown. To date, the role of gonadal hormones in MdDS pathophysiology has not been investigated. This study aimed to evaluate the hormonal profile of MdDS patients, the presence of hormonal conditions, the influence of hormones on symptomatology and to assess possible hormonal differences between onset groups. In addition, the prevalence of migraine and motion sickness and their relation to MdDS were assessed. METHOD: Retrospective online surveys were performed in 370 MdDS patients from both onset groups. Data were analyzed using Fisher's exact test or Fisher-Freeman-Hanlon exact test. When possible, data were compared with normative statistical data from the wider literature. RESULTS: From the data collected, it was evident that naturally cycling female respondents from the MT group were significantly more likely to report an aggravation of MdDS symptoms during menses and mid-cycle (p < 0.001). A few preliminary differences between the onset groups were highlighted such as in regular menstrual cycling (p = 0.028), reporting menses during onset (p < 0.016), and migraine susceptibility after onset (p = 0.044). CONCLUSION: These results demonstrate a potential relation between hormone fluctuations and symptom aggravation in the MT group. This study is an important first step to suggest a hormonal involvement in the pathophysiology of MdDS and provides a base for further hormonal investigation. Future prospective studies should expand upon these results and explore the implications for treatment.

Paraneoplastic polyarteritis nodosa with cerebral masses: case report and literature review.

Polyarteritis nodosa (PAN) as a paraneoplastic vasculitis is rarely described, especially in association with squamous cell carcinoma (SCC). Furthermore, only 5% of all PAN patients have central nervous system (CNS) involvement, almost exclusively in the form of cerebral infarction or intracerebral haemorrhage. We report the first case of PAN with multiple immunosuppressant-responsive, cerebral vasculitic lesions in association with metastatic SCC.

Severe depression masquerading as Creutzfeldt-Jakob disease.

We report a case of melancholic depression with catatonic features presenting as a rapidly progressive organic brain syndrome, initially thought to be probable Creutzfeldt-Jakob disease. The case highlights the fundamental importance of thorough exclusion of treatable pathology masquerading as an irreversible syndrome.

Evidence of T-cell mediated neuronal injury in stiff-person syndrome with anti-amphiphysin antibodies.

Paraneoplastic stiff-person syndrome (SPS) has been associated with antibodies against amphiphysin. Current evidence supports a pathogenic role for anti-amphiphysin antibodies. A 74-year-old female was diagnosed with amphiphysin-associated paraneoplastic stiff-person syndrome and associated encephalomyelitis. She had initial response to IVIG, however her symptoms worsened after two months and were resistant to further treatment. Subsequently the patient died and a post-mortem was performed. Neuropathology revealed perivascular and parenchymal lymphocytic infiltrates, with neuronophagia mediated by CD8+ T cells and microglia in brainstem, spinal cord, and mesial temporal lobe structures. These findings suggest a pathogenic role of cytotoxic CD8+ T-cells, with potential implication for therapy of future patients.

Axonal function in a family with episodic ataxia type 2 due to a novel mutation.

Episodic ataxia type 2(EA-2) is a rare, autosomal dominant disorder characterised by recurrent episodes of ataxia and dysarthria,due to mutations in the CACNA1A gene on chromosome 19 encoding voltage-dependent Ca2+ channels. The aim of the present study was to explore whether axonal membrane properties, assessed using nerve excitability techniques, were abnormal in patients with EA-2 . Nerve excitability techniques were applied to the median nerve of three individuals from three generations of a single family, all of whom had typical features of EA-2. This family was found to have a novel mutation at codon 1451 of the Ca2+ channel alpha 1A subunit. Nerve excitability testing demonstrated significant abnormalities,with all patients outside the normal 95 % confidence limits in having a high rheobase and reduced early hyperpolarizing threshold electrotonus. On average there were also significant reductions in refractoriness,late sub excitability and early depolarizing threshold electrotonus. Mathematical modelling indicated that a similar pattern of abnormalities may result from a reduced voltage dependence of slow K+ channels (KCNQ channels). There are significant and distinctive changes in peripheral nerve excitability in EA-2 patients,which are presumably induced indirectly. These findings raise the possibility that excitability testing may prove a convenient screening test for patients with this suspected channelopathy.