Cranial nerve palsies in patients with hematological malignancies: a case series.

Objectives: Cranial neuropathies (CNs) can be due to a wide spectrum of causes, and the differential diagnosis is particularly challenging in patients with positive history of hematological malignancies, when neoplastic meningitis (NM) must be excluded.Patients and Methods: We retrospectively selected a series of twelve haematological patients with isolated cranial neuropathies (ICNs) or multiple cranial neuropathies (MCNs). among 71 patients that developed neurologic symptoms during different stages of the cancer, between 1 January, 2010 and 31 December, 2017. Brain and cauda equina magnetic resonance imaging (MRI) with gadolinium, cerebrospinal fluid (CSF) analysis, including flow cytometry for cell immunophenotyping and microbiological exams were performed in all patients.Results: Patients developed signs and symptoms of involvement of isolated (n = 11) or multiple (n = 1) cranial nerves, at different stages of the primary disease, and, in 5 of these cases in complete remission after hematopoietic stem cell transplantation. Among the 5 cases that eventually were diagnosed as having NM, cerebrospinal fluid was positive for neoplastic cells in 3, and MRI gadolinium-enhancement was present in 3. The other episodes were attributed to heterogeneous pathologies that were unrelated to meningeal infiltration by neoplastic cells.Conclusions: Our observations confirm that NM in haematological malignancies can yield insidious isolated signs of cranial nerves. Only a multidisciplinary approach allows prompt recognition of these conditions through a challenging process of differential diagnosis, and proper therapies.

Quantification and regulation of the adipokines resistin and progranulin in human cerebrospinal fluid.

BACKGROUND: Adipokines bearing the potential to cross the blood-brain barrier (BBB) are promising candidates for the endocrine regulation of central nervous processes and of a postulated fat-brain axis. Resistin and progranulin concentrations in paired serum and cerebrospinal fluid (CSF) samples of patients undergoing neurological evaluation and spinal puncture were investigated. MATERIALS AND METHODS: Samples of n = 270 consecutive patients with various neurological diseases were collected without prior selection. Adipokine serum and CSF concentrations were measured by enzyme-linked immunosorbent assay and serum and CSF routine parameters by standard procedures. Anthropometric data, medication and patient history were available. RESULTS: Serum levels of resistin and progranulin were positively correlated among each other, with respective CSF levels, low-density lipoprotein cholesterol levels and markers of systemic inflammation. CSF resistin concentrations were generally low. Progranulin CSF concentrations and CSF/serum progranulin ratio were significantly higher in patients with infectious diseases, with disturbed BBB function and with elevated CSF cell count and presence of oligoclonal bands. Both adipokines are able to cross the BBB depending on a differing patency that increases with increasing grade of barrier dysfunction. Whereas resistin represents a systemic marker of inflammation, CSF progranulin levels strongly depend on the underlying disease and dysfunction of blood-CSF barrier. CONCLUSIONS: Resistin and progranulin represent novel and putative regulators of the fat-brain axis by their ability to cross the BBB under physiological and pathophysiological conditions. The presented data provide insight into the characteristics of BBB function regarding progranulin and resistin and the basis for future establishment of normal values for CSF concentrations and CSF/serum ratios.

Cranial nerve palsies after shunting of an isolated fourth ventricle.

An isolated fourth ventricle is characterised by cerebrospinal fluid (CSF) trapping in the fourth ventricle. Although there is no consensus regarding treatment, ventriculoperitoneal (VP) shunting of the fourth ventricle is an option. Complications include infection, mechanical irritation of the brainstem, malfunction and overdrainage. Cranial nerve palsy is a rare complication and has been mostly described in children. We present two adult cases of abducens and facial nerve palsies occurring secondary to this procedure. Placement of a higher resistance valve brought about complete recovery in one patient while withdrawal of the catheter by a few millimetres led to complete recovery in the second patient.

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.

Ophthalmoplegic "migraine" or recurrent ophthalmoplegic cranial neuropathy: new cases and a systematic review.

Ophthalmoplegic migraine is a poorly understood neurologic syndrome characterized by recurrent bouts of head pain and ophthalmoplegia. By reviewing cases presenting to our centers in whom the phenotype has been carefully dissected, and systematically reviewing all published cases of ophthalmoplegic migraine in the magnetic resonance imaging (MRI) era, this review sets out to clearly define the syndrome and discuss possible etiologies. We found that in up to one-third of patients, the headache was not migrainous or associated with migrainous symptoms. In three-quarters of the cases involving the third nerve, there was focal nerve thickening and contrast enhancement on MRI. Observational data suggest systemic corticosteroids may be beneficial acutely. The etiology remains unclear, but may involve recurrent bouts of demyelination of the oculomotor nerve. "Ophthalmoplegic migraine" is a misnomer in that it is probably not a variant of migraine but rather a recurrent cranial neuralgia. A more appropriate name might be "ophthalmoplegic cranial neuropathy."

Acute leukemia presenting with blasts first found in the cerebrospinal fluid but not in the peripheral blood.

Acute leukemia presenting with central nervous system (CNS) signs and symptoms is uncommon and prone to be misdiagnosed. Here, we report nine patients with acute leukemia, including five patients with acute lymphoblastic leukemia (ALL) and four patients with acute myeloid leukemia (AML). These patients presented with symptoms suggestive of involvement of multiple cranial nerves, the spinal cord, and meningeal involvement. Moreover, we found that all these patients unexpectedly showed the presence of blasts in the cerebrospinal fluid (CSF) but not in the peripheral blood despite repeated examinations. Bone marrow examination confirmed the presence of acute leukemia in these patients. Seven patients died within 18months of diagnosis and two patients developed stable disease. Our findings show a novel presenting feature of acute leukemia and highlight the importance of CSF cytology in the diagnosis of acute leukemia.

[Predictive value of MRI for detecting and characterizing vascular compression in cranial nerve hyperactivity syndromes (trigeminal and facial nerves)]. / Valeur prédictive de l'IRM pour la détection et la caractérisation de la compression vasculaire dans les syndromes d'hyperactivité des nerfs crâniens (trijumeau et facial).

MRI detects vascular compression of the cranial nerve in the majority of the cases. High-resolution 3D-T1 and 3D-T2 MRI gives detailed images, particularly the 3D-T2 MRI sequences, with good contrast between cerebrospinal fluid and vascular and nerve structures. TOF-AMR (native sequence and vertebrobasilar reconstruction) shows the vascular structures in hypersignal and therefore differentiates the vessels from the cranial nerves. The 3D-T1 sequence with gadolinium reinforces the signal of the venous structures. Thus, preoperative MRI makes it possible to predict the existence of a vascular compression. The correlation study between imaging data and intraoperaitive anatomical findings showed a sensitivity of MRI of 97% and a specificity of 100%. In addition, it can specify the type and the degree of the compression. This information may help in selecting the most appropriate surgical method.

Effect of craniocervical decompression on peak CSF velocities in symptomatic patients with Chiari I malformation.

BACKGROUND AND PURPOSE: Peak CSF velocities detected in individual voxels in the subarachnoid space in patients with Chiari I malformations exceed those in similar locations in the subarachnoid space in healthy subjects. The purpose of this study was to test the hypothesis that the peak voxel velocities are decreased by craniocervical decompression. METHODS: A consecutive series of patients with symptomatic Chiari I malformations was studied before and after craniocervical decompression with cardiac-gated, phase contrast MR imaging. Velocities were calculated for each voxel within the foramen magnum at 14 time points throughout the cardiac cycle. The greatest velocities measured in a voxel during the cephalad and caudad phases of CSF flow through the foramen magnum were tabulated for each patient before and after surgery. The differences in these velocities between the preoperative and postoperative studies were tested for statistical significance by using a single-tailed Student's t test of paired samples. RESULTS: Eight patients with a Chiari I malformation, including four with a syrinx, were studied. Peak caudad velocity diminished after craniocervical decompression in six of the eight patients, and the average diminished significantly from 3.4 cm/s preoperatively to 2.4 cm/s postoperatively (P =.01). Peak cephalad velocity diminished in six of the eight cases. The average diminished from 6.9 cm/s preoperatively to 3.9 cm/s postoperatively, a change that nearly reached the significance level of.05 (P =.055). CONCLUSION: Craniocervical decompression in patients with Chiari I malformations decreases peak CSF velocities in the foramen magnum. The study supports the hypothesis that successful treatment of the Chiari I malformation is associated with improvement in CSF flow patterns.

Peripheral third cranial nerve enhancement in multiple sclerosis.

Cranial nerve III dysfunction in multiple sclerosis (MS) is uncommon. Seven cases of isolated cranial nerve III paresis associated with MS have been reported in the English-language literature. MR imaging was obtained in five cases demonstrating lesions within the midbrain. We present the detailed clinical and MR imaging findings of a young woman with MS and an isolated, painful pupil involving complete left cranial nerve III palsy. Initial MR imaging showed isolated enhancement of the cisternal portion of the cranial nerve III, suggesting that peripheral nervous system involvement may develop as part of the disease process in some patients with MS.

Prednisolone (30-60 mg/day) for diseases other than AD decreases amyloid beta-peptides in CSF.

The effect of corticosteroid on the concentration of amyloid beta-peptide (Abeta) in human CSF obtained from 16 patients without dementia treated with prednisolone (> or =30 mg daily) was studied. The concentrations of Abetax-40 and Abetax-42 in CSF decreased after treatment was started (p < 0.002). A moderate- or high-dose regimen of prednisolone decreases Abeta production or increases Abeta degradation in the human brain and deserves further study in AD.

Relapsing-remitting autoimmune agrypnia.

A woman affected by multiple cranial nerve palsy developed several episodes of total insomnia and respiratory crises resulting from central breathing depression associated with dysautonomic symptoms. Oligoclonal IgG bands were present in her cerebrospinal fluid, and immunohistochemistry showed increased binding of serum and cerebrospinal fluid on gamma-aminobutyric acid-ergic, synapse-rich neuronal cells. Immunosuppressive treatment and plasma exchange were followed by clinical improvement, with restoration of sleep architecture and disappearance of respiratory crises, suggesting autoimmune pathogenesis of the syndrome.

[Idiopathic cranial polyneuropathies]. / Polyneuropathies crâniennes idiopathiques.

A series of 43 cases with multiple cranial nerve deficits was collected between 1972 and 1990. No diagnosis was established in 15 cases. The facial and the trigeminal nerves were most frequently affected. In 10 cases, a monophasic course was observed usually with recovery. Recurrence was present in 5 cases. An inflammatory mechanism was likely in 10 cases. Nosological relations between these cases and either Bell's palsy (idiopathic facial paralysis) or Tolosa-Hunt's syndrome are discussed.