Pretreatment diagnosis of suprasellar papillary craniopharyngioma and germ cell tumors of adult patients.

BACKGROUND AND PURPOSE: Suprasellar papillary craniopharyngiomas and germ cell tumors in adults share some clinical and imaging similarities but have different therapeutic strategies and outcomes. This study aimed to evaluate the pretreatment diagnosis of these 2 tumors to improve the therapeutic outcome. MATERIALS AND METHODS: We retrospectively enrolled 18 adults with papillary craniopharyngiomas and 17 with germ cell tumors. The MR imaging findings were evaluated, including signal change and anatomic extension. The medical records were reviewed to collect clinical findings, management, and outcomes. RESULTS: The clinical findings of papillary craniopharyngiomas versus germ cell tumors were as follows: age: 46 ± 13.9 years versus 23 ± 7.1 years (P < .0001); diabetes insipidus: 2/18 (11%) versus 11/17 (65%) (P = .001); recurrence 13/16 (81%) versus 4/17 (24%) (P = .0031). The MR imaging findings of papillary craniopharyngiomas versus germ cell tumors were as follows­pituitary stalk thickening: 1.6 ± 0.4 mm versus 5.4 ± 4.2 mm (P < .0001); vertical infundibular extension: 1/18 (6%) versus 16/17 (94%) (P < .0001); sagittal spheric shape: 17/18 (94%) versus 1/17 (6%) (P < .0001); diffusion restriction: 1/17 (6%) versus 8/12 (67%) (P = .0009). CONCLUSIONS: Younger age, diabetes insipidus, MR imaging characteristics of restricted diffusion, and vertical infundibular extension favor the diagnosis of germ cell tumors. Spheric shape without infundibular infiltration provides clues to papillary craniopharyngiomas, which originate from the pars tuberalis and are located outside the third ventricle. We suggest that suprasellar germ cell tumor is possibly an intraventricular lesion. Appropriate treatment planning can be initiated according to the diagnosis and anatomic location.

Osmotic demyelination syndrome caused by normal saline correction in a patient admitted for gastric ulcer bleeding.

A 61-year-old male had osmotic demyelination syndrome caused by rapid correction of gastric ulcer bleeding and vomiting related hyponatraemia with normal saline. It is rare to see severe hyponatraemia caused by gastric ulcer bleeding and vomiting. Hypokalaemia may be the determinant predisposing factor. There was no specific brain image finding until 17 days after the initial clinical presentation of this disease. Brain diffusion weighted MRI series did not help for the early diagnosis in this case. Outcome of this case may be more favourable if we corrected his hyponatraemia with half-saline or other hypotonic saline and close monitored serum sodium level, and relowered with dextrose water and desmopressin once we observed that the correction rate of hyponatraemia was beyond the recommended rate.

Differences in brain metabolism associated with agitation and depression in Alzheimer's disease.

OBJECTIVE: Agitation and depression are among the commonest behavioural and psychological symptoms exhibited by Alzheimer's disease patients. However, their pathophysiology remains unclear. We therefore investigated the relationship between the brain metabolism in the posterior cingulate gyrus and the dorsolateral prefrontal cortex, and agitation and depression in patients diagnosed with Alzheimer's disease. METHODS: We recruited 26 patients (14 women and 12 men) with a mean age of 75 years and probable Alzheimer's disease. All patients completed the Mini-Mental State Examination (MMSE), the Geriatric Depression Scale-Short Form (GDS) assessment, and the Cohen-Mansfield Agitation Inventory (CMAI) in order to evaluate cognition, depression, and agitation, respectively. All subjects underwent magnetic resonance imaging and (1)H-magnetic resonance spectroscopy of the brain. The ratios of N-acetylaspartate (NAA), choline (Cho), and myo-inositol (mI) to creatine (Cr) in the posterior cingulate gyrus and the dorsolateral prefrontal cortex were measured and compared with neuropsychological test results. RESULTS: The MMSE scores correlated positively with the NAA/Cr ratio in the left posterior cingulate gyrus (r = 0.56; p = 0.001). The CMAI scores correlated negatively with the NAA/Cr ratio in the left posterior cingulate gyrus (r = -0.46; p = 0.02). The GDS scores correlated positively with the Cho/Cr ratio in the left dorsolateral prefrontal cortex (r = 0.59; p = 0.01), and mI/Cr in both left (r = 0.47; p = 0.03) and right (r = 0.47; p = 0.03) cingulate gyri. CONCLUSIONS: Agitation and depression levels correlated with different neurochemical metabolites in specific brain areas. We conclude that various neuropsychiatric symptoms might have separate pathophysiologies.

Evaluation of the outcomes of endovascular management for patients with head and neck cancers and associated carotid blowout syndrome of the external carotid artery.

AIM: To evaluate factors related to the technical and haemostatic outcomes of endovascular management in patients with head and neck cancers (HNC) associated with carotid blowout syndrome (CBS) of the external carotid artery (ECA). MATERIALS AND METHODS: Between 2002 and 2011, 34 patients with HNC with CBS involving branches of the ECA underwent endovascular therapy. Treatment included embolization with microparticles, microcoils, or acrylic adhesives. Fisher's exact test was used to examine demographic features, clinical and angiographic severities, and clinical and imaging findings as predictors of endovascular management outcomes. RESULTS: Technical success and immediate haemostasis were achieved in all patients. Technical complications were encountered in one patient (2.9%). Rebleeding occurred in nine patients (26.5%). Angiographic vascular disruption grading from slight (1) to severe (4) revealed that the 18 patients with acute CBS had scores of 2 (2/18, 11.1%), 3 (3/18, 16.7%), and 4 (13/18, 72.2%). The 16 patients with impending and threatened CBS had scores of 1 (1/16, 6.25%), 2 (5/16, 31.25%), and 3 (10/16, 62.5%; p = 0.0003). For the 25 patients who underwent preprocedural computed tomography (CT)/magnetic resonance imaging (MRI) examinations within 3 months of treatment, the agreement between clinical and imaging findings reached the sensitivity, specificity, and kappa values for recurrent tumours (1, 0.7143, 0.7826), soft-tissue defect (0.9091, 0.3333, 0.2424), and sinus tract/fistula (0.4737, 0, 0.4286). CONCLUSION: Endovascular management for patients with CBS of the ECA had high technical success and safety but was associated with high rebleeding rates. We suggest applying aggressive post-procedural follow-up and using preprocedural CT/MRI to enhance the periprocedural diagnosis.

Identification of two novel missense mutations in the KAL1 gene in Han Chinese subjects with Kallmann Syndrome.

OBJECTIVES: To identify mutations in the KAL1, the KAL2, and PROKR2/PROK2 genes and to characterize phenotypic features in 5 Chinese subjects with Kallmann Syndrome (KS) and 6 subjects with normosmic hypogonadotrophic hypogonadism (NHH) in Taiwan. DESIGN AND PATIENTS: Five unrelated males (age range 22-52 yr) with clinical manifestations of KS and 6 unrelated males (age range 24-47 yr) with NHH were analyzed. In addition, 5 relatives of KS subjects were also evaluated. Genomic DNA extraction, PCR, and DNA sequence analyses were performed using standard procedures. RESULTS: The 1st patient had a single missense mutation in his copy of the KAL1 gene, a T→G transversion in codon 134 that results in replacement of cysteine by gly cine. The 2nd affected subject had a single missense mutation in the KAL1 gene, a T→C transition in codon 163 that results in replacement of cysteine by arginine. The 3rd case was hemizygous for a nonsense mutation in codon 424 of exon 9 (c.CGA→TGA) of the KAL1 gene. This mutation predicts a markedly truncated protein. Two of the mutations (p.C134G and p.C163R) we identified in the KAL1 gene are novel. CONCLUSIONS: We identified 3 mutations, including 2 novel mutations, in the KAL1 gene in patients with KS in Taiwan. These data extend the variety of KAL1 gene mutations in KS and further define the role of the KAL1 protein in olfactory bulb development.

Contribution of intracranial vertebral artery asymmetry to vestibular neuropathy.

OBJECTIVES: To test the hypothesis that vertebral artery hypoplasia (VAH) may affect the lateralisation of vestibular neuropathy (VN), probably through haemodynamic effect on the vestibular labyrinth. METHODS: 69 patients with unilateral VN were examined with a magnetic resonance angiographic (MRA) and caloric test. 50 healthy subjects served as controls. The diagnosis of intracranial VAH was based on MRA if <0.22 cm in VA diameter and a diameter asymmetry index >40%. The authors then correlated the canal paretic side with the VAH side. RESULTS: MRA study revealed 29 VAH (right/left: 23/6) in VN subjects and six VAH in controls (right/left: 5/1). The RR of VAH in VN subjects compared with controls was elevated (RR=2.2; 95% CI 1.8 to 2.8). There was a high accordance rate between the side of VAH and VN. Among 29 patients with unilateral VAH, 65.5% (N=19) had an ipsilateral VN, in which left VAH showed a higher accordance rate (83.3%) than the right side (60.9%). VN subjects with vascular risk factors also had a higher VAH accordance rate (81%) than those without (25%). CONCLUSIONS: VAH may serve as a regional haemodynamic negative contributor and impede blood supply to the ipsilateral vestibular labyrinth, contributing to the development of VN, which could be enhanced by atherosclerotic risk factors and the left-sided location.

Three paediatric patients with reversible cerebral vasoconstriction syndromes.

Reversible cerebral vasoconstriction syndromes (RCVS) occur predominantly in middle-aged women. Only one child with RCVS has ever been reported. We report three boys (aged 10, 16 and 10 years) who had multiple thunderclap headaches, including one boy with bath-related attacks. None of them had secondary causes. All their magnetic resonance angiography demonstrated reversible cerebral vasoconstrictions of the major arteries with increased cerebral blood flow velocities on transcranial colour-coded sonography studies. Two of the three had hypertensive surges during headache attacks. None of the three boys had seizure, stroke or other neurological deficits. However, a left occipital subcortical lesion of unknown nature was noted in one boy. The efficacy of calcium channel blockers was variable in these three boys. This report suggests that, although rare, RCVS should be considered in paediatric patients if they present with a history of multiple attacks of thunderclap headache.

Heavily T2-weighted MR myelography vs CT myelography in spontaneous intracranial hypotension.

OBJECTIVE: To assess the diagnostic accuracy of heavily T2-weighted magnetic resonance myelography (MRM) in patients with spontaneous intracranial hypotension (SIH). METHODS: Patients with SIH were recruited prospectively, and first underwent MRM and then computed tomographic myelography (CTM). The results of MRM were validated with the gold standard, CTM, focusing on 1) CSF leaks along the nerve roots, 2) epidural CSF collections, and 3) high-cervical (C1-3) retrospinal CSF collections. Comparisons of these 3 findings between the 2 studies were made by kappa statistics and agreement rates. Targeted epidural blood patches (EBPs) were placed at the levels of CSF leaks if supportive treatment failed. RESULTS: Nineteen patients (6 men and 13 women, mean age 37.9 +/- 8.6 years) with SIH completed the study. MRM did not differ from CTM in the detection rates of CSF leaks along the nerve roots (84% vs 74%, p = 0.23), high-cervical retrospinal CSF collections (32% vs 16%, p = 0.13), and epidural CSF collections (89% vs 79%, p = 0.20). MRM demonstrated more spinal levels of CSF leaks (2.2 +/- 1.7 vs 1.5 +/- 1.5, p = 0.011) and epidural collections (12.2 +/- 5.9 vs 7.1 +/- 5.8, p < 0.001) than CTM. The overall level-by-level concordance was substantial for CSF leaks along the nerve roots (C1-L3) (kappa = 0.71, p < 0.001, agreement = 95%) and high-cervical retrospinal CSF collections (C1-3) (kappa = 0.73, p < 0.001, agreement = 92%), and moderate for epidural CSF collections (C1-L3) (kappa = 0.47, p < 0.001, agreement = 72%). Ten of the 14 patients (71%) receiving targeted EBPs experienced sustained symptomatic relief after a single attempt. CONCLUSIONS: Heavily T2-weighted magnetic resonance myelography was accurate in localizing CSF leaks for patients with spontaneous intracranial hypotension. This noninvasive technique may be an alternative to computed tomographic myelography before targeted epidural blood patches.

Comparisons between heavily T2-weighted MR and CT myelography studies in two patients with spontaneous intracranial hypotension.

Our previous study demonstrated that heavily T2-weighted magnetic resonance (MR) myelography could successfully detect abnormal cerebrospinal fluid (CSF) collections in patients with spontaneous intracranial hypotension (SIH); however, its ability to demonstrate ongoing CSF leakage sites is uncertain. Currently, computed tomographic (CT) myelography is still considered the study of choice for such a purpose. In this study, we compared the results of the two imaging modalities in two patients with SIH. CSF leakages, such as C1-2 extraspinal collections, CSF along nerve root sleeves, and epidural fluid collections were noted on both studies with minor discrepancies. CSF collection along nerve root sleeves demonstrated by MR myelography was the most likely ongoing leakage site in comparison with CT myelography. Targeted epidural blood patches in both patients resulted in complete headache resolution. Non-invasive, non-contrasted and time-saving MR myelography is a promising study for patients with SIH. Further large-scale validation studies are needed.

Bath-related thunderclap headache: a study of 21 consecutive patients.

We consecutively recruited 21 patients (all women, mean 54 +/- 8 years) with bath-related thunderclap headache (BRTH). Thirteen of them were in menopause, two had just ceased hormonal therapy, and one was at 3 months postpartum. Bathing was the initial trigger for thunderclap headaches in nine patients (43%). Many patients (n = 15, 71%) had other non-bath-related attacks. Most patients (n = 18, 86%) reported that the headache occurred immediately when water was sprayed over their body, with warm water (52%) as the most common. During the disease course [mean 14 days (6-34)], the mean number of BRTH was 5.1 +/- 3.6 attacks. Nineteen patients (90%) changed bathing habits to prevent attacks. Thirteen patients (62%) had magnetic resonance angiography vasoconstrictions, and two of them (15%) developed reversible posterior encephalopathy. None of the patients without vasoconstrictions had this complication. Nimodipine was effective in stopping further attacks in 84% (16/19) treated patients. No relapse was reported at a mean follow-up of 30 months. BRTH occurred exclusively in women and predominantly in middle age. Deficiency or fluctuation of female sex hormones may play a role. About 60% patients showed cerebral vasospasms, fulfilling the diagnosis of reversible cerebral vasoconstriction syndrome and indicating a risk of posterior encephalopathy.

Spontaneous intracranial hypotension with isolated cortical vein thrombosis and subarachnoid haemorrhage.

Spontaneous intracranial hypotension (SIH) associated with subarachnoid haemorrhage (SAH) has never been reported. Here, we report on a case of a 33-year-old woman with SIH, who developed simple partial sensory seizures 3 weeks later. Neuroimaging studies, including brain computed tomography and angiography, were initially normal, but revealed an isolated cortical venous thrombosis at 3 weeks. One week later, brain magnetic resonance imaging showed SAH around the thrombosed cortical vein. We postulate that the decline in the venous blood flow velocity due to SIH may have resulted in cortical venous thrombosis, which in turn led to rupture of the vessel wall and SAH in this patient.

Heavily T2-weighted MR myelography in patients with spontaneous intracranial hypotension: a case-control study.

We performed whole-spine heavily T2-weighted magnetic resonance (MR) myelography using a single-shot fast spin-echo pulse sequence in 17 patients (8 M/9 F) with spontaneous intracranial hypotension (SIH) to detect abnormal cerebrospinal fluid (CSF) collections. In addition, a group of age- and sex-matched controls were recruited. Follow-up MR myelography was also done at 3 weeks. MR myelography showed three kinds of abnormal CSF collections in 15 patients with SIH (88%): epidural fluid collection (n = 15, 88%), C1-2 extraspinal collections (n = 6, 35%) and CSF collections along nerve roots in the lower cervical or upper thoracic spines (n = 6, 35%). One patient (6%) showed a meningeal diverticulum. In contrast, none of the controls showed these findings. Overall, MR myelography results helped in early diagnosis of SIH in four (24%) patients whose initial brain MRIs failed to show typical SIH findings. Follow-up MR myelography results were compatible with the clinical changes with kappa statistics of 0.52 and an agreement rate of 76%. Our study showed heavily T2-weighted MR myelography provided a rapid, non-invasive and high yield method to diagnose and follow-up patients with SIH. Whether the CSF collections along the nerve roots represent the ongoing leakage sites warrants further study.

Carotid blowout syndrome in patients with head-and-neck cancers: reconstructive management by self-expandable stent-grafts.

BACKGROUND AND PURPOSE: Some reports of reconstructive management of carotid blowout syndrome (CBS) with stent-grafts are promising, but some are unfavorable. This study sought to evaluate the hemostatic efficacy, safety, and outcome of reconstructive, endovascular stent-graft placement in patients with head-and-neck cancers in association with CBS. METHODS: Eight patients with head-and-neck cancers with CBS were treated with self-expandable stent-grafts. We evaluated the initial hemostatic results, complications, and outcomes by assessing the clinical and imaging findings. RESULTS: Immediate hemostasis was achieved in all patients. Initial complications included stroke in 1 patient and asymptomatic thrombosis of the carotid artery in 2 patients. Delayed complications included rebleeding, delayed carotid thrombosis, and brain abscess formation. Rebleeding was noted in 4 patients and was successfully managed with a second stent-graft and embolization in 2 of them. Delayed carotid thrombosis with follow-up after 3 months was found in 3 patients, 1 of whom had associated brain abscesses. CONCLUSION: Although stent-grafts achieved immediate and initial hemostasis in patients with head-and-neck cancers and CBS, long-term safety, stent patency, and permanency of hemostasis appeared unfavorable. This treatment may be for temporary or emergency purposes rather than serving as a permanent measure. We suggest its applications in patients with acute CBS that precludes performance of an occlusion test, as well as when carotid occlusion poses an unusually high risk of neurologic morbidity. We also propose prophylactic antibiotic treatment and combined embolization of pathologic vascular feeders to improve outcomes.

Subdural haematoma in patients with spontaneous intracranial hypotension.

The incidence and clinical relevance of subdural haematoma (SDH) in patients with spontaneous intracranial hypotension (SIH) remain undetermined. We reviewed 40 consecutive SIH patients (18 female, 22 male) in a tertiary hospital. Eight (20%) of them had SDH and nine (23%), non-haemorrhagic subdural collections. The presence of SDH was associated with higher frequencies of male gender, recurrence of severe headache and neurological deficits. Outcomes were satisfactory after supportive care or epidural blood patches except for one SDH patient, who developed transtentorial herniation resulting in Duret haemorrhage and infarctions of bilateral posterior cerebral artery territories. In conclusion, subdural fluid collections were common in patients with SIH. SDH was associated with headache worsening or neurological deficits. Patients with SDH generally recovered well; however, serious sequela might occur.

Recurrent primary thunderclap headache and benign CNS angiopathy: spectra of the same disorder?

OBJECTIVES: To investigate the clinical pictures of patients with recurrent thunderclap headaches of unknown etiology and to field-test two relevant International Classification of Headache Disorders, 2nd edition (ICHD-II) criteria, i.e., primary thunderclap headache (Code 4.6) and benign (or reversible) angiopathy of the CNS (Code 6.7.3). METHODS: We prospectively recruited patients presenting with idiopathic recurrent thunderclap headaches from a hospital-based headache clinic. Detailed histories, neurologic examinations, and MRIs and magnetic resonance angiographies (MRAs) were performed in all patients to exclude secondary causes. Patients with cerebral vasoconstriction received serial MRA follow-up. RESULTS: Fifty-six consecutive patients (51 female/5 male, mean age 49.6 +/- 9.8 [range 22 to 76] years) were enrolled. Segmental vasoconstriction (or benign CNS angiopathy) was found in 22 patients (39%). Thunderclap headache recurred in all patients with a median frequency of 0.7 times per day for a median period of 14 days (range 6 to 86 days). The median duration for each single attack was 3 hours. Most patients (84%) reported at least one trigger. Nimodipine effectively aborted further attacks in 83% of the treated patients. Headache attacks subsided within 3 months. Four patients (7%) developed ischemic complications. Patients with and without vasoconstriction based on MRA images were similar regarding demographics and headache profile. Except for the duration criterion, our patients generally mapped well into the proposed ICHD-II criteria. CONCLUSIONS: This study suggests that the two diagnostic entities proposed by the ICHD-II may present different spectra of the same disorder. The distinct headache profile may help physicians quickly recognize this disabling headache disorder with risk of stroke and provide timely treatment.

Brain abscess formation: a delayed complication of carotid blowout syndrome treated by self-expandable stent-graft.

A patient with hypopharyngeal cancer developed carotid blowout syndrome (CBS) treated by self-expandable stent-graft in the left carotid artery. CT scan for progressive right hemiparesis 4 months later showed multiple left cerebral abscesses and left carotid thrombosis. Although deployment of stent-grafts for CBS can achieve initial hemostasis in patients with head-and-neck cancer, the placement of a stent-graft in a field of necrosis and infection is associated with poor long-term outcome. We recommend the use of prophylactic antibiotics if endovascular foreign materials are placed in a contaminated field.

Is vasospasm requisite for posterior leukoencephalopathy in patients with primary thunderclap headaches?

Primary thunderclap headache (TCH) is sometimes associated with cerebral vasospasm. However, the role of vasospasm in relation to the development of reversible or irreversible posterior leukoencephalopathy among patients with primary TCH has never been fully addressed. This paper includes a report on a 51-year-old woman with primary TCH complicated with posterior leukoencephalopathy and a literature review of 16 further patients with the same illness. Their magnetic resonance or conventional angiographic findings were clearly described. Our review found that all these 17 patients showed evidence of cerebral vasospasm. Eleven (65%) of them developed permanent ischaemic infarctions, almost exclusively located at the watershed zones. We suggest that the presence of vasospasm might be requisite for posterior leukoencephalopathy as well as for permanent infarctions in these patients. Therefore, searching for any clue of vasospasm is mandatory in treatment of patients with primary TCH. Absence of an accompanying vasospasm might predict a good outcome.

Reduction in hypothalamic 1H-MRS metabolite ratios in patients with cluster headache.

OBJECTIVE: To determine the 1H-MR spectroscopic (MRS) findings in the hypothalamus in patients with episodic cluster headache. METHODS: 47 patients were recruited with episodic cluster headache (35 in cluster period and 12 in remission), 21 normal controls, and 16 patients with chronic migraine. The hypothalamic 1H-MRS metabolite ratio changes in patients with cluster headache were evaluated and compared with results in the normal controls as well as patients with chronic migraine. Seven patients in the cluster period group underwent a follow up hypothalamic MRS study five to six months after remission. RESULTS: In patients with cluster headache, the hypothalamic N-acetylaspartate (NAA)/creatine (Cr) and choline (Cho)/Cr ratios were similar between those in cluster period and in remission. As a group, both NAA/Cr and Cho/Cr levels were significantly lower in patients with cluster headache in comparison with either the control or chronic migraine groups. In those with a follow up MRS study, the levels of metabolite ratios did not differ between the cluster and remission periods. CONCLUSIONS: This study provides evidence of persistent biochemical change of the hypothalamus in patients with episodic cluster headache. Low levels of NAA/Cr and Cho/Cr suggest that cluster headache might be related to both neuronal dysfunction and changes in the membrane lipids in the hypothalamus.