Awake tracheal intubation with the ProVu™ video stylet: a case series.

Management of the difficult airway is integral to peri-operative practice. The development of devices to simplify airway handling are changing our approach to tracheal intubation. We report a case series of three patients who underwent awake tracheal intubation with the ProVu™ video stylet (Flexicare Medical Ltd, Mountain Ash, UK). All three patients had predicted difficult airways, including: limited mouth opening secondary to radiotherapy; previous exenteration, hemi-maxillectomy and scapular free flap formation; and cervical fixation. Awake tracheal intubation was performed successfully in all three cases, with no complications reported. This is the first reported case series of the ProVu™ video stylet as an awake tracheal intubation device. The benefits and limitations of this video stylet are discussed.

Apraxia of speech and cerebellar mutism syndrome: a case report.

BACKGROUND: Cerebellar mutism syndrome (CMS) or posterior fossa syndrome (PFS) consists of a constellation of neuropsychiatric, neuropsychological and neurogenic speech and language deficits. It is most commonly observed in children after posterior fossa tumor surgery. The most prominent feature of CMS is mutism, which generally starts after a few days after the operation, has a limited duration and is typically followed by motor speech deficits. However, the core speech disorder subserving CMS is still unclear. CASE PRESENTATION: This study investigates the speech and language symptoms following posterior fossa medulloblastoma surgery in a 12-year-old right-handed boy. An extensive battery of formal speech (DIAS = Diagnostic Instrument Apraxia of Speech) and language tests were administered during a follow-up of 6 weeks after surgery. Although the neurological and neuropsychological (affective, cognitive) symptoms of this patient are consistent with Schmahmann's syndrome, the speech and language symptoms were markedly different from what is typically described in the literature. In-depth analyses of speech production revealed features consistent with a diagnosis of apraxia of speech (AoS) while ataxic dysarthria was completely absent. In addition, language assessments showed genuine aphasic deficits as reflected by distorted language production and perception, wordfinding difficulties, grammatical disturbances and verbal fluency deficits. CONCLUSION: To the best of our knowledge this case might be the first example that clearly demonstrates that a higher level motor planning disorder (apraxia) may be the origin of disrupted speech in CMS. In addition, identification of non-motor linguistic disturbances during follow-up add to the view that the cerebellum not only plays a crucial role in the planning and execution of speech but also in linguistic processing. Whether the cerebellum has a direct or indirect role in motor speech planning needs to be further investigated.

Central nervous system metastases of pulmonary adenocarcinoma mimicking neurofibromatosis type 2.

We report a case of a 51-year-old man presenting with rapidly progressive unilateral tinnitus, hearing loss and imbalance. Neuroimaging revealed bilateral VIIIth cranial nerve masses and multiple cerebral and spinal cord lesions that were interpreted as being acoustic schwannomas and multiple meningeomas. An initial tentative diagnosis of neurofibromatosis type 2 (NF2) was made. Both clinical and radiological evolution were atypical for NF2 and the initial diagnosis of NF2 was questioned. Additional technical investigations demonstrated a pulmonary adenocarcinoma. Postmortem examination confirmed that this patient had multiple central nervous system metastases of a primary pulmonary adenocarcinoma, presenting clinically and neuroradiologically as a probable neurofibromatosis type 2. Clinicians should be aware of the rare possibility of central nervous system metastases mimicking NF2.

Radiological classification of anterior skull base anatomy prior to performing medial orbital wall decompression.

PURPOSE: To study a radiological classification, originally described by Keros in 1965, which provides an objective assessment of anterior skull base anatomy relevant in patients undergoing external medial orbital decompression. MATERIALS AND METHODS: The classification is based on anatomical landmarks measured via coronal CT-scan. The patients are divided into 3 Keros categories based on their olfactory fossa depth; Keros 1 (1-3 mm), Keros 2 (4-7 mm) and Keros 3 (8-16 mm). A cross-sectional group of 32 consecutive patients on the hospital radiology database with coronal CT scans were classified according to the Keros system. RESULTS: All the patients fell into one of the three Keros categories. Anatomical associations of the Keros classification suggest that Keros 1 patients have the least risk of intracranial entry whilst Keros 3 patients carry the greatest risk. CONCLUSION: Keros classification provides an objective assessment of anterior skull base anatomy and can therefore guide the surgeon on the superior extent of medial wall bone removal during orbital decompression. This may help improve the safety profile of the procedure.

Occipital pain in osteoid osteoma of the atlas. A report of two cases.

STUDY DESIGN: Two cases of occipital pain caused by an osteoid osteoma of the atlas are presented. OBJECTIVES: To describe the management of occipital pain in two young patients. SUMMARY OF BACKGROUND DATA: Osteoid osteoma is a benign lesion mostly affecting the long bones. A spinal location is uncommon. To the authors' knowledge, there are only five other reports of an osteoid osteoma located in the atlas. METHODS: Occipital headache, which was relieved by salicylates, was the major symptom reported by the two adolescents. In the first patient, a lesion of C1 was seen on plain radiographs. In the second patient, the diagnosis of osteoid osteoma was suggested by scintigraphic imaging and subsequently by computed tomography. RESULTS: Pain disappeared in both cases after surgical excision of the lesion. Histologic examination disclosed characteristic features of osteoid osteoma. CONCLUSIONS: Occipital pain in adolescents, which is relieved by aspirin, should raise suspicion about the possibility of an osteoid osteoma of the atlas. If standard cervical spine radiographs are negative, isotope scanning and computed tomography can help to establish the diagnosis. Complete excision eliminates the lesion and produces immediate relief for the patient.

Obstructive hydrocephalus as complication of a giant nonfunctioning pituitary adenoma: therapeutical approach.

We report the case of a 61-year-old male presenting with a giant nonfunctioning pituitary tumour extending into the third ventricle and thereby causing obstructive hydrocephalus. The main complaints consisted of disturbed vision and somnolence. The patient was initially treated by ventriculoperitoneal shunting, with immediate improvement of his mental status. Transcranial resection of the tumour was performed shortly thereafter, but only a limited part of the tumour could safely be removed. By immunohistochemistry the diagnosis of gonadotroph adenoma was made. After the operation, the patient's vision deteriorated temporarily. Because of the risk of further damage to the optic nerve, radiation therapy was postponed. Instead, treatment with the long-acting dopamine agonist cabergoline was instituted, which resulted in a gradual improvement of vision. Two years later a transsphenoidal operation was performed because of acute worsening of the visual fields due to central tumour necrosis with slight volume expansion. Vision improved considerably after surgery. Radiotherapy is scheduled in the near future. This case is an illustration of the complications of giant nonfunctioning pituitary adenomas. The therapeutical approach to obstructive hydrocephalus in these instances is reviewed.

Synthesis of glycosides in which the aglycon is an N-(hydroxymethyl)amino-1,3,5-triazine derivative.

The synthesis of analogues of the anti-tumour drug 2-[N-(hydroxymethyl)methylamino]-4,6-bis(dimethylamino)-1,3,5-triazine (HMPMM) in which the OH or a dimethylamino group is replaced by a carbohydrate has been explored. Triazinyl beta-glycosides were readily prepared by reaction of sugars with trimethyl-triazinylammonium salts. These were made with one or two methylamino groups on the triazine for reaction with formaldehyde to give the cytotoxic NMeCH2OH group. However, reaction of the triazinyl glycosides with formaldehyde gave complex intractable mixtures. When the carbohydrate portion was changed to the fully protected 2,3,4,6-tetra-O-acetyl glucose a good yield of the 2-[N-(hydroxymethyl)methylamino]-4-(dimethylamino)-1,3,5-triazin-2 -yl tetra-O-acetyl beta-glucoside was obtained. However, de-acetylation using sodium methoxide also removed the N-CH2OH group. We are investigating protection of the base-sensitive N-CH2OH group as trialkylsilyl and benzyl ethers and are looking at de-acetylation methods that are more selective. We have prepared glycosides in which the sugar is joined through the oxygen of the NMeCH2OH group. Coupling of acetobromoglucose with HMPMM catalysed by silver salts was not successful. Although methyl and cyclohexyl derivatives of HMPMM may be produced in high yields by reaction of HMPMM with methyl and cyclohexyl alcohols under acidic catalysis, production of glycosides in this way gave poor yields. MNDO calculations on reactions of HMPMM helped us devise improved reaction conditions for the condensation of 2,3,4,6-tetra-O-acetyl glucose with HMPMM and its derivatives. The best procedure to generate one of the target glycosides is to react 2,3,4,6-tetra-O-acetyl glucose and formaldehyde with 2-methylamino-4,6-bis(dimethylamino)-1,3,5-triazine. The beta-glycoside product was de-acetylated using potassium carbonate in dry methanol.

Pituitary metastasis mimicking a pituitary adenoma. A description of two cases.

The cases of two elderly women treated for temporal hemianopsia due to a large pituitary mass with suprasellar extension are presented. In both cases, the clinical picture, without diabetes insipidus and cranial nerve paralysis, as well the neuroimaging and endocrinological investigation showing hypopituitarism, were suggestive of a non-secreting pituitary adenoma. In the first patient malignant tissue was unexpectedly encountered during transsphenoidal surgery. Anatomopathological investigation confirmed the presence of a metastasis of a breast carcinoma for which she had been treated 17 years earlier. In the second patient, a preoperative chest X-ray before transsphenoidal surgery revealed an asymptomatic bronchial tumour. Subsequently a squamous cell carcinoma with a metastasis in the pituitary was confirmed. These two cases illustrate the fact that a pituitary metastasis can closely mimic a pituitary adenoma. Even in the absence of suggestive symptoms such as diabetes insipidus and/or cranial nerve paralysis the possibility of metastatic disease in the differential diagnosis of a pituitary mass should always be considered.

High cervical pain and impairment of skull mobility as the only symptoms of an occipital condyle fracture. Case report.

Occipital condyle fractures are rarely reported in survivors of trauma. Most cases involve patients with a major head trauma, lower cranial nerve palsy, and/or suspected lesions demonstrated on plain x-ray films of the skull or cervical spine. The authors describe a traffic-accident victim in whom an atlanto-occipital joint lesion was suspected based only on mobility investigation of the skull. Axial high-resolution computerized tomography of the atlanto-occipital joint showed a fracture of the right occipital condyle.

Localized craniofacial fibrous dysplasia in a young girl.

We consider here the case of a young girl presenting a cystic bone mass in the left orbital roof. Although standard X-rays at first were not convincing, the findings on the CT examination and the histological aspect of the mass were typical of fibrous dysplasia. Although the exact diagnosis of fibrous dysplasia can only be made upon precise histological examination, radiological, and more specifically CT findings, may indicate this pathology.

Spinal angiomas as a cause of non-traumatic paraplegia: case report.

A case is presented of a 17-year-old boy who developed paraplegia after a sudden low back pain, like a 'stab in the back', with radicular radiation into the lower limbs. No trauma preceded the onset of symptoms. The diagnosis was finally made by neuroradiological investigations (magnetic resonance imaging (MRI), myelography and CT-scan). A review of the physiopathology of spinal angiomas, extra and/or intramedullary, by exclusion of vertebral (haem) angiomas, is made.

Posttraumatic diabetes insipidus.

The authors report seven patients with fractures of the sella turcica. Four of them developed Diabetes Insipidus as a consequence of their traumata. The importance of computed tomography as a diagnostic procedure is paramount in order to make early diagnosis of this potential life-threatening condition possible.

Bulbo-spinal lower motor neuron disease. Accumulation of neurofilaments in perikarya and axons.

Two sporadic adult cases of bulbo-spinal lower motor neuron disease are reported. In the first patient, the disease lasted 8 months and was characterized by a bulbar onset followed by a progressive cephalocaudal involvement of the lower motor neurons in the spinal cord. In the second case of 14 months duration, the cervical spinal cord was affected at first while medulla oblongata and lower limbs were involved later on. There was a slight increase of the protein contents in the CSF. Postmortem examination confirmed the selective involvement of the lower motor neurons in medulla oblongata and spinal cord with severe loss at cervico-medullary level in case 1 and more diffuse loss in case 2, in keeping with the clinical signs. Bodian silver staining and electron microscopy showed the accumulation of neurofilaments in anterior born cells' perikarya and in proximal axonal dilatations. The nosology of the disorder and the comparison with lesions found in various types of motor neuron disease in humans and animals are discussed.

Study of the pituitary-thyroid functions at high altitude in man.

The alterations in serum levels of T3, T4, TSH and TBG, TSH response to 100 mug iv TRH, and urinary excretion of T3 and T4 were studied in 8 healthy men at sea level (SL), on days 1, 2, 4, 8 and 16 after arrival by air at high altitude (3,700 m, HA), and during days 5 to 7 after their return to SL. No significant alterations in serum levels of TSH and TBG or TSH response to TRH were observed during exposure to HA or on return to SL. There was, however, an acute elevation in both serum total T3 and T4. Serum total T3 from a mean basal+/-SE value of 128+/-13 ng/dl increased to 320+/-18 on day 1 and remained significantly elevated at 225+/-48 up to day 8 after arrival at high altitude. Similarly serum total T4 increased from basal level of 9+/-0.92 mug/dl to 15.2+/-1.2 and remained elevated till day 16 and it was 11+/-1.19 mug/dl during days 5 to 7 after return to SL. The urinary excretion of both T3 and T4 was decreased. These changes perhaps were the result of complex physiologic adjustments on acute exposure to high altitude, like shrinkage of the T3 and T4 distribution pools, altered binding capacities of thyroid hormones binding proteins, and a reduction in clearance of thyroid hormones from the plasma compartment; and were probably not suggestive of an enhanced thyroid activity. Their actual significance in high altitude adaptation in man is not clearly understood.

Responses of the autonomic nervous system during acclimatization tp high altitude in man.

A study has been conducted on 20 sojourners, between the ages of 20-30 years, to evaluate responses of the autonomic nervous system during acclimatization to high altitude. The responses measured consisted of heart rate (HR), blood pressure (BP), oral temperature (Tor), mean skin temperature (Tsk), cold pressor response (CPR), orthostatic tolerance to tilt, and urinary catecholamines. The subjects were tested initially at Delhi (altitude 260 m) and thereafter, on acute induction to an altitude of 3500 m periodically for 3 weeks. For comparison, the same responses were studied on 10 acclimatized lowlanders (AL) who had been staying at the same altitude for more than a year and on 10 high-altitude natives (HAN). The studies showed a rise in HR, BP, Tor, and urinary catecholamines, and a fall in Tsk, CPR, and orthostatic tolerance immediately on arrival at HA, indicating a relative hyperactivity of the sympathetic system. After a stay of 1 week, there was a gradual recovery in all the responses, though sympathetic hyperactivity was still maintained throughout the 3 weeks of stay. In AL also there was a preponderance of sympathetic activity, though of relatively lesser magnitude than that seen in sojourners. In HAN, on the other hand, there was a relative parasympathetic predomination. It has been concluded that in lowlanders it takes more than a year of stay at altitude for complete recovery of autonomic balance.

Changes in exercise heart rate in lowlanders after prolonged stay at high altitude (4000 m).

Studies were conducted on cardiac frequency during submaximal and maximal work on 26 sea-level residents prior to transfer to and during stay at high altitude for 1, 10, and 20 months. Maximal O2 uptake and performance in a 1.6 km run were observed. Results indicated a significant drop in Vo2 after arrival at altitude followed by recovery with further stay. The mean maximum heart rate decreased to 182.8 beats/min after 1 month at high altitude from a seal-level mean value of 188.4 beats/min. It increased to 199.2 beats/min and decreased to 185.6 beats/min after 10 and 20 months, respectively. Heart rate, during submaximal work requiring 1.0 and 1.5 102/min, indicated the highest rate after 1 month at altitude and decreased with prolonged stay, but remained higher than the sea-level value. These changes were compared with the high-altitude native residents.