Formation of hyaline bodies in hyaline body myopathy is associated with either myolysis or defective incorporation of heavy chain slow myosin into the cytoskeleton. Hyaline bodies very likely contain additional unidentified proteins. Neurogenic factors are also involved in the hyaline body myopathy pathogenesis.

The differential diagnosis of a sellar lesion includes pituitary adenoma, cranio-pharyngioma, tumour cyst, benign cyst, and other less common lesions such as aneurysm, squamous cell carcinoma and metastases. Pure cystic lesions within the sella turcica are not uncommon and may appear clinically and radiologically as pituitary adenoma. Intrasellar cysts are broadly classified as neoplastic or non-neoplastic; the latter may be primary lesions of the pituitary fossa or they may arise from the parasellar region and invade into the sella. The lesions occupy space and may cause disturbance of pituitary function; if they extend into the suprasellar region they can affect visual fields and acuity. Non-neoplastic cystic lesions usually appear well defined radiologically and have distinct pathological features. Modern imaging modalities, such as computed axial tomography and magnetic resonance imaging, are important complementary tools of investigation for reaching a differential diagnosis, and for surgical planning. Most non-neoplastic sellar cysts can be approached through the trans-sphenoidal route, but large lesions with suprasellar extension may require transcranial surgery. We report the presentation and management of eight cases (four males, four females, all adult) of non-neoplastic cyst involving the sella turcica and the parasellar region. Investigations included computed tomography scan of the head for all and magnetic resonance imaging for six (75%) patients. Work-up included endocrinological and opthalmological evaluations. All presented with headache; six (75%) had visual acuity change, two (25%) had evidence of visual field defects, four (50%) had optic atrophy on fundoscopy, three (37.5%) had endocrine symptoms and hormone reduction was found in four (50%).

The authors describe four siblings from consanguineous parents who presented with oculomotor deficit in early childhood characterized by impaired volitional horizontal saccades, compensatory lateral head thrust, and preservation of vertical movement. When about 10 years of age, heavily calcified aortic and mitral valves required surgery. Fibroblast beta-glucocerebrosidase activity was markedly reduced. Genotype analysis indicated that the two patients who were tested were homozygous for the D409H (1342G-->C) mutation. Relating this rare phenotype of Gaucher disease to D409H mutation will facilitate management of the disease and counseling of families.

The aetiological spectrum of sphenoid sinus mucocele includes congenital anomaly, trauma, infection, allergy and surgery of the sphenoid sinus. Enlargement of the mucocele, even with a short history, can result in progressive expansion of the sinus and extension of the lesion into the pituitary fossa, the suprasellar region, nasopharynx, orbits, clivus or ethmoid air cells. It is a benign cystic lesion with an excellent prognosis when treated appropriately. Generally, these lesions are managed by an ear, nose and throat surgeon, but when there is extension into the sellar and parasellar (especially suprasellar) regions they are managed by the neurological surgeon. Sphenoid sinus mucocele should be considered in the differential diagnosis when there is suspicion of a cystic lesion in these regions. Three cases of large sphenoid sinus mucocele are presented, with discussion on their neurosurgical management and a review of the literature.

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