Ram Menon

Hemangioblastoma of the filum terminale.

A 52-year-old man presented with low backache, paraesthesiae and spasticity of both lower limbs. He had urinary retention and constipation. Investigations revealed a vascular intradural cauda equina-conus tumor. MRI scan demonstrated an enhancing mass at the second and third lumbar vertebral levels. There were multiple dilated and tortuous veins draining from both poles of the tumor. Digital subtraction spinal angiogram showed the tumor to be supplied mainly by the radiculo-medullary artery from first lumbar artery and dural branches of the second and third lumbar arteries. At surgery, after pre-operative embolization, a well-defined tumor with an orange hue and fleshy consistency was encountered arising from the filum terminale. The tumor was excised en bloc. A sporadic hemangioblastoma arising from the filum terminale should be considered as a rare cause of back pain and sciatica. Total excision of the tumor offers cure.

Ossified ligamentum flavum of the atlantoaxial region

A 30-year-old male presented complaining of a six-month history of progressive weakness and paraesthesia in all four limbs. Symptoms occurred following moderately severe neck trauma. Investigations revealed ossification of the ligamentum flavum (OLF) between the atlas and axis, with marked cord compression. The patient showed remarkable neurological recovery following excision of the OLF. OLF causing cord compression is rare and has never been reported in the atlantoaxial region.

Chudley McCullough syndrome

BackgroundChudley McCullough syndrome is characterized by partial agenesis of the corpus callosum, interhemispheric cyst, cerebral and cerebellar cortical dysplasias, and hydrocephalus. This syndromic form of sensorineural hearing loss is rare. Our literature search has located 13 siblings in 6 families with this syndrome. We report a case of Chudley McCullough syndrome and discuss the relevant literature. It is amply clear from the review of literature that treatment of ventricular dilatation or drainage of arachnoid cysts in these cases will not improve the sensorineural hearing loss.Case IllustrationA 14-month-old female child presented with bilateral profound sensorineural hearing loss. Neuroimaging revealed partial agenesis of the corpus callosum, colpocephaly, and an interhemispheric arachnoid cyst. These associations suggested a diagnosis of Chudley McCullough syndrome.

Cranial migration of complete ventriculo-peritoneal shunt assembly

A 10-month-old male child with severe congenital hydrocephalus due to aqueduct stenosis presented with cranial migration of the entire ventriculo-peritoneal (VP) shunt. The complete shunt assembly, including the shunt chamber, was noted in the dilated ventricles. The migrated shunt was left in situ. A VP shunt was performed on the opposite side. The complete intraventricular migration of a VP shunt is a rare complication. This complication may be avoided by firm anchoring of the connector sites to the periosteum, and avoidance of large burr holes and dural openings. The possible mechanisms of such an event and the relevant literature are discussed.

Cerebellar oligodendroglioma in a young adult.

A rare case of an oligodendroglioma of the cerebellum in an 18-year-old male is presented. Radical surgical excision and craniospinal radiotherapy appear to be the treatment options for these potentially malignant lesions.

Dandy-Walker malformation associated with polycystic kidneys: Goldston syndrome revisited

A 32-year-old woman was diagnosed with chronic bilateral frontoparietal and interhemispheric subdural haematomas (SDH). Abdominal ultrasonography revealed chronic renal failure due to bilateral large cystic kidneys. Brain MRI demonstrated Dandy-Walker malformation associated with hypoplastic vermis, in addition to the chronic subdural haematomas. This association of polycystic kidneys with Dandy-Walker malformation has been referred to as Goldston or cerebrorenal syndrome. The case is rare and is the first to be reported in an adult patient. The relevant literature is reviewed.

Segmental costovertebral malformation associated with lipomyelomeningocoele

We describe 2 patients with segmental costovertebral malformation, a form of spondylocostal dysostosis, associated with tethering of the conus to a lipomyelomeningocoele. Such an association is rare. In both these patients the defects occurred sporadically. The relevant literature is reviewed.

Blood-fluid level in cortical venous thrombosis—a rare diagnostic entity

BACKGROUND With improvement in imaging, CVT is being diagnosed more frequently. Early diagnosis and rapid institution of remedial measures such as anticoagulation, anticonvulsants, hydration, and treatment of underlying abnormality will aid in salvaging life. CASE DESCRIPTION We report an autopsy case of a 34-year-old man who presented with sudden onset of seizures, followed by right hemiplegia and altered sensorium. Magnetic resonance imaging of the brain revealed intraparenchymal hemorrhage in the left posterior frontal region with a blood-fluid level. The SSS was devoid of the routine hypointense signal intensity. Routine evaluations for hypercoagulable states were negative. All the coagulation parameters were within normal limits. He was managed conservatively with anticonvulsants, anticoagulants, and hydration. A CT scan of the brain done 24 hours later revealed left posterior frontal hemorrhage with perilesional edema. A CT venogram confirmed the presence of CVT involving the SSS and the right transverse sinus. However he died within next 24 hours. An autopsy confirmed thrombosis in the SSS, right transverse sinus, lungs, and kidneys. CONCLUSIONS Blood-fluid level in CVT may be an early radiologic sign in the absence of any other imaging abnormality. The presence of this sign in the early stages warrants an aggressive treatment because this probably suggests a large bleed with raised capillary and venous pressures. The case is discussed in view of the unusual radiologic sign in CVT in the absence of anticoagulant therapy.

Unilateral atlantal mass hypertrophy in acromegaly. Case report.

The authors report an extremely rare case of a patient with acromegaly who had unilateral enlargement of an atlas facet resulting in cord compression and progressive quadriparesis. Although unilateral atlas facet enlargement has been identified in the literature in cases of spondylosis and as a component of congenital malformation, its association with acromegaly has not previously been reported. Resection of the offending facetal bone and atlantoaxial fixation resulted in rapid neurological recovery.

Spontaneous cerebrospinal fluid rhinorrhea following excision of a massive torcular meningioma

A massive supratentorial torcular meningioma was completely resected in a 50-year-old woman. On the tenth postoperative day she presented with spontaneous cerebrospinal fluid (CSF) rhinorrhea. The site of the CSF leak was through the region of the cribriform plate, remote from the site of surgery. The possible cause of the leak and the relationship with the massive meningioma are analyzed.