Suprava Naik

Anterior spinal artery aneurysm in a case of Moyamoya disease

Aneurysm of spinal artery is rare but can be found in association with spinal cord arteriovenous malformation, coarctation of aorta, arteritis and vasculitis. Early diagnosis and management of this potentially treatable condition is necessary to prevent associated morbidity and mortality. Here, we report a rare case of Moyamoya disease (MMD) presented with intraventricular hemorrhage due to rupture of anterior spinal artery (ASA) aneurysm.

Anterior spinal artery aneurysm in aortic stenosis of different etiology: Report of three cases

Isolated aneurysms of spinal arteries are rare. Spinal artery aneurysms are commonly found in association with spinal cord arteriovenous malformation and coarctation of aorta and rarely with aortic arch interruption and Klippel-Trenaunay syndrome. Spinal angiograms are the gold standard for diagnosing these spinal artery aneurysms but with the advances in computed tomography technology these aneurysms can also be very well demonstrated in computed tomography angiograms. We describe three cases of anterior spinal artery aneurysm, those are flow related aneurysms, associated with coarctation of aorta and with Takayasu arteritis.

Delayed onset dancing hand syndrome following thalamic hemorrhage

Dear Editor, Unilateral chorea or hemichorea is a form of hyperkinetic movement disorder. It is usually associated with vascular causes, such as cerebral infarctions, arteriovenous malformations, and subdural hematomas [1]. These movements usually occur within the first week of strategic stroke when the patient starts recovering from the acute stroke. Delayed onset occurrence of hemichorea has not been described. In this communication, we report a case of hemichorea which developed after 7 months of thalamic hemorrhage and its management. A 53-year-old female presented to our department with history of involuntary movement of right upper limb of 1-month duration. Themovement was sudden in onset, non progressive, low amplitude, coarse in nature, typically involving distal part of right upper limb. It was persisting throughout the day, more prominent during activity and disappeared during sleep. There was associated twitching movement of right angle of jaw. She is a known hypertensive from 8 months; hypertension was detected when she developed acute onset right hemiplegia, medical research scale (MRC) grade 2/5. Non-contrast CTscan at that time demonstrated acute left thalamic hemorrhage with intraventricular extension (Fig. 1). Her motor power improved to MRC grade 4/5 in the next 2 months and was independent on activity of daily living with residual mild spasticity and post stroke pain, visual analogue scale 5/10. She had history of right Bell’s palsy 20 years before without residual deficit. Family history was not significant. On examination, blood pressure was 130/90 mmHg, vitals were normal, she was conscious and oriented, and speech was spastic. Motor examination revealed hypertonia and hyperreflexia of right limbs. Right hand grip was 80% of normal; power was MRC grade 4/5 on right side with no new motor deficit. Gait and sensory examination was normal. There was an irregular, coarse, continuous, nonsuppressible, quasi-purposive involuntary movement that was more prominent on action involving the right hand and forearm which were suggestive of right hemichorea (HC) (video 1). In view of new-onset hyperkinetic movement (HC) on right half without new focal motor deficit in a patient of 8 months old left thalamic hemorrhage possibility of post stroke seizure or new-onset lacunar stroke was considered. Video EEG did not reveal any seizure activity corresponding to site of HC. Magnetic resonance imaging of brain (Fig. 1) showed an irregular T2 hyperintense lesion in the left thalamus with a hypointense rim likely to be due to hemosiderin deposition. The lesion was showing blooming in susceptibility-weighted image suggesting the presence of blood degradation products. The same area shows hypoperfusion in perfusion map. No new lesion was seen on diffusion-weighted MRI to suggest acute infarct. The lesion was hypointense on T1WI without obvious post contrast enhancement. No evidence of mass effect noted on adjacent brain parenchyma. MR angiogram was normal. Routine hemogram; liver, renal, and thyroid function tests; and lipid profile were normal. Fasting and postprandial blood glucose were normal, serum sodium was 134 meq/L, glycosylated hemoglobin was 6.5. ECG, and 2D echocardiography was normal. Serological testing for HIV, HBsAg, HCV, and VDRL was negative. ESR was normal; serum antinuclear antibodies were negative. She was started on tetrabenazine (25 mg) twice daily and her antihypertensive medication was continued. There was improvement in his involuntary movements and was discharged on day 5 (video 2). On 1month follow-up, her hemichorea subsided. Our patient demonstrated late-onset occurrence of hemichorea after 7 months of thalamic hemorrhage. Unilateral choreiform movements are called hemichorea, which accounts for Electronic supplementary material The online version of this article (https://doi.org/10.1007/s10072-018-3549-1) contains supplementary material, which is available to authorized users.

A Vasculitis-Associated Neuromuscular and Vascular Hamartoma Presenting as a Fatal Form of Abdominal Cocoon

Neuromuscular and vascular hamartoma (NMVH), also known as neuromesenchymal hamartoma, is a rare hamartomatous condition of the intestine. It usually presents with submucosal humps protruding in the intestinal lumen causing obstructive features. The other clinical manifestations are hematochezia or melena and protein-losing enteropathy. The etiopathogenesis of these lesions is not well known, although an association with small bowel Cröhn’s disease and diaphragm disease had been postulated, the latter related to chronic nonsteroidal anti-inflammatory drug intake. Only 24 cases of NMVH are reported in the English literature and all of them could be adequately cured by resection of the affected part of the bowel. Moreover, none of these cases presented with abdominal cocoon or showed any evidence of vasculitis. We describe a peculiar case of NMVH in a 45-year-old male who presented with abdominal cocoon with symptomatic recurrence and fatal outcome within a month of surgery. Histopathology revealed classical histomorphology of NMVH with evidence of vasculitis. This appears to be the first case of a fatal form of NMVH, presenting with abdominal cocoon and associated with vasculitis.

Periureteral inferior vena caval venous ring presenting as urinary obstruction

The embryological development of the inferior vena cava (IVC) is complex, and thus the vena cava may undergo a large number of congenital anomalies. Periureteric venous ring is a rare developmental anomaly of IVC where the right ureter passes through a slit-like opening in a partially duplicated infrarenal IVC, resulting in dilatation of upper urinary tract. Split-bolus multidetector computed tomography technique is useful in detecting such vascular anomaly causing ureteric obstruction as it can clearly show the vascular and ureteric phase in a single acquisition.

Role of MR spectroscopy in diagnosis of intracranial neuroenteric cyst

IntroductionNeuroenteric cysts are rare benign endodermal lesions of the central nervous system that result from incomplete resorption of neuroenteric canal and mostly found in cervical and upper thoracic spinal canal. Intracranial neuroenteric cysts are extra axial and commonly located anteriorly in the posterior cranial fossa. MRI demonstrates variable intensity within the lesion on T1, T2W, and DWI sequences.MethodsThree cases of posterior fossa non-enhancing cystic lesions of variable signal intensity underwent MRI with MR spectroscopy, where MR spectroscopy demonstrated dominant peak at 2 ppm, mimicking normal Nacetyl aspartate (NAA).ConclusionMR spectroscopy in addition to conventional MRI may help in differentiating intracranial neuroenteric cyst from its close differentials.

Dynamic contrast-enhanced magnetic resonance imaging in diagnosis of cavernous hemangioma of cavernous sinus

Sir, Cavernous hemangiomas of the cavernous sinus (CHCS) comprise <1% of all parasellar masses and closely mimic tumors such as meningioma, pituitary macroadenoma, or schwannomas at this site.[1] Although magnetic resonance imaging (MRI) can help in diagnosis, addition of dynamic contrast‐enhanced sequence to conventional MR sequences demonstrates the characteristic gradual “filling in” of the lesion and helps in preoperative diagnosis with a better accuracy, thus guiding appropriate surgical approach and reducing the associated perioperative morbidity.

Transdiscal fracture of thoracic spine presenting as sensory ataxia

Transverse fracture of spine is rare; however it is a wellknown complication of ankylosing spondylitis and diffuse idiopathic skeletal hyperostosis (DISH). These fractures may remain undetected or present with neurological complication. MRI is very sensitive for detection of such fractures and associated neurological complications. Here, we describe a case of transdiscal fracture of thoracic spine presenting with sensory ataxia in a previously unrecognised DISH.