A rare case of dural arteriovenous fistula presenting as primary intraventricular hemorrhage.

Primary intraventricular haemorrhage (PIVH) is rare. Dural arteriovenous fistula causing PIVH is extremely rare. We report a case of a 17 year old boy who presented with left hemiparesis, left lower motor neuron facial palsy and ataxia. His computed tomography head revealed primary intraventricular hemorrhage. Catheter super selective angiography revealed a dural arterio venous fistula with arterial feeder arising from the middle meningeal artery as well as from the inferior marginal tentorial artery. Glue injection led to successful disappearance of the fistula and eventual clinical recovery.

Kleine-Levin syndrome with comorbid iron deficiency anemia.

Kleine-Levin syndrome (KLS) is a rare chronic sleep disorder of unknown etiopathology, which typically occurs in adolescent males. Although the severity of symptoms and disease course varies between the KLS patients, it usually resolves spontaneously, but sometime comorbid conditions may worsen the symptoms. Herein, we report a case of KLS who presented with severe episodic hypersomnia. During episodes, the patient used to sleep as long as 20 h in a day, affecting his daily living activities. All the relevant investigations including electroencephalography, magnetic resonance imaging of brain and cerebrospinal fluid analysis were normal except for severe iron deficiency anemia (IDA). In our patient, the severity of symptoms worsened due to coexistent IDA. The treatment of IDA along with modafinil decreased the severity of symptoms and shortened the hospital stay during episodes. This might be the first case report of KLS with comorbid IDA.

Clinical and Radiological Outcome in Cases of Posterolateral Fusion with Instrumentation for Lumbar Spondylolisthesis.

INTRODUCTION: Lumbar Spondylolisthesis as a cause of low back pain and lower limb radiculopathy has been treated using varied surgical options. The role of laminectomy for decompression of neural elements and stabilization using instrumentation in the form of pedicle screws and rod construct has been a well-established and time tested treatment modality. AIM AND OBJECTIVES: This study analyses the role of laminectomy and instrumentation in obtaining clinical and radiologically favourable outcome. MATERIALS AND METHODS: Data was analysed from the case records for the duration from January 2010 to March 2014. The study analyses the influence of lumbar decompression (laminectomy) and transpedicular instrumentation using titanium pedicle screws and intertransverse process iliac crest graft on patients with degenerative lumbar spondylolisthesis and spinal stenosis. CONCLUSION: Decompression primarily relieves radicular symptoms and neurogenic claudication whereas fusion primarily relieves back pain by elimination of instability. The addition of posterolateral instrumentation (pedicle screws) enhances the ability to obtain a solid arthrodesis. Posterolateral instrumentation enables improved functional outcome, better patient satisfaction and less back and lower limb symptomatology. This is irrespective of bony arthrodesis or pseudoarthrosis, at least in the short term follow-up.

Safe, Effective and Easily Reproducible Fusion Technique for CV Junction Instability.

INTRODUCTION: The Craniovertebral junction (CVJ) refers to a bony enclosure where the occipital bone surrounds the foramen magnum, the atlas and the axis vertebrae. Because of the complexity of structures, CVJ instability is associated with diagnostic and therapeutic problems. Posterior CV fusion procedures have evolved a lot over the last couple of decades. There has been a lookout for one such surgical procedure which is inherently safe, simple, easily reproducible and biomechanically sound. In our study, we present the initial experience the cases of CV junction instrumentation using O-C1-C2 screw & rod construct operated by the author. AIMS AND OBJECTIVES: The current study is a descriptive analysis of the cases of CVJ instability treated by us with instrumentation using O-C1-C2 screw and rod construct fusion technique. MATERIALS AND METHODS: It is a retrospective, analytical study in which cases of CV junction instability operated by the author between January 2010 to March 2014 were analysed using various clinical, radiological and outcome parameters. CONCLUSION: CV junction instrumentation using O-C1-C2 screw and rod construct fusion technique proved to be safe, effective, easily reproducible and biomechanically sound technique which can be adopted by all surgeons who may be at any stage of their learning curve.

An institutional experience of 26 patients with Moyamoya disease: A study from Northwest India.

AIM: Moyamoya disease (MMD) is a slowly progressive bilateral stenocclusive process of the distal internal carotid and proximal portions of the anterior and middle cerebral arteries and the formation of an abnormal vascular network at the base of the brain. The purpose of this retrospective study was to identify clinical features, salient features, radiological features and yield of diagnostic cerebral angiography in MMD. MATERIALS AND METHODS: We analyzed the records of 26 patients with MMD evaluated and treated at our institute from August 2010 until March 2013. Diagnosis of MMD was made on the basis of features of angiographic findings. Cerebral angiography showed typically fine network of vessels at the base of the brain with puff of smoke appearance suggestive of MMD. CT angiography (CTA) was done in 25 (96.15%) patients where as Digital substraction angiography (DSA) was done in 18 (69.23%) patients. RESULTS: Out of the 26 patients 13 were in the pediatric age group and 13 were adults. At presentation 14 patients had infarcts and 10 patients had hemorrhages. Among the hemorrhagic group 20% had isolated intracerebral hemorrhage (ICH), 50% patients had ICH with intraventricular extension (IVE) and 30% patients had primary intraventricular hemorrhage (PIVH). 50 % of the patients had involvement of the posterior circulation. CONCLUSION: Posterior circulation involvement is frequent in MMD. Though parenchymal bleed with/without intraventricular extension is the usual presentation of hemorrhagic MMD, isolated intraventricular hemorrhage could also be the mode of presentation.

Primary intraventricular hemorrhage: clinical features, risk factors, etiology, and yield of diagnostic cerebral angiography.

BACKGROUND: Primary intraventricular hemorrhage (PIVH) is a rare neurological disorder, with bleeding confined to the ventricles only, without recognizable parenchymal or subarachnoid component. AIM: The purpose of this retrospective study was to identify clinical features, predisposing risk factors, etiology, radiological features and yield of diagnostic cerebral angiography in identifying the etiological causes. SETTINGS AND DESIGN: Records of patients admitted in neurology division were analyzed in a tertiary care teaching hospital. MATERIALS AND METHODS: We analyzed the records of 27 patients with PIVH evaluated and treated at our institute from August 2010 to April 2013. PIVH was diagnosed as hemorrhage in the ventricles only, detected by computed tomography scan without evidence of intraparenchymal, subarachnoid hemorrhage or intraventricular hemorrhage associated with trauma. CT angiography (CTA) alone was done in 10 patients (37.03%), digital subtraction angiography (DSA) in 2 patients (7.4%) and both CTA as well as DSA was done in 15 patients (55.5%). STATISTICAL ANALYSIS USED: Categorical and continuous data were analyzed using SPSS version 17. RESULTS: 17 (62.96%) patients were females and 10 (37.03%) were males with ratio of F:M= 1.7:1. Headache was the commonest mode of presentation (85.18%). Hypertension was most common predisposing factor (29.62%) followed by arterio-venous malformations (AVMs) (25.92%), moyamoya disease (MMD) (11.11%), lenticuostriate artery aneurysm (LSA) (11.11%), arterial dissections (7.4%) and dural arteriovenous fistula (dAVF) (3.7%). CONCLUSIONS: PIVH is rare and hypertension is important predisposing factor. Yield of cerebral angiography is high in diagnosing the etiology. AVMs and other rare etiological causes like MMD, LSA aneurysm, arterial dissection, and dAVF should be kept in mind with a high index of suspicion and warrants cerebral angiography in them, as some of the causes are potentially treatable.

Scalp fibroma: a rare cutaneous manifestation of tuberous sclerosis.

We report a case of a 23-year-old woman with a history of generalised tonic-clonic seizures, reddish brown maculopapular swelling over the face and an enlarging swelling over the scalp. Physical examinations revealed angiofibroma of the face and other typical cutaneous lesions of tuberous sclerosis, for example, shagreen patch and periungual fibroma. Scalp swelling was labelled as fibroma by dermatologists, which was further supported by the histopathological findings. Fibroma of the face is one of the commonest lesions, however, fibroma of the scalp is a rarely described entity.

Lenticulostriate artery aneurysm presenting as primary intraventricular haemorrhage.

Isolated or primary intraventricular haemorrhage (PIVH) is rare and usually caused by hypertension, vascular malformations, aneurysms and moyamoya disease. Lenticulostriate artery aneurysm (LSA) is also a rare entity, and LSA aneurysm rupture causing primary intraventricular haemorrhage is extremely rare. There are only a few case reports. LSA aneurysms can occur medially or laterally, proximally or distally, though distal lateral LSA aneurysms are more common. They are usually associated with various vascular conditions. We report two rare cases of LSA aneurysms presenting as PIVH, one being medial and other being lateral distal LSA aneurysm. Both were normotensives, and one was associated with arteriovenous malformation.

Combination of Steven-Johnson syndrome and neuroleptic malignant syndrome following carbamazepine therapy: a rare occurrence.

Stevens-Johnson syndrome (SJS) is a severe, episodic, acute mucocutaneous reaction that is most often elicited by drugs and occasionally by infections. The drugs commonly implicated as the cause of SJS are anticonvulsants, sulfonamides, non-steroidal anti-inflammatory drugs and antibiotics. Carbamazepine (CBZ) has been commonly implicated in SJS. Neuroleptic malignant syndrome (NMS) is a rare, life-threatening but potentially treatable condition. Among the neuroleptics, haloperidol (parenteral) is implicated as a most common drug for NMS. Though rare, association of NMS with CBZ and association of NMS with toxic epidermal necrolysis (TEN) in a single patient after administration of neuroleptics has been reported in the literature before. However, a combination of NMS and SJS in a single patient after administration of CBZ has not been reported so far. We present a patient with seizure who developed SJS and NMS following administration of CBZ.

Ophthalmoplegic migraine with trigeminal nerve involvement.

We report a 25-year-old man with a history of uncontrolled migrainous headaches who developed third nerve palsy and sensory loss over V1 distribution of trigeminal nerve, during an attack of severe migraine. Gadolinium-enhanced MRI of the brain and cavernous sinus was normal and did not disclose nerve enhancement. CT angiogram was also normal. The patient recovered uneventfully in 2 weeks on oral steroids. The commonest cranial nerve implicated in ophthalmoplegic migraine is the occulomotor nerve. Involvement of the fifth nerve has never been reported.

Erdheim-Chester disease with isolated craniocerebral involvement.

Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis with distinctive radiographic and pathological features. Intracranial involvement is further a rarity, usually producing diabetes insipidus or cerebellar-brainstem symptoms. We report a 40-year-old man presenting with recurrent secondarily generalised seizures. An MRI scan of the brain revealed multiple enhancing intracranial masses in frontal, temporal and parietal regions. Biopsy from the left frontotemporal lesion confirmed it to be a rare case of ECD. The patient received a short course of corticosteroids initially and subsequently remained well-controlled on antiepileptic therapy alone. A repeat MRI of his brain showed significant resolution of lesions. Osteolytic lesions in the skull vault were detected during follow-up which also disappeared. Interestingly, there was no involvement of long bones or any other system even after 12 years of follow-up.

A rare case of alcoholic pellagra encephalopathy with startle myoclonus and marked response to niacin therapy: time for a new dictum?

We report a case of 56-year-old man, chronic alcoholic, presented to us with progressive weakness in all the four limbs with stiffness and gait disturbance since 1-year associated with cognitive impairment. On examination he had mild confusion, spastic quadriparesis with brisk reflexes, extensor plantars and cerebellar features. During the hospital stay myoclonus was noticed in the patient, which was startle in nature. He did not have dermatitis, ascites or any stigmata of liver failure. MRI of brain revealed bilateral subdural effusion, left focal subarachnoid haemorrhage at perisylvian area and diffuse cortical atrophy. He was treated with supportive measures including thiamine with which his condition worsened. His serum niacin was low. With a possibility of alcoholic pellagra encephalopathy (APE) the patient was treated with niacin. His clinical condition improved drastically over next 1 week and startle myoclonus disappeared, favouring the diagnosis of APE though multiple confounding factors were present.

'Hot cross bun' sign in a case of cerebrotendinous xanthomatosis: a rare neuroimaging observation.

We report a 25-year-old young man presenting with cognitive decline, pancerebellar features, spastic quadriparesis, bilateral cataract (operated) and tendo-Achilles swelling (xanthoma). The CT of the head showed bilateral cerebellar hypodensities. There were bilateral cerebellar hypointensities involving dentate nuclei on T1-weighted images with corresponding hyperintensities on T2-weighted MRI. Additionally, an interesting MRI finding-'hot cross bun' appearance was seen in pons which has not been reported in the literature so far. Biopsy from tendo-Achilles confirmed xanthoma. He was treated with chenodeoxycholic acid following which he showed improvement in cognition and weakness.

Wrist clonus mimicking as action-induced tremors: an important clinical lesson.

We report a 35-year-old man who presented with abnormal movement of hand upon extension at wrist joint in an outstretched arm. He was diagnosed and treated elsewhere as having action-induced tremors. On examination, he had action-induced clonus at wrist joint with bilateral corticospinal tract features in the form of weakness, spasticity and extensor plantars. Evaluation also revealed cervical compressive myelopathy. The purpose of presenting this case report is to highlight this rare clinical sign which can be mistaken for action-induced tremors.