ABSTRACTS Papers for BC Roy Gold Medal Award

ABSTRACTS
Papers for BC Roy Gold Medal Award
1.ANTI TPO RELATED ENCEPHALOPATHY
Synmon Baiakmenlang , Basumatary LJ*, Das M**, Goswami M ***, Kayal A K ***.
Department of Neurology, Guahati Medical College and Hospital, Guwahati, India
INTRODUCTION:
Presence of antithyroid peroxidase antibody (anti TPO) in a patient of encephalopathy or other related
clinical features brings a possibility of autoimmune encephalopathy classically known as Hashimoto
encephalopathy (HE), but this antibody may be found in cases not typical of HE. Response to steroids
clinches its diagnosis; hence it has been labeled as ‘steroid responsive encephalopathy’ (SRE).
OBJECTIVES:
To study clinical manifestations who presented with encephalopathy with positive anti TPO antibodies,
and response to steroids.
METHODS:
Observational study conducted from September 2013 – September 2014
Detailed neurological evaluation, biochemistry, thyroid function test, anti TPO level, CSF study, EEG
and neuro-imaging were performed. Patients were treated with steroid and followed up.
RESULTS:
Total 10 patients were enrolled. The age of presentation ranged from 4th to 7th decade, with male
predominance (7:3). Clinical manifestation included dementia (4), behavioral abnormality (4), delirium
(4), myoclonus (2), sleep disturbance (2), seizure (1) and depression (1). Pyramidal & extrapyramidal
features were observed in 3 & 1 patient respectively. Regarding thyroid status, hyperthyroid (2),
hypothyroid (1), euthyroid (7) and active thyroiditis was found in 2 patients. The highest level of anti
TPO found was 1300 in serum. Steroid (pulse therapy) was given to 7 patients with a good response in 5
patients and a relapse in two. Based on Chong criteria, 4 patients were labeled as HE and the others as
anti TPO related encephalopathy.
CONCLUSION:
All anti TPO positive encephalopathy do not classically fit as HE, but should not be missed out as many
of them shows a dramatic response to steroids.
Key words:
Anti TPO, Hashimoto encephalopathy, steroid responsive encephalopathy
TITLE : “ Clinical features of Huntington’s disease and correlation of clinical features
with genetic study in tertiary care centre in Eastern India”
Dr Zakir Hussain, PG Student,IGPMER SSKM Hospital Kolkata
INTRODUCTION : Huntington’s disease HD is a progressive degenerative disorder of the
central nervous system with autosomal dominant inheritance characterized by involuntary
movements, cognitive impairment and behavioral disturbances. HD is associated with an
expanded sequence of CAG repeats on the IT15 gene that is located on chromosome 4.
Materials and methods : Herein, we report clinical data on 20 genetically confirmed HD
patients from 17 Indian families predominantly from North East India. Clinical data and
evaluation was performed using standardized formats used by the Huntington Disease Study
Group.
Results : Adult onset HD was commonest while .Juvenile HD onset - 20 years was observed in ;
10% of patients. Chorea was the commonest presenting symptom (n17, 85%) while remaining
presented with psychiatric symptoms (n3, 15%) . Impairment of saccades was observed in 75%
of patients. Mean (SD) CAG repeats in the abnormal allele was 47.8 (8.7) . Total motor score but
not the total behavioral score worsens with duration of symptoms. The functional checklist score
correlates with total motor score rather than with duration of symptoms.
Conclusions: We detail clinical characteristics in genetically confirmed HD patients from a
predominantly North East Indian cohort. We observed a slightly higher occurrence of Juvenile
HD. Functional disabilities in our patients correlate with worsening of motor rather than
behavioral symptoms.
3.TITLE :CLINICORADIOLOGICAL ANALYSIS AND OUTCOME STUDY OF TRAUMATIC
INTRACRANIAL EPIDURAL HEMATOMAS IN PEDIATERIC POPULATION.
Presenting Author: Waqar Ahmad
Co Author: Dr C B Sahay, Dr Thomas J Minz, Prof Anil Kumar
Institution : Department of Neurosurgery, Rajendra Institute of Medical Sciences
ABSTRACT :
AIM :Theaims of this analysis were to review the cause and outcome of pediatricEpidural
Hematoma(EDH) and to discuss criteria of medical/surgical management & outcome-related variables.
BACKGROUND :Traumatic head injury is a common cause of mortality and acquired neurological
impairment in children. Pediatric EDHconstitute a distinct subgroup of head injury in pediatric
population.
METHODS :A series of 90 children (below 16 years of age )withEDH treated at the Department
of Neurosurgery , RIMS , Ranchi between July 2011 and August 2014 were analysedin detail.
Clinical course, radiological findings and outcomes were evaluated. Computed Tomography(CT)
scan constituteda valuable tool for an early and correct diagnosis apart from Magnetic resonance
imaging(MRI) in few. Patients’ medical records, CT scans were reviewed to define
variablesassociated with outcome.
Management &Outcome Variables included in the analysis were age of patient,time-lapsed,
mode of injury, signs & symptoms, associatedextracranial and/or intradural injury, abnormal
pupillary response, lucid interval,hematomalocation &volume, Child coma scale at time of
admission and diffuse axonal injury.
RESULTS:The most common symptoms werevomiting (78%, n=70) loss of consciousness (72%, n=65) ,
headache & irritability( 67%, n=60). The most frequent mechanisms of injury was fall from a
height(64%, n=58).Barring a few cases( 10% ,n=9)who underwent medical management( vol< 25 ml with
<5 mm midlineshift& GCS >9), most were treated surgically( 90%, n=81).Overall mortality was
5.5%(n=5).The mean follow-up was six months.
CONCLUSION:The outcome& management protocolwas found to be predominantly affected by the
preoperative neurological status,by the duration of the time interval between injury & onset of
neurological deterioration, and mainly by the presence of associated brain lesions.
4.MEDULLOBLASTOMA: A NEW CLINICO-IMMUNO-HISTOPATHOLOGICAL
CLASSIFICATION: IS IT REQUIRED?
Ravi Shankar1, Medha Tatke2, Ajay Sharma2
Jaypee Hospital, Noida & 2 G.B. Pant Hospital, New Delhi.
1
Aim: Medulloblastoma, one of the common brain tumors of childhood has been prognosticated
on the basis of Chang’s classification system for a long time. However with the advent of new
factors, we propose a new classification system based on combination of various prognostic
factors.
Material & Method: In a consecutive series of 63 patients operated for medulloblastoma over a
period of three years, overall outcome was studied in reference to a set of various clinicoimmuno-histopathological features.
Result: The mean age of 63 cases at presentation was 11.6 yrs with 39 males and 24 females (M:
F = 1.6:1) and mean duration of symptoms was 14.14 weeks with first symptom being headache
in 76.2% (48/63) of patients. Majority of the tumors was vermian (87%; 55/63). VP shunt prior
to definitive surgery was done in 28% (18/63) cases. Near/ gross total resection was achieved in
74.6% (47/63) of the cases. All, except 5 patients completed radiotherapy while 52% (33/63)
completed chemotherapy. The most common variant was classical (68.3%; 43/63) followed by
the desmoplastic variant (22.2%; 14/63) and large cell/anaplastic variants each comprised (4.8%;
3 cases each/63). GFAP was positive in 54 % (34/63) of the cases. Synaptophysin was positive in
81 % (51/63) of the cases & the mean MIB-LI of the 63 cases was 20.59 ± 7.86 (range 8-40).
The median duration of follow up was 41 month (Mean: 45 months; range 1-130 months) with
33 (52.4%) documented recurrence with 13 mortality (20.6%).
Conclusion: Classical medulloblastoma had a better prognosis than the desmoplastic variant with
LC/A variants having the worse prognosis. Severity of nuclear atypia (p – 0.08), presence of
mitotic/ apoptotic figures in sheets (p – 0.01), presence of necrosis (p – 0.03), endothelial
proliferation (p – 0.08) & MIB > 20 are associated with increased recurrence rates. Extent of
resection (p – 0.004), chemotherapy (p – 0.02), and presence of gross anaplasia (p – 0.03), are
the most important and independent predictors of recurrence rates.
5.Title of Paper - “Caregiver burden of dementia: correlation with stages of dementia and
behavioral and psychological symptoms of dementia”
Presenting Author- Adreesh Mukherjee
Qualification- MBBS, MD (Medicine), DM PDT (Neurology)
Co-authors- Atanu Biswas, Arijit Roy
Place of work- Department of Neurology, Bangur Institute of Neurosciences, Kolkata
Title/Position- DM PDT (Neurology)
ABSTRACT
Objective- To evaluate caregiver burden (CB) of dementia alongwith the correlation between
caregiver burden with stages of dementia and behavioral and psychological symptoms of
dementia (BPSD).
Methods- Study population consisted of 20 consecutive patients of dementia and their caregivers.
Dementia was diagnosed by DSM-IV criteria. Patients were assessed using structured proforma
of cognitive clinic of Bangur Institute of Neurosciences. Staging was done by Clinical Dementia
Rating (CDR) scale. Standard criteria were used to delineate types of dementias. BPSD were
assessed with Neuropsychiatric Inventory (NPI). CB was evaluated with Zarit Burden Interview
(BI).
Results- Of the 20 patients included in the study, 14 were male and 6 female. Average age of
patients was 66.2 years. 12 patients had Alzheimer Disease, 3 had Mixed dementia, 3
Frontotemporal Dementia, 1 Vascular dementia, 1 Dementia with Lewy Bodies. Average BI
scores in Global-CDR groups were 11.5 for CDR-0.5, 37.9 for CDR-1, 57 for CDR-2 and 54 for
CDR-3. Total NPI scores were: 3.3 for little-or-no burden, 19.7 for mild-to-moderate burden,
24.9 for moderate-to-severe burden, 41.2 for severe burden, respectively. CDR- sum of boxes
scores were: 3.3 for little-or-no burden, 6.7 for mild-to-moderate burden, 13 for moderate-tosevere burden, 13.4 for severe burden, respectively.
Conclusions- CB was more with increasing severity of dementia. However, moderate-to-severe
and severe burden groups had similar severity of dementia. Behavioral and psychological
symptoms of dementia have immense effect on the caregiver burden; especially in moderate to
severe dementia it seems to have greater effect than the stage of dementia itself.
Chronic Inflammatory Demyelinating Polyradiculoneuropathy and associated diseases
Authors: Bhowmick Suvorit1, Kayal Ashok Kumar2, Goswami Munindra3, Das Marami4,
Basumatary Lakshya Jyoti5
1.
2.
3.
4.
5.
2nd year DM student, Neurology Department, Gauhati Medical College
Professor and Head, Neurology Department, Gauhati Medical College
Professor, Neurology Department, Gauhati Medical College
Associate Professor, Neurology Department, Gauhati Medical College
Assistant Professor, Neurology Department, Gauhati Medical College
Place of Work: Department of Neurology, Gauhati Medical College, Guwahati
Number of Words (Abstract): 250
__________________________________________________________________________
Abstract
Introduction
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a clinically
heterogeneous, symmetric, sensory and motor neuropathy evolving over more than 8 weeks. It is
associated with various diseases.
Objectives
1. To evaluate CIDP patients for associated diseases
2. To study the clinical spectrum
Methods
In an observational study from the Neurology Department of Gauhati Medical College from
January 2013 to September 2014, 22 consecutive patients of CIDP (as per EFNS/PNS criteria)
were evaluated with complete blood count, serum protein electrophoresis, blood glucose, serum
electrolytes, liver and renal function tests, TSH, ANA, HBsAg, Anti-HCV, HIV, chest
radiograph, ultrasound abdomen, nerve electrophysiology and CSF analysis. Borrelia burgdorferi
serology, serum ACE, skeletal survey and bone marrow study were done whenever indicated.
Results
Results are shown in the following table:
Clinical features
Age, median (range)
Duration of illness before diagnosis
Males
Course
Relapsing
Chronic progressive
Monophasic
Type
Typical
Atypical (pure motor)
Manifestations
Weakness
Facial weakness
Bulbar weakness
Positive sensory symptoms
Sensory loss
Sensory ataxia
Tremor
Pseudoathetosis
Postural hypotension
Urinary hesitancy
CNS involvement
Girdle sensation
Seizure
Pyramidal, extrapyramidal, cerebellar
Associated conditions
Diabetes Mellitus (DM)
IgG MGUS
IgM MGUS
POEMS
Cryptogenic CLD
HIV
Membranous glomerulonephritis
Pyloric stenosis
n (N = 22)
45 (10 – 75) years
3 – 42 months
18
6
5
11
19
3
22
4
1
7
19
6
4
2
2
1
3
1
1
1
9 (40.9%)
1
2
1
1
1
1
1
1
Conclusion
41% of our CIDP patients had associated conditions including DM, MGUS, POEMS, HIV, CLD
and membranous glomerulonephritis.
7.Dural sinus thrombosis in trans-labyrinthine approach for vestibular schwannomas:
SHSC, University of Toronto Experience
Ashish Kumar1, Farhad Pirouzmand2, Jospeh Chen3
1 Department of Neurosurgery, Nizams Institute of Medical Sciences, Hyderabad, Telangana,INDIA
2 Division of Neurosurgery, Sunnybrook Health Sciences Centre, University of Toronto
3 Division of Otolaryngology and Skullbase surgery, Sunnybrook Health Sciences Centre, University of
Toronto
Background:
Dural sinus thrombosis (DST) is a rare complication associated with skull-base surgery.
Vestibular schwannomas (VS) have been approached traditionally by middle fossa (MF), translabyrinthine (TL) and retro-sigmoid (RS) approaches. We analyzed the incidence of dural sinus
thrombosis associated with trans-labyrinthine approach for VS and analyzed the possible factors
associated with the same.
Material and methods:
All the patients of VS who were operated by the TL approach between January 2012 and April
2014 at Sunnybrook Health Sciences Centre, Toronto were analyzed for the incidence of DST
post operatively. The data was collected retrospectively from the electronic patient records and
follow up was done clinically and radiologically. Pre-operative MRI and post-operative CT scans
(post-operative day 1) were studied in details for detection of DST and for analyzing the preoperative factors which may influence its incidence. Follow-up was assessed by the Glasgow
Outcome Score (GOS).
Results:
A total of 67 patients were operated by the trans-lab approach in the mentioned period. 12
patients were detected to have DST (incidence: 18%). None of the patients were symptomatic for
acute or chronic sinus thrombosis. Mean age of presentation was 47 years (range: 20-75 years).
Most common presentation was hearing loss (100%) followed by gait ataxia (52%) and dizziness
and vertigo (25%). 44 patients (21 males;23 females) were assessed for different possible factors
responsible for DST (cystic component/size/T2W hyperintensity/pre-sigmoid space/ tumour
anteromedial to internal auditory canal (IAC)/ intra-operative injury to sinuses/dominance of
sinuses). None of the above said factors were found to be statistically significant (p<0.05)
although certain trends were seen. Overall CSF leak was seen as 6.8% while only 1 patient of
DST presented with CSF leak. 11 out of 12 patients were followed up for a period ranging from
2 months-2 years. The mean GOS was grade V where all patients were doing well in the follow
up.
Conclusions:
Dural sinus thrombosis is more common than expected after trans-labyrinthine surgeries for
vestibular schwannomas. Majority of these patients with DST are completely asymptomatic
(incidental DST) and hence may not need anti-coagulation treatment. This should encourage
people in our own country to choose trans-lab approach in skull base surgery without getting too
much perturbed (though realistic and careful) about possible venous complications.
8. A Cross Sectional Study on types and triggers of Headache Patients Presenting to a
tertiary care Hospital in cuttack.
NiharRanjan Biswal1, Ashok kumar Mallick.2
1
DM student, Department of Neurology,S.C.B.Medical College, CUTTACK
2
Professor &Head,Dept. of Neurology,S.C.B. Medical College, CUTTACK..
Abstract
Objective
A study to describe the classification of types of headache and triggersof migraine in patients
who seek medical advice.
Materials & Methods
Interview of 200 consecutive patients who presented to neurology OPD atS.C.B.Medical college
& hospital in cuttackwas conducted. Demographic details, pattern of headache, associated factors
and family history were recorded. International Classification of Headache Disorders version 2
was applied.
Results
70% of all patients were women and 82% of them were between 20 and 60 years of age.
Migraine was the most common disorder (60%) followed by tension-type headache (32.5%),
medicationoveruse headache (6.5%) and cluster headache (1%). Chronic daily headache was
seen in 48 patients. Eleven percent of female migraineurs suffered from MRM. Study subjects
with presence of factors like marriage, family history, inadequate sleep, family stress and
exposure to onions had a statistically significant higher proportion than those who did not have
such exposure.
Conclusions
Patients who seek medical advice for headache are usually in their most productive ages.
Migraine and tension-type headache are the most common clinical presentations of headache.
Onset of migraine is earlier in patients with first-degree family history. Menstrually related
migraine affects women with headache episodes of longer duration than other patients and it
warrants special therapeutic consideration.
9.. Infantile Cerebral Aspergillosis in an ImmunoCompetent HostA rare case report & review of literature
Authors- Prof. Sudhanshu Sekhar Mishra,
Dr. Soubhagya Ranjan Tripathy.
Abstract
-
Invasive
craniocerebral aspergillosis,
often
encountered
in
an
immunocompromised setting, is almost uniformly fatal despite radical surgical and medical
management, and is frequently a necropsy finding1. Although occurring most commonly in the
immunosuppressed patient, cerebral aspergillosis infection has been reported after trauma or
neurosurgical procedures 2.
Attempts at fungal isolation are often unrewarding and a high index of radiological
suspicion is essential3. In the present case, the infant presented with progressive increase in head
size (for 2 months since birth) i.e. a head circumference of 55 cm with delayed developmental
milestones and intermittent vomiting for 1 month.
Histopathological findings were suggestive of invasive aspergillosis, although there was
no evidence of the infection in the lungs or paranasal sinuses. Voriconazole therapy was started
at 7th post-operative day after the biopsy report. Histologically, aspergillus hyphae were detected
in the biopsy material taken during the ETV procedure from the aqueduct.
The child has reported back for follow up 4 weeks after discharge, when her head
circumference was 47.5 cm; and she was active & feeding well.
The first reported case of an aqueductal stenosis caused by an aspergilloma was in 2000 Jan- Feb
in Clin Neuropathol. 2000 Jan-Feb;19(1):26-9 (by van Landeghem FK1, et al). Hence our patient
may only be the 2nd reported case of hydrocephalus due to aqueductal stenosis caused by
Aspergillus.
And to the best of our knowledge, this may be the 1st aqueductal stenosis by Aspergillosis
in an immunocompetent child, as the earlier case had aplasia cutis congenita at the scalp vertex
and hypoplastic left heart syndrome.
10.SPECTRUM OF DEMENTIA IN A RURAL BASED MEDICAL COLLEGE
DEPT OF NEUROLOGY,BURDWAN MEDICAL COLLEGE
Principal Author: Dr Souvik Dubey
Co –Author: Dr Bitoti Chattopadhyay*, Dr A P Bhattacharya*, Dr Mahua Dubey**, Dr H
Ahmed*, Dr S P Nandi***, Dr D Laha*****, Dr M K Ghosh*****, Dr S Pal******, Dr A K
Mallick*******, Prof S K Das******** *Resident, Dept of Neurology, Burdwan Medical
College ** Resident, Dept of Psychiatry, Burdwan Medical College ***RMO-CT, Dept of
Neurology, Burdwan Medical College ****Asst. Prof, Dept of Neurology, Burdwan Medical
College ***** Associate Professor, Dept of Radiology, Burdwan Medical College
******Professor, Dept of Neurology, Medical College Kolkata *******Professor, Department
of Psychiatry, Burdwan Medical College ******** Professor and HOD, Department of
Neurology, Burdwan Medical College
BACKGROUND:Most of the studies on dementia in India are from urban areas which only
constitutes 30% of Indian population,So the idea of this study is to study dementia among rural
population from a rural medical college to highlight rural scenario of dementia.
OBJECTIVES:Objectives are to diagnose dementia and its various subtypes clinically and
radiologically.
METHODOLOGY:We evaluated the suspected subjects with possible cognitive dysfunction
through history and clinical examination including BMSE(validated Bengali Mental State
Examination) as screening tool and KCB(Kolkata cognitive battery),for various domain
involvement and EASI(Everyday ability scale for India),for activities of daily living and geriatric
depression scale to rule out coexisting depression.Investigation include neuroimaging,Blood and
relevant biochemical tests.
RESULTS:Among 38 dementia patients,6 reversible(RD),32 irreversible(IRD) and 8 had young
onset dementia.Among IRD the frequency was AD(8) ,FTD(3),VaD(12),mixed dementia
(4),Parkinson disease with dementia (2) and Parkinsonism plus syndrome in 3(PSP-2;DLB-1).In
IRD predominant domain involvement,memory-25,social-24,execuetive-14.5AD had temporal
lobe atrophy(B/L).sevenVAD patients had past history of stroke and 10 had typical MRI
finding. two FTD had frontal lobe atrophy.onePDD had mid brain atrophy. All mixed dementia
had 2 or more vascular risk factor and mixed MRI features.Among RD, probable etiology was
(1)hypothyroidism,(1)NPH,(2)tumor,and (2)subdural hematoma.
CONCLUSION:In our study, vascular dementia remains top among irreversible dementia.
Temporal lobe atrophy in AD and periventricular ischemia in VaD common.Young onset
dementia common. Since rural people seek medical consultation late in disease course,social
life are most affected.MRI imaging helps in diagnosis.
Platform Presentation
Comparison of Clinico-radiological Profile of Posterior Circulation Strokes with
anterior circulation stroke - Study from a Tertiary Care Centre in Eastern India
Bhushan Joshi, Dr. B. K Roy ,Dr D.S. Guin
Introduction: Very limited hospital statistics are available for Comparison of Clinicoradiological Profile of Posterior Circulation Strokes with anterior circulation stroke
Aims: To evaluate and compare cases of posterior circulation strokes and anterior circulation by
analyzing history, conventional risk factors, clinical, laboratory and radiological profile.
Material and Methods: 50 cases of posterior circulation strokes and 50 cases of anterior
circulation storke visiting Bangur Institute of Neurosciences, IPGMER, Kolkata, from Dec 2013
to September 2014 were evaluated for clinical and laboratory profile and neuroimaging
(CT/MRI/DSA).
Results : Of the total 50 cases of posterior circulation stroke, 32 (64%) were males (mean age –
48.7yrs) while 18 (36%) were females (mean age – 44.4yrs); 34(68%) were ischemic while 16
(32%) were hemorrhagic. Among ischemic strokes,30% had TIAs (18% had only posterior
circulation TIAs). Large artery (hemodynamic) (36%) and embolism (36%) (26%cardiembolism,
10% artery-to-artery embolism) were the most common stroke mechanisms. Distal posterior
circulation territory infarcts (44%) were much common than the proximal(26%) or middle
territory(30%) infarcts. For hemorrhagic strokes, uncontrolled hypertension was the commonest
mechanism while cerebellum and thalamus were the commonest sites. Commonest predisposing
factors for ischemic stroke were tobacco addiction followed by hypertension while for
hemorrhagic strokes, it was hypertension. Out of 50, 07 died,(14% mortality) 05 of hemorrhagic
strokes and 02 of ischemic stroke, commonest causes being septic and metabolic complications
Of the total 50 cases of Anterior circulation stroke, 35 (70%) were males (mean age – 49.7yrs)
while 15 (30%) were females (mean age – 50.4yrs); 29(58%) were ischemic while 21 (42%)
were hemorrhagic. Among ischemic strokes,34% had TIAs. Embolism (60%)
(46%cardiembolism, 14% artery-to-artery embolism) was the most common stroke mechanism.
For hemorrhagic strokes, uncontrolled hypertension was the commonest mechanism while Basal
ganglia were the commonest sites. Commonest predisposing factors for ischemic stroke were
tobacco addiction followed by hypertension while for hemorrhagic strokes, it was hypertension.
Out of 50, 10 died,(20% mortality) 08 of hemorrhagic strokes and 02 of ischemic stroke,
commonest causes being septic and metabolic complications.
Conclusion: Clinical profile of posterior and anterior circulation strokes are similar. Embolic
stroke mechanism, distal territory location, low GCS and high NIHSS at presentation conveyed
the worst prognosis.
2.Title:“Phrenic nerve conduction study in the early stage of Guillain–Barre syndrome
as a predictor of respiratory failure”
Dr. Barun Kumar
Associate Professor
Sen,
Professor
Asit
Kumar
Senapati,
Dr.
Alak
Pandit,
Introduction: Guillain-Barre´ syndrome (GBS) is an acute onset, immune-mediated disorder of
the peripheral nervous system. Guillain- Barre Syndrome (GBS) has an unpredictable clinical
course with up to 30% of patients requiring
assisted ventilation during the course of their illness. In severe cases, a combination of multiple
clinical factors culminate in neuromuscular respiratory failure and its main cause is weakness of
the diaphragm. Other factors are weakness of intercostal, abdominal, and accessory muscles of
respiration, retained airway secretions, atelectasis, and supine posture. Successful management
mandates anticipation, prompt recognition and optimal treatment of neuromuscular respiratory
failure in GBS.
Aims: To evaluate the frequency of phrenic nerve abnormalities in the early Guillain-Barre
syndrome and phrenic nerve conduction study as a predictor of respiratory failure in the early
stage of Guillain–Barré syndrome.
Material and Methods: Fifteen adult patients of GBS above 18 yrs of age admitted to Bangur
Institute of Neurosciences, IPGMER, Kolkata, from Jan 2014 to September 2014 and admitted
within 14 days of onset, were studied. Clinical and electrodiagnostic data were assesed and
compared between those developed respiratory failure and having no respiratory failure. The
clinical parameters assessed were median age, gender, antecedent infection, time to peak
disability, bifacial weakness, upper limb weakness, bulbar paralysis, neck weakness and
autonomic dysfunction. Electrodiagnostic studies included motor nerve conduction and phrenic
nerve conduction study (CMAP amplitude, distant latency, duration) done. Multiple logistic
regression analysis was used to compare the two groups.
Results : Of the total 15 cases of GB syndrome, clinical parameters like median age, gender,
antecedent infection, bifacial weakness, upper limb weakness and autonomic dysfunction did not
influence the development of neuromuscular respiratory weakness but time to peak disability,
bulbar paralysis, neck weakness influenced . Nerve Conduction Study revealed abnormal H
reflex and F waves to
be the commonest abnormality. Phrenic nerve CMAP latencies, CMAP amplitude, and CMAP
duration were significantly different between GBS patients with respiratory failure and without
respiratory failure. Two patients with the sum of phrenic nerve latency longer than 18 ms and
the sum of bilateral diaphragmatic CMAP amplitude smaller than 0.2 mV and longer CMAP
duration required respiratory assistance.
Conclusion: Phrenic nerve conduction study (CMAP latency, amplitude and duration) was
found to be a sensitive parameter in assessing the severity of the disease and predicting
impending respiratory failure in Gb syndrome patient.
3. TranscallosalInterhemispheric Approach for Colloid Cyst: Technical pearls
Dr Ashish Kumar
Assistant Professor, Department of Neurosurgery, Nizams Institute of Medical Sciences, Hyderabad, Telangana,
INDIA
Background:
Colloid cysts arise from the roof of the third ventricle and are developmental in origin. They
commonly obstruct the Foramen of Monroe and result in tri-ventricular hydrocephalus.
Headache is most common presenting symptom and timely surgical management is the
key.Colliod cysts can be tackled by both open and endoscopic means. Ventricular dilatation
remains the key in determining the approach (Trans-callosal Vs Trans-cortical). Surgical
disconnection and seizures being the chief drawbacks of trans-callosal and trans-cortical routes
respectively.
Material & Methods:
Four patients were operated in between 2012-1013 at Nizams Institute of Medical Sciences,
Hyderabad by single surgeon. 3 patients were operated by trans-callosal and 1 was operated by
trans-cortical routes. We describe the approach by demonstrating the key surgical steps by means
of operative video and discuss possible complications and its avoidance.
Results:
All four patients did well. No memory impairment and post-operative hydrocephalus was
observed. Intra-operative bleeding occurred in 2 patients which was controlled easily.
Conclusions:
Interhemispherictrans-callosal approach is a safe and effective corridor for surgical management
of III ventricular colloid cysts. Basic understanding of surgical anatomy and adequate expertise
may help in achieving excellent outcomes.
4. TITLE : “EVALUATION OF HEADACHE DISORDERS IN PATIENTS OF
IDIOPATHIC EPILEPSY”
•
PRESENTING AUTHOR- UJJAWAL*
•
QUALIFICATION- MD(GENERAL MEDICINE)
•
CO-AUTHORS-KUNDU TN**, ROY A***
•
PLACE
OF
WORK-BANGUR
INSTITUTE
OF
NEUROSCIENCES,
IPGMER(KOLKATA)
*DM Resident, Department of Neurology, Bangur Institute of Neurosciences, IPGMER(KOLKATA)
**Professor, Department of Neurology, Bangur Institute of Neurosciences, IPGMER(KOLKATA)
***Assistant Professor, Department of Neurology, Bangur Institute of Neurosciences, IPGMER(KOLKATA)
INTRODUCTION : Epilepsy is a condition characterized by recurrent seizures, unprovoked by
any immediate identifiable cause. Various types of headache, especially migraine, is considered
a relevant co-morbidity in epilepsy. Headache in idiopathic epilepsy may have diagnostic and
therapeutic implications.
Materials and methods : Herein, we report clinical data on 40 patients of Idiopathic epilepsy. All
eligible patients of idiopathic epilepsy were recruited from general OPD or epilepsy clinic of
Bangur Institute of Neurosciences. Headache was classified according to ICHD III and headache
disability before and after treatment was calculated according to Henry Ford Headache Disability
Index(HDI).
Results : Out of 40 cases recruited, 23(57.50%) had some or the other type of headache.
Maximum number of cases had Idiopathic Generalized Epilepsy(IGE) with Generalized Tonic
Clonic Seizures(GTCS) only and most common headache type in these patients being post ictal
followed by probable migraine without aura and infrequent episodic Tension type headache.
Other types of seizure noted were Juvenile Myoclonic Epilepsy(JME) , Benign Epilepsy with
Centro-Temporal Spikes(BECTS) and Juvenile Absence Epilepsy(JAE). Additional drug
requirement was high in cases of migraine without aura and in cases of episodic TTH. HDI was
reduced to a significant amount in 100% of the cases after additional treatment.
Conclusions: This is an ongoing study. Overall, apart from post ictal headache, TTH and
migraine was seen in patients of JME and IGE with GTCS only. We noted migraine in one out of
2 patients of BECTS as expected. Patients of JAE did not have any type of headache in the
course of the disease. In majority of patients Migraine and TTH required additional therapy, but
surprisingly in all of the cased there was significant reduction in the headache frequency and
reduction/ improvement in HDI.
5. Visual and surgical outcome in craniopharyngioma
Mukund Prasad, Rabindra Kumar, Deepak Karn
PARAS HMRI Hospital, Raja Bazar, Bailey Road, Patna-800014
Background: Craniopharyngiomas present with systemic as well as visual symptoms. Treatment strategy
varies from subtotal resection(STR) with radiotherapy of residual lesion to gross total resection(GTR).
Three consecutive cases of craniopharyngioma treated over last 10 months are presented.
Method: Three consecutive cases of craniopharyngioma, first a 17 year old boy with complete loss of
vision in both eyes of five days’ duration; second, a 31 year old male with hydrocephalus but no visual
symptoms and third, a 32 year old male with temporal field cut in right eye and perception of light only in
left eye, were treated surgically with aim of GTR.
Results: All were operated via right pterional route with or without additional obitozygomatic or
zygomatic osteotomy.
Near total resection was achieved in blind seventeen year old boy with return of vision to 6/6 in left eye
and 6/24 in right eye and bitemporal field cut after 2 months. Clinical and radiological follow up at six
months showed no regrowth or vision deterioration.
Thirty one year old male with hydrocephalus had GTR with resolution of hydrocephalus.Clinical and
radiological follow up at four months did not reveal any recurrence or new visual deficit.
GTR was achieved in third patient also with improvement of field cut in right eye and perception of
movement in left eye.
Conclusion: Safe GTR of craniopharyngiomas is possible and should be the aim of surgery.
8.Compound Head injuries in adult, An Institutional Experience
Ahmed Junaid, Borah Naba, Hussain Zakir , Baishya Kr. Basanta, Ahmed
Shameem , Barooah Kr. Rajesh.
Department of Neurosurgery, Gauhati Medical College, Assam.
Abstract:- compound depressed skull fractures occur when there is a laceration
over the fractures, resulting in the internal cranial cavity being in contact with the
outside environment. Compound head injuries are associated with a high risk of
infection, injury to the underlying brain and post traumatic seizures.
Aims and objectives: To analyze the patients with compound head injury, their
management protocol and evaluate the incidence of complications and ultimate
outcome
Materials and methods: 110 consecutive patients with compound head injury
admitted under the department of neurosurgery, Gauhati Medical College and
Hospital during the period of one year from January 2013 to December 2013 were
studied retrospectively.
Results And Observations: In our study, majority of patients 57 (51.8%) were
found between 16-30 years of age. Male to female ratio was 4.5:1. Mean age was
24.3 years. Most common mode of injury was , other causes included assault, fall
from the height etc. Out of total 110 patients, 85 patients (72.27%) were treated
surgically and 25 (22.73%) were managed conservatively. Complications occurred
in 31 patients (28.18%). Meningitis was the most common complications. Good
recovery was observed in 87 cases (79.09%) and 15 cases (13.63%) died.
Conclusion: Compound head injury always has the risk of complications i.e.
meningitis, seizure, CSF leak etc. early surgery and adequate debridement with
antibiotic cover tend to reduce the complication rate.
6. Compound Head injuries in adult, An Institutional Experience
Ahmed Junaid, Borah Naba, Hussain Zakir , Baishya Kr. Basanta, Ahmed
Shameem , Barooah Kr. Rajesh.
Department of Neurosurgery, Gauhati Medical College, Assam.
Abstract:- compound depressed skull fractures occur when there is a laceration
over the fractures, resulting in the internal cranial cavity being in contact with the
outside environment. Compound head injuries are associated with a high risk of
infection, injury to the underlying brain and post traumatic seizures.
Aims and objectives: To analyze the patients with compound head injury, their
management protocol and evaluate the incidence of complications and ultimate
outcome
Materials and methods: 110 consecutive patients with compound head injury
admitted under the department of neurosurgery, Gauhati Medical College and
Hospital during the period of one year from January 2013 to December 2013 were
studied retrospectively.
Results And Observations: In our study, majority of patients 57 (51.8%) were
found between 16-30 years of age. Male to female ratio was 4.5:1. Mean age was
24.3 years. Most common mode of injury was , other causes included assault, fall
from the height etc. Out of total 110 patients, 85 patients (72.27%) were treated
surgically and 25 (22.73%) were managed conservatively. Complications occurred
in 31 patients (28.18%). Meningitis was the most common complications. Good
recovery was observed in 87 cases (79.09%) and 15 cases (13.63%) died.
Conclusion: Compound head injury always has the risk of complications i.e.
meningitis, seizure, CSF leak etc. early surgery and adequate debridement with
antibiotic cover tend to reduce the complication rate.
1. Compound Head injuries in adult, An Institutional Experience
Ahmed Junaid, Borah Naba, Hussain Zakir , Baishya Kr. Basanta, Ahmed
Shameem , Barooah Kr. Rajesh.
Department of Neurosurgery, Gauhati Medical College, Assam.
Abstract:- compound depressed skull fractures occur when there is a laceration
over the fractures, resulting in the internal cranial cavity being in contact with the
outside environment. Compound head injuries are associated with a high risk of
infection, injury to the underlying brain and post traumatic seizures.
Aims and objectives: To analyze the patients with compound head injury, their
management protocol and evaluate the incidence of complications and ultimate
outcome
Materials and methods: 110 consecutive patients with compound head injury
admitted under the department of neurosurgery, Gauhati Medical College and
Hospital during the period of one year from January 2013 to December 2013 were
studied retrospectively.
Results And Observations: In our study, majority of patients 57 (51.8%) were
found between 16-30 years of age. Male to female ratio was 4.5:1. Mean age was
24.3 years. Most common mode of injury was , other causes included assault, fall
from the height etc. Out of total 110 patients, 85 patients (72.27%) were treated
surgically and 25 (22.73%) were managed conservatively. Complications occurred
in 31 patients (28.18%). Meningitis was the most common complications. Good
recovery was observed in 87 cases (79.09%) and 15 cases (13.63%) died.
Conclusion: Compound head injury always has the risk of complications i.e.
meningitis, seizure, CSF leak etc. early surgery and adequate debridement with
antibiotic cover tend to reduce the complication rate.
Digital Poster presentation
1. Comparison of Clinico-radiological Profile of Posterior Circulation Strokes with
anterior circulation stroke - Study from a Tertiary Care Centre in Eastern India
Bhushan Joshi, Dr. B. K Roy ,Dr D.S. Guin
Introduction: Very limited hospital statistics are available for Comparison of Clinicoradiological Profile of Posterior Circulation Strokes with anterior circulation stroke
Aims: To evaluate and compare cases of posterior circulation strokes and anterior circulation by
analyzing history, conventional risk factors, clinical, laboratory and radiological profile.
Material and Methods: 50 cases of posterior circulation strokes and 50 cases of anterior
circulation storke visiting Bangur Institute of Neurosciences, IPGMER, Kolkata, from Dec 2013
to September 2014 were evaluated for clinical and laboratory profile and neuroimaging
(CT/MRI/DSA).
Results : Of the total 50 cases of posterior circulation stroke, 32 (64%) were males (mean age –
48.7yrs) while 18 (36%) were females (mean age – 44.4yrs); 34(68%) were ischemic while 16
(32%) were hemorrhagic. Among ischemic strokes,30% had TIAs (18% had only posterior
circulation TIAs). Large artery (hemodynamic) (36%) and embolism (36%) (26%cardiembolism,
10% artery-to-artery embolism) were the most common stroke mechanisms. Distal posterior
circulation territory infarcts (44%) were much common than the proximal(26%) or middle
territory(30%) infarcts. For hemorrhagic strokes, uncontrolled hypertension was the commonest
mechanism while cerebellum and thalamus were the commonest sites. Commonest predisposing
factors for ischemic stroke were tobacco addiction followed by hypertension while for
hemorrhagic strokes, it was hypertension. Out of 50, 07 died,(14% mortality) 05 of hemorrhagic
strokes and 02 of ischemic stroke, commonest causes being septic and metabolic complications
Of the total 50 cases of Anterior circulation stroke, 35 (70%) were males (mean age – 49.7yrs)
while 15 (30%) were females (mean age – 50.4yrs); 29(58%) were ischemic while 21 (42%)
were hemorrhagic. Among ischemic strokes,34% had TIAs. Embolism (60%)
(46%cardiembolism, 14% artery-to-artery embolism) was the most common stroke mechanism.
For hemorrhagic strokes, uncontrolled hypertension was the commonest mechanism while Basal
ganglia were the commonest sites. Commonest predisposing factors for ischemic stroke were
tobacco addiction followed by hypertension while for hemorrhagic strokes, it was hypertension.
Out of 50, 10 died,(20% mortality) 08 of hemorrhagic strokes and 02 of ischemic stroke,
commonest causes being septic and metabolic complications.
Conclusion: Clinical profile of posterior and anterior circulation strokes are similar. Embolic
stroke mechanism, distal territory location, low GCS and high NIHSS at presentation conveyed
the worst prognosis.
2. AN UNUSUAL CASE OF STROKE DUE TO MULTIPLE ISCHEMIC
INFARCTION FOLLOWING RUSSELL'S VIPER ENVENOMATION
PRESENTING AUTHOR :- Dr LULUP KUMAR SAHOO
DM Student Dept. of Neurology
CO-AUTHORS-Dr.Ashok
Kumar
Mallick,Dr.KaliPrasanaSwain,Dr.GeetaMohanty,
NiharRanjanBiswal,Dr.Srikant Kumar Sahu
Dr
PLACE:- S.C.B. MEDICAL COLLEGE ,CUTTACK,ODISHA
ABSTRACT
BACKGROUND:- Snake bites are common cause of morbidity and mortality in
India.Neurological deficit following viper bite is not uncommon and is usually due to
intracerebral bleed. Ischemic infarction following viper envenomation has been described by few
authors.
CASE PRESENTATION:- We report a case of acute ischemic infarction following viper bite in
a previously healthy man.36 yr healthy male presented with snake bite over right foot.1hour
later he developed local swelling followed by loss of consciousness. During transportation to
hospital he had 1 bout of hematemesis. Patient’s attendants noticed decreased movement of right
upper & lower limb. He required mechanical ventilation as the Glasgow coma scale was 7/15.
Laboratory parameters revealed a normal coagulation profile and no hematuria. Biochemical
profile, ECG, X-ray chest and 2D-ECHO were normal. He had no repeat bleeding or seizure.On
day of admission 20WBCT was done & blood didn’t clot. On day2,CT scan brain was done
which showed multiple infarction involving left Fronto-Temporal lobes,right basal
ganglia,thalamus,occipital lobe & left cerebellar parenchyma.He was treated with equine
polyvalent ASV( 30 vials), anti-edema measures and anti-platelets. After ASV treatment his
20WBCT became normal. Patient showed good recovery with treatment.
CONCLUSION:-Occurrence of ischemic infarction in multiple arterial territories though rare
may occur with viper envenomation.
20WBCT—20 min Whole Blood Clot Test
3. AN UNUSUAL CASE OF HIGH DOSE SHORT DURATION OF ASPIRIN
INDUCED SPONTANEOUS FRONTAL LOBAR HEAMMORHAGE; A CASE
REPORT
Author-;DrNihar Ranjan Biswal. DM student Dept. of Neurology ,S.C.B.MEDICAL COLLEGE
CUTTACK
CO-Authors-Dr.Ashok Kumar Mallick, Dr.KaliPrasanaSwain, Dr.GeetaMohanty,Dr.Lulup
Kumar Sahoo, Dr.Srikant Kumar Sahu,Dr.B.Rath,Dr.M.Samanta,Dr.B.K.Mohanty
ABSTRACT
BACKGROUND; Aspirin has a very high benefit-risk ratio and has been widely used in the
secondary prevention of cardiovascular and cerebrovascular diseases.Although sometimes
aspirin after taken for long duration associated with gastrointestinal and skin bleeding but it can
also cause life threatening intracerebralheammorhage.
CASE PRESENTATION; we report a 30 yr male with RHD,severe MR, polyarthritis was on
aspirin325mg 4tablets thrice daily for last 2days(6 doses within 48 hours) presented with sudden
onset vomiting and altered sensorium with lthemiparesis.Immediate CT scan showed right
frontal lobe heammorhage with intraventricularextension.His coagulation profile was within
normal range.MRI revealed rt frontal lobe heammorhage with intraventricularextention.MRA
was normal.At admission his NIHSS was-8/42
and at discharge his NIHSS was
0/42.BERTHEL index at discharge was 100/100 .He was managed conservatively.
Conclusion-spontaneous intracerebralheammorhage should be kept in mind even with short
duration of high dose aspirin prescribed for rheumatic arthritis .
4. LINDAU BROTHERS: TWO SIBLINGS WITH HEMANGIOBLASTOMAS
Authors- Dr Somnath Prasad Jena, 2nd year MCh student,
Prof. (Dr) Sudhansu Sekhar Mishra, Head Of the Department
Dept of Neurosurgery, SCB MCH, Cuttack, Odisha
INTRODUCTION
Hemangioblastomas are benign, slow growing, vascular neoplasms which occur in sporadic and
inherited forms. These inherited hemangioblastomas are associated with Von-Hippel Lindau
Syndrome(VHL). VHL gene mutations (Chromosome 3p25.3) cause inactivation of the VHL
protein resulting in this phenotype. VHL patients must be screened for multiple
hemangioblastomas, along with screening for other visceral lesions associated with this
condition. We evaluated and operated 2 siblings with Infra-tentorial Hemangioblastomas
CASES
1ST BROTHER: The elder brother, a 56year male presented with cerebellar involvement and
features of raised ICP 4 years back, with MRI impression Cerebellar Hemangioblastoma. He
underwent near-total excision, with histo-pathology confirming Hemangioblastoma. This
resulted in resolution of symptoms for 3 years followed by recurrence of symptoms for which he
was re-evaluated and re-operated.
2ND BROTHER: The younger brother, a 46 year male, presented with symptoms of foramen
magnum compression, on MR imaging revealing multiple cystic lesions in the Posterior fossa,
extending through the foramen magnum. He underwent Foramen magnum decompression and
excision of the lesion, with histo-pathological proof of hemangioblastoma. Screening of spine
and the younger brother revealed a lesion in conus region.
FAMILY HISTORY: Mother had renal cell carcinoma
DISCUSSION
Familial hemangioblastomas are found in VHL syndrome. The complexity of associated
conditions like pheochromocytoma adds to the anesthetic challenge and the possibility of a RCC
creates diagnostic dilemma of a posterior fossa secondary vs. hemangioblastoma.
CONCLUSION
Von Hippel Lindau, presenting as infratentorial hemangioblastomas in the siblings is uncommon
in routine neuro-surgical practice. These challenges are to be met with clinical know-how and a
strict screening protocol in cases encountered.
5. CONGENITAL MALROTATION OF ATLAS WITH UNILATERAL
HYPERTROPHY OF ATLANTO-OCIPITAL JOINT – A RARE ANOMALY OF
CV JUNCTION AND ITS MANAGEMENT: A CASE REPORT
Presenting and corresponding author
Pratap Chandra Nath
Co-authors- Sudhansu Sekhar Mishra,
Rama Chandra Deo, Itibrata Mahanta
ABSTRACT
Congenital anomalies are commonly encountered in CVJ region due to its unique
embryological development. CV junction usually comprises occiput, atlas and axis. However
malrotation of atlas in between occiput and Axis with Arnold Chiari Malformation and unilateral
lateral mass hypertrophy with atlanto-ocipital joint hypertrophy manifesting features of high
cervical myelopathy is hardly reported anomaly.
Here we report a case of successfully managed 22 yrs female who presented to us with
high cervical compressive myelopathy. Imaging revealed rotation of C1 vertebra around 200
towards left side, with right atlanto-ocipital joint dislocation and hypertrophy of the joint with
hypertrophy of right lateral mass of C1, causing Chiari malformation type-1 and right atlanto
axial dislocation exposing right C2 superior facet.
She underwent right far lateral approach comprising right C1 hemilamiectomy and
medialization of right vertebral artery with excision of hypertrophied occipitoatlantal joint
extradurally and posteromedial excision of superior articular facet of C1.
The patient’s compressive myelopathy features improved post operatively.
We report the management of this rare congenital anomaliy with review of litretures
related to or like this anomalies.
Key Words- Malrotation, Hypertrophy, chiari malformation type-1, compressive myelopathy,
Farlateral approach, Hemilaminectomy, posteromedial excision.
6. MULTIPLE SPINAL SCHWANNOMA
INTRADURAL- A CASE REPORT
BOTH
EXTRADURAL
AND
Presenting Author-Dr Gyani J S Birua
Co-Authors- Dr Anil Kumar, Dr C B Sahay, Dr T J Minj, Dr Jeevesh mallik
Case Report
A 35 yrs old female came to our OPD with complains of inability to stand and walk for 1 month.
Her symptoms had started 6 months back and were gradually progressive. She complained of
low back pain with radiation to left thigh, of insidious onset which was more prominent in lying
down position and on straining. There was associated tingling over both lower limbs. Clinical
examination revealed an increased tone and gross sensory diminution in both legs. Motor power
was grade 1/5 in both lower limbs in all groups.
On further investigation MRI revealed multiple well defined, contrast enhancing dumb-bell
lesions at T7-8, T12-L1 and L4-5 level.
All the lesions extradural and intradural were completely excised in 2 stages and a good recovery
was achieved. Histopathological examination confirmed the lesions to be schwannoma.
Discussion
Schwannomas are benign, slowly growing, encapsulated peripheral nerve tumours.multiple level
schwanomas have been previously reported but existence of both extradural & intradural
schwanoma with involvement of cauda equina in a single patient is a rare entity.
Keywords – schwanoma, dumb-bell lesions, extra dural , intra dural
7. An interesting neuroimaging case of multiple intracranial SOL.
Dr. Mithilesh K. Pandey, Dr. Sunil Baranwal, Associate prof. Kaushik Roy,
Prof. Suniti K. Saha, Prof. S. Ghosh
Department of Neurosurgery, N R S Medical College and Hospital, Kolkata.
Abstract
A 58 yrs female was presented in our institution in conscious state with history of headache,
vomiting, recurrent generalised seizure, and progressive left sided weakness since 3 months
and on examination having anosmia, bilateral temporal hemianopia, left 7th UMN palsy, left
spastic hemiparesis. Past history of two episode of generalised seizure 2 years back for which
she consulted to local doctor and he advice CT scan brain which was reported normal.
20days before coming to this hospital another CT scan brain done in a medical college and
reported two lesion first one suggestive of anterior communicating artery aneurysm and 2nd
heterogeneously enhancing lesion in rt. fronto-parietal region.
MRI brain was done in our institution showed two lesion an intra-axial heterogenously
enhancing lesion in rt. parietal paraventricular region and another strongly enhancing extra-axial
lesion in basifrontal region. Patient was operated and biopsy came out for rt. fronto-parietal SOL
[Glioblastoma multiforme] and for basifrontal SOL [ Meningothelial menengioma ].
The simultaneous presence of primary brain tumors of different histological type in same patient
is very rarely occurs spontaneously. An association with multiple primary intracranial tumors of
different histological types not relating to radiotherapy or phacomatosis is a rare event. The
incidence of primary brain tumors with different histological types is only 0.3% of all brain
tumors. The true incidence of this association may be much higher, because many cases may
have a silent clinical course or have been identified but not reported in the literature.
8. Two Cases of Vertical gaze palsy due to same Metabolic etiology
Jacky Ganguly
There are multiple causes of conjugate vertical gaze impairment like vascular insult to
midbrain or thalamus, infective etiologies, multiple sclerosis, neuro-degenerative
diseases, metabolic diseases, drugs, etc. Among the metabolic causes, Niemann-Pick type
C disease and its variants are one of the most important causes. Niemann-Pick disease
type C (NPC) is a rare and fatal neurovisceral lipid storage disorder resulting from
autosomal recessively inherited loss-of-function mutations in either Npc1 or Npc2. This
disrupts intracellular lipid transport, leading to the accumulation of lipid products in the
late endosomes and lysosomes that affects both children and adults. Whereas the disease
in children is characterised by mental retardation, seizures and often rapid
neurodegeneration, in adults the disease is characterised by slow cognitive decline, major
neuropsychiatric illness, development of ataxia, vertical supranuclear opthalmoplegia and
dystonia. We have reported cases of two male patients, both presented with dysphagia,
spastic dysarthria, vertical gaze palsy (down-gaze more restricted than up-gaze),
dystonia without any involvement of higher mental function, muscle power, sensory
system and sphincters. One of them had ataxia and the other had parkinsonism too. Bone
marrow examination in both the cases had revealed sea blue histiocytes. Vulnerability of
cerebellar Purkinje neurons and of neurons in the rostral interstitial nucleus of the medial
longitudinal fasciculus (riMLF) to disrupted intracellular lipid storage may explain ataxia
and vertical gaze palsy respectively. Considering heterogeneous presentation of NPC, it
is often misdiagnosed as other movement or psychiatric disorders and better awareness of
this disease is thus required among the clinicians.