Q7

Uhthoff phenomenon causing worsening of myelitis symptoms (secondary to MS, PPMS?)

Workup: MRI Brain and spinal cord with contrast ,CSF analysis, CSF OCB, VEP, elevated total IgG and IgG ratio

MRI: multiple areas of abnormal signal intensity are present on T2-weighted and FLAIR brain MRI imaging in
juxtacortical periventricular, infratentorial and spinal cord, cord atrophy /swelling ay be seen

Volume loss seen in brain and spinal cord with progressive forms of MS and correlates with Disability

Acute plaques show contrast enhancement

Vep shows prolonged latency of P100

Delays or block of the N-20 potential of the median nerve, or the P37 potential of the tibial nerve, on
somatosensory-evoked potential tests can also help in diagnosing MS

To rule out other diagnoses Additional studies include blood tests for vitamin B 12, angiotensin-converting

enzyme, anti-SSA and anti-SSB, rheumatoid factor, antinuclear antibody, VDRL and FTA-ABS, HIV, and HTLV I/II
serologies

Treatment of choice

DMT: Ocrelizumab anti cd20 antibody, maintenance infusion once every six months, depletes b cells only

Antispasmodic baclofen 80mg/day/ tizanidine/ Gaba up to 3600mg

Dalfampridine for gait

Modafinil for fatigue up to 200 BD

Physical therapy

The radiographic hallmark of progressive MS appears to be volume loss of the spinal cord and brain that represent
end organ damage from inflammatory injury causing axonopathy, Wallerian degeneration, and subsequent neuropil
atrophy
Q8

Conus medullaris compression

Multiple myeloma

TB spine

EDH

Disc Herniation

Tumour/metastasis

TB spine is most likely diagnoses

MRI of the spine look for compression, confirm the cause, level of compression, see the spinal cord,

Biopsy of the lesion if needed

Xray chest and pulm TB work up if Xray shows

Serum protein immuno fixation

Bone marrow for plasma cells

HIV

Q5

Parkinson Disease

Examination:

Tremors,remergence, bardykinesia, glabellar tap, micrographia, hypophonia,

Pull test for postural insatbility

EOM for gaze palsy of PSP

Cerebellar signs for MSA and CBD

Orthostatic hypotension

MMSE for dementia

Other features

Craniofacial

•Hypomimia (masked facial expression)

•Decreased spontaneous eye blink rate

•Speech impairment, including hypokinetic dysarthria, hypophonia, and palilalia (repetition of a phrase or
word with increasing rapidity)

•Dysphagia

•Sialorrhea

●Visual

•Blurred vision

•Impaired contrast sensitivity

•Hypometric saccades

•Impaired vestibuloocular reflex

•Impaired upward gaze and convergence

●Musculoskeletal

•Micrographia

•Dystonia

•Myoclonus

•Stooped posture

•Camptocormia (severe anterior flexion of the thoracolumbar spine) [39]

•Pisa syndrome (subacute axial dystonia with lateral flexion of the trunk, head, and neck) [40-42]

•Kyphosis

•Scoliosis

•Difficulty turning in bed

●Gait

•Shuffling, short-stepped gait

•Freezing

•Festination

Non motor symptoms

●Cognitive dysfunction and dementia

●Psychosis and hallucinations

●Mood disorders including depression, anxiety, and apathy/abulia

●Sleep disturbances

●Fatigue

●Autonomic dysfunction

●Olfactory dysfunction

●Gastrointestinal dysfunction

●Pain and sensory disturbances

Q6

Acute angle closure galucome in patient with sturge webber syndrome due to topiramamte
Acute angle closure glaucoma due to topiramate, sturge webber syndrome itself is associated with raised
IOP and glaucoma that is worsened by topiramate

Clinical featuress: cutaneous capillary malformation ore commonly in v1 and v2 distribution , called port
wine stain

Leptomeningeal vascular malformation on the same side a ortwine stain

Parenchymal calcifications from chronic venous stasis

Seizures ande epilepsy: focal, gtcs and ocassioanlly myoclonic

Stroke

Cognitive disturbances in 60%

Visual field problems if involving optic tracts or occipital lobe

Venous malformations in eye , choroid, and conjunctiva ,

Growth harmone deficiency

Associated disorders

Neurocutaneous syndromes plus

Klippel-Trenaunay syndrome

parkes webber syndrome

Servelle-Martorell syndrome

Q3

Landau Kleffner syndrome

Bilateral centrotemporal spikes and sharp waves that spread to both hemispheres and become continuous during
sleep giving appearance of ESES

Treatment: Antiseizure drugs with some reported benefit include valproate, clonazepam, levetiracetam, and others;
polytherapy is often needed

A trial of corticosteroids is recommended if there is not a rapid response to antiseizure drugs, The effectiveness of
therapy is measured by the patient's clinical response (ie, control of seizures), improving developmental milestones,
and the proportion of spike-wave activity on overnight EEG recordings

Some patients with LKS and CSWS may benefit from surgical treatment, but the approach must be individualized.

Multiple subpial transection is one such approach [72]. Subpial transection consists of closely spaced slicing of the
temporal lobe gyri involved in the epileptic process, with interruption of the short cortical-cortical fibers in the white
matter immediately under the cortical gray. This limited slice disrupts the epileptic interconnections and stops the
epileptiform activity while sparing the thalamocortical fibers that subserve normal cortical function. The results can
be quite dramatic, with complete disappearance of the epileptiform activity on the EEG and rapid return of language
function. Despite the rather extensive transections undertaken in some patients, few postoperative deficits have
been noted. Other surgical approaches in selected patients include lesionectomy, corpus callosotomy, and
hemispherectomy.
Q2

Hypoxic ischemic encephalopathy due to cardiac brady-arrythmia.

Sequelae:

Lance adam syndrome

Cognitive dysfunction

Can go to vegetattive state, MCS

Critical illness myopathy and neuropathy

Ventilation associated complications: VAP , treacheal stenosis, bed sores

Management:

Hypothermia: 32-34 degree celsius trough cooling and preventing shiverin

Seizures: myoclonic usually respond to valproate or clonazepam

Supportive care

Q4

Webber syndrome

Base of midbrain

Neurologicak signs : EOM, pupil, acomodation vision , craial nerves, cerebellar signs, sesnory loss on face and hemi
body , swallowing assesmnet , visual fields, fundoscopy also for htn and dm changes,

Systemic , Bp , cardiac auscultation , carotid auscultation , lung exam ,signs of dm or peripheral vd.

Investigations :

MRI brain with Dwi/adc, hyper on dwi , hypo on adc map

BSR

Hba1c

Fasting cholesterol

Echo

Carotid doppler

Q5

Other symptoms: visual loss , jaw claudication , headache , proximal weakness , myalgias

Enlarged and tender temporal artery, diagnosis , temporal artery and PMR

Investigations,

ESR, temporal artery biopsy , steroids, stomach protection , bone density monitoring , Bisphosphonates

Q6

Refsum disease
DDs

Neuropathy: Krabbe disease

Metachromatic leukodystrophy

Fabry disease

Visual loss: Leber

Leigh syndrome

Defect is in peroxisomal function resulting in phytanic acid accumulation

pahx and pex7 genes

Clinical diagnosis is confirmed by the finding of increased phytanic acid in the blood of a patient with a chronic, mainly sensory
neuropathy; the normal level is less than 0.3 mg/dL but in patients with this disease, it constitutes 5 to 30 percent of the total fatty
acids of the serum lipids. Urinary phytanic acid concentration is also raised. Genetic testing reveals the mutation, which is in
PHYH in 90 percent of cases and in PEX7 in the others
Q1

Nocturnal frontal lobe epilepsy/RBD

Causes:

Autosomal dominat inheritance

Sporadic

Focal cortical dysplasia type 2

DD’s

●NREM parasomnias (eg, confusional arousals, sleep terrors, and sleepwalking)

●REM sleep behavior disorder (RBD)

●Periodic limb movements

●Psychogenic nonepileptic spells

●Nocturnal panic attacks

●Other sleep related movements (eg, rhythmic movement disorder, hypnic jerks)
 Management:

AEDs: clonazpeapam in non confirm diagnoses can be used

Carbazepine is first line , oxcarpazepine , lamotrigine and lacosamide are also effective; 1/3 may have resistent
epilpesy

Sleep hygeine , maintainence of goof sleep routine , poor sleep increases incidence of seizures.

Care during sleep, matress in open space or floor away from furniture so patient doesn’t get hurt

PSG with EG and EMG

Long term EEG

MRI brain with epilepsy protocol

Q2

Dengue virus encephalitis

Q1

HSP/ Segawa

Q2

Two daignoses
: vascular parkinsonism, NPH

MRI brain : look for vascular disease, strokes, binswanger, disproportionate dialted ventricles, any SOL, B12 levels, CBC
for aneimia ,CJD changes , infections, CSF analysis csf pressure , CSF for VDRL,

Treatment:
Q3

Collet siccard syndrome

Tumour at bas of skull lesion at anterior occipital condyle

DDs

Jugulr foramen syndrome-12th nerve)

Villaret syndrome ( plus horner syndrome lesion at retropharyngeal space)

Q7

Serotonin syndrome

Q8

Stiff person syndrome

Q5

CP angle tumour

Q6

Tardive dystonia