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Flaccid paresis
Mech:
It occurs as a result of interruption of motor unit system along its course (ant horns or roots, plexus or
peripheral nerves.)
It is characterized by:
Decreased overall strength
Hypotonia/atony of muscle
Hyporeflexia/ areflexia
Neurogenic muscle deterioration
Fasciculations of muscles
If damage is in the plexus or peripheral nerve, it is acc by sensory loss.
Diagnostical variants:
Radicular syndromes
Peripheral nerve lesion (Wrist drop <radial nerve>, claw hand <ulnar nerve>)
Plexus damage
Sp cord damage
Q2. Cluster headache
It is thus named because of its seasonal nature, is common in men and manifests as severe, stabbing,
periorbital pain, unilaterally. Accompanied by ipsilateral tearing, blood shot eyes, nasal congestion and
rhinorrhea, partial Horner’s. Unlike patients with migraine CH pts prefer to keep moving/pace.
It may either be episodic or chronic
Duration: 30mins-3hrs, esp. at night
Rx: divided into
Symptomatic Prophylactic
In cases of chronic cluster headache (i.e. cycle of headache is more than 6mnths without remission);
Lithium is reported to be effective. Side effects include confusions, drowsiness, seizures, thirst and
rashes.
Combination therapy is also beneficiary. Surgical treatment is indicated in patients with cluster headache
that is unresponsive to treatment. Trigeminal nerve surgery is carried out:
Gangliotomy or pre-ganglionic nerve- root section. These provide numbness tho pts still feel cluster
headache sensations, pain is absent.
Psychogenic headache:
Unlike the name suggests, the patient is not psychotic. The symptoms are constant and localized in a
discrete area.
• Pain which can be pointed at by a finger
• Sensations are described by delusional overtones. “ I feel lump on my skull”, “ my bones are
rotting”
• Negative tests don’t reassure pt
• Willing undergo investigations.
Q3. Classification of hereditary neurological Dx
1) Single gene disorders
Autosomal dominant: Huntington’s disease
Autosomal recessive: cystic fibrosis
X –linked traits: Duchenne’s syndrome
X-dominant: phosphate diabetes
2) Chromosome anomalies 3) multifactoral disorders
Structural cancer
Deletion rheumatism
Microdeletion atherosclerosis
Duplication hypertension
Inversion
Iso chromosome
Translocation
Ring shaped
Numerical
Aneuploidy (Down’s)
Polyploidy
4. PHM progressive her myopathy
Duchenne
Myasthenia
5. Neurohereditry amyotrophies
ALS
Syringomyelosis
6. Pathology of extra pyramidal system
Q4. Spastic paralysis
This is evidence of involvement in damage of CNS structures (brain and sp cord) and occurs as result of
pyramidal tract lesion as well as extrapyramidal. Spastic palsies are characterized by presence of spastic
finger/toe signs (e.g. Babinski’s), absence of skin reflexes e.g. abdominal and cremasteric reflexes. If the
lesion is after the decussation there is ipsilateral hemiplegia and if the lesion is bilateral, tetraplegia.
Gen signs
Decreased strength and loss of subtle mvmnt
Hypertonia
Hyperreflexia (Exaggerated proprioceptive reflexes)
Decr or loss of extraproprioceptive reflexes
Presence of pathological reflexes
No degenerative atrophy
Diagnostic variants:
• Subcortical lesion: contralateral monoparesis of hand
• Internal capsule: spastic contralateral hemiplegia
• Peduncles: same as abv + Oculomotor paralysis
• Pons: contralateral hemiplegia + ipsilateral paralysis of trigeminal etc
• Pyramidal lesion: contralateral, flaccid hemiparesis
• Cervical: ipsilateral, spastic hemiplegia
• Thoracic: spastic, ipsilateral monoplegia of leg( if damage is bilateral paraplegia)
• Ant root: flaccid, ipsilateral plegia
• Alternating syndrome: if lesion involves decussation.
Q5. Meningococcal infection
Acute inflammation of subarachnoid space and meninges characterized by presence of PMN in CSF.
Infection may be direct (sinusitis) or indirect (blood)
Etiology
Neisseria meningitis
Mixed infections may occur after head injury, mastoiditis or after lumber puncture.
The blood brain barrier limits host defenses thus promoting bacterial multiplication. Purulent exudate
extends thru out subarachnoid space. The pia mater normally protects against brain abscess formation.
Vascular structures may also be affected producing vasculitis: arteritis, venous thromboembolitis=
infarction.
Clinical
TRIAD: Fever, headache, neck stiffness are the classical picture.
Meningitic symptoms: severe fronto-occipital headache, stiff neck, photophobia
Meningitic signs: +ve kernig’s symptom, neck stiffness
Systemic signs: pupuric or petechial skin rash, high fever
Neurological signs:
Impaired consciousness
Seizures (focal& generalized)
CN signs
Direct cochlear involvement (sensorineural deafness)
Focal neurological signs (hemiparesis, dysphasia, and hemianopia)
Non neurological signs:
Inappropriate ADH
DIC
Arthritis
Endocarditis
Diagnostics
1. CT scan : to exclude intracranial mass
2. Lumber puncture
3. CSF exam
Protein
Decreased glucose levels
Culture(gram –ve cocci)
Color change (yellowish)
4. Blood analysis: neutrophillic leukocytosis
5. X-ray to detect source of infection e.g. Chest- pneumonia, Skull- fracture
Rx and prophylaxis
Once meningitis is suspected Rx is started IMMEDIATELY. ABiotics must penetrate BB- barrier.
Initial blind therapy: ampicillin/ penicillin G + cephalosporin (+ aminoglycoside in neonates)
Thereafter according to causative agent. Duration of Rx is 1wk
Remove any source of infection, and in the critically ill supportive therapy.
Q6. Myasthenia gravis, Myasthenia syndrome
This is a disorder in neuromuscular transmission resulting in fatigue of skeletal muscles esp. ocular,
oropharygeal and facial musc. When edrophonium Cl is introduced there is drastic muscle strength
increase
The forms: Neonatal, congenital, drug induced autoimmune forms.
Clinical features include:
Class 1- only ocular muscles
Class 2- mild generalized weakness
Class 3- moderate generalized and mild to moderate ocular-bulbar weakness
Class 4- severe gen and O-B weakness
Class 5- myasthenic crisis( resp failure etc)
Diplopia & ptosis
Dysarthria, Dysphagia, jaw mus weakness + fatigue of palate musc= myasthenic voice
Strengthless, cannot walk.
Myasthenic syndrome: a disorder of neuromuscular jxn caused by AB dev against Ca++ channels.
It is characterized by
Primary weakness of lower then upper limbs
Fatigue but unlike M.G the ocular and bulbar mus are unaffected.
Reduced tendon reflexes
Atrophy of affected musc
Cholinergic dysfunction (impotence, excessive sweating, and xerostomia).
Anti cholinesterases don’t relieve muscle weakness.
Emergency cases (myasthenic crisis & cholinergic crisis)
Primary care includes:
ID and treat primary cause e.g. infection, drug overdose.
Improve ventilation(intubate, improve posture as required)
Specific Rx:
Neostigmine(anticholinesterase) IV 8-12 mg/24hrs
Atropine(to counteract cholinergic effects)
Prednisolone 100mg daily
Plasmapheresis or IVIG(immunoglobulin G, IV)
Management:
Main aim is to restore or maintain breathing:
1. clear airway
2. sit pt at 450
3. Give nasal O2, and intubate and ventilate for as long as required.
Q7. Brown-Sequard syndrome
Occurs in cases of partial/ unilateral lesion of the spinal cord, e.g. in cases of tumors. Complete
hemisection is rare.
Motor deficit: dragging leg and in higher lesions (cervical) weakness of finger
mvmnts on side of lesion.
Upper motor neuron signs (esp. in side of lesion): extensors of arms and flexors in
legs paralysis, increased reflexes, increased tone and extensor plantar response. i.e.
ipsilateral spastic paralysis below level of lesion
Sensory deficit: numbness on side of lesion and contralateral dyaesthesia i.e. joint
position sense and touch localization is impaired ipsilaterally while pain and temp
sensation is impaired contralaterally.
Q8. Sciatic nerve neuropathy
It is formed by union of the common peroneal and tibial nerves. It passes through the greater sciatic
foramen and in the popliteal fossa splits to form its constituent nerves. The nerve descends btwn the
ischial tuberosity and the greater trochanter (femur). It innervates the hamstring mus of the thigh (semi
tendinous, membranous and biceps femoris)
Causes of damage:
Hip dislocation
Penetrating injuries
Incorrect IM injections
Entrapment
Systemic causes( Diabetes mellitus, sarcoidosis)
Symptoms:
Muscle weakness
Loss of knee flexion
Distal foot and leg muscle weakness
Ankle reflex is absent
Sensory loss in outer leg.
Rx:
Treatment of primary cause
Physiotherapy
Knee brace
Q9.Modes of inheritance
Autosomal dominant:
Autosomal recessive:
Sex-linked:
Q10. Alternating paralysis in mesencephalon lesion
Mesencephalon (midbrain) consists of 4 parts:
Tectum
Tegmentum
Sub nigra
Cerebral peduncles
In case of lesion involving the decussation of pyramidal tracts an alternating spastic paralysis develops.
The fibres of the tracts cross at the decussation therefore, in case of a lesion half the fibres which crossed
innervate one side each, and one limb on each side is paralysed.
• Lesion of Oculomotor nerve: Opthalmoplegia
• Lesion of Trochlear nerve: strabismus(cross eyedness), downward Diplopia, Nystagmus,
Notangelic symptoms: choreic hyperkinesia, paralysis of extremities, disbalance, cerebellar distress.
Parino syndrome: vertical stare paresis, abs of convergence, two sided ptosis
Red nucleus: intentional hemi tremor, hemi hyperkinesia
Clod (inf red nuc): ptosis, divergence strabismus, ipsilateral midriasis, intentional hemi tremor,
hypomyotnia contralaterally
Fua (sup red nuc): same as red nuc.
Substantia nigra: hypermyotonia, akinetic rigidity contralaterally
Tegmentum: ipsilateral; ataxia, Claude- Bernard syndrome, tremor, myoclonia. Contralateral
hemanesthesia and colliculi reflex abs
Weber syndrome: flaccid paresis of CN III ipsilateral with hemiplegia contralateral side
Benedict’s syndrome: paralysis of CN III; ipsilateral ptosis, divergence strabismus. Contralateral; atheoid,
intentional tremor
Q11. Poliomyelitis
An acute viral infection affecting the ant horn cells of spinal cord and the motor nuclei of the brainstem
Etiology:
Polio virus, an RNA containing enterovirus. 3 strains exist (coxsackie and echovirus). Fecal oral
transmission, 5-35 days incubation period.
Clinical picture:
Stages
1. Preparalytic: fever, sweating, malaise, headache, GIT upset. This stage may improve or progress.
2. Severe headache, back and limb pain, mus tenderness, meningism, may improve or progress to
paralytic stage.
3. Paralytic:
Spinal form Brainstem form
Mus fasciculation pharyngeal, facial, laryngeal, lingual mus weakness
Pain increases cardiac & resp mus involvement
Paralysis develops
Resp mus involvement
Diagnosis:
Feces
Nasopharyngeal secretions
CSF examination (polymorphs and lymphocytes incr, protein elevated, norm glucose) + Clinical picture
are enuf to confirm diagnosis.
Serological tests and virus isolation for later confirmation.
Exclude:
Acute meningitis (during meningial stage)
Guillian- Barre syndrome( paralytic stage)
Rx and prophylaxis:
Bed rest with well monitored fluid balance
Ventilation
Physiotherapy and splints in case of limb deformities
Sabin and Salk vaccinations are available
Clinical forms:
1. sub-clinical + resultant immunity
2. mild+ resultant immunity
3. meningism without paralysis(pre paralytic+ ri)
4. meningism with paralysis( paralytic + ri)
Post-polio syndrome: years after og Dx; malaise, fatigue, myalgia
Q12. Progressive myopathies
Erb-Goldflam Dx
Becker’s dystrophy
Dejenrine’s dystrophy
Duchenne’s dystrophy:
X-linked recessive disorder affecting boys
Clinical:
delayed motor dev
clumsiness( 3-5 yrs)
waddling gait(glutei mus involvement)
sway back posture
Gower’s sign(difficulty in rising, child “climbs up” himself to get up)
Kyphosis in later stages(age 20)
Pseudohypertrophy usually occurs, with enlarged, rubbery hard gastrocnemius muscle, quadriceps and
deltoid may also be affected.
Investigation:
Musc enzyme creatine kinase
ECG
EMG
Musc biopsy
Tx
Orthopedic Tenotomy may prolong mobility
Steroids
Intrauterine diagnosis+ abortion
Q13. Pallidar system and its lesion. Parkinson’s Dx.
The pallidum is a gray structure that develops from the 3rd ventricle and is related to the subthalamic
nucleus. It is covered by the putamen laterally and together they are known as the lentiform nucleus. It
also forms part of the extrapyramidal system. Formerly it was known as the paleostriatum and acted as
the center of lowest order of the brain.
Lesion produces:
Hypokinesia-hypertonia syndrome (Parkinson’s Dx, paralysis agitans)
Akinesia: hypomimia, only eyes move; head doesn’t, speech becums monotonous, body
is stiff, arms don’t swing when walking
Rigor: cog wheel phenomenon upon attempts to relax limbs, head drop test (head falls
slowly back when raised). No pathological reflexes
Tremor: pin rolling tremor that disappears with intention.
Types of tremor: parkinsonian, intention, posture/static, essential
Bilateral lesion is known to produce severe consciousness disorders and delirium or amentia.
Hyperkinesia-hypotonia syndrome
Athetosis: slow, writhing, worm-like involuntary mvmnts. Grimacing and abnorm tongue
mvmnts, spasmodic outburst of laughing or crying
Chorea: short, fast involuntary jerks in single muscles randomly. Characteristic decrease
in tone
Spasmodic torticollis: tonic disorder of neck muscles= slow involuntary turning of head
Torsion dystonia: extensive turning and twisting mvmnts of the trunk and proximities. Px
may not be able to stand or walk.
*Q14. Vascular encephalopathy
Q15. Progressive Dx of peripheral nerves. Charko’s neural amyotrophia & Hoffman’s
amyotrophia.
The cause of motor neuron disorder is unknown but it is linked with: ageing, viruses, toxins, mineral
deficiency, genes, and excitotoxins.
Clinical features:
Frontal dementia
Pseudobulbar palsy
Progressive bulbar palsy(tongue has atrophy, fasciculations, looks wrinkled and wasted,
gag and jaw reflex are absent)
Corticospinal involvement(brisk reflexes, incr tone)
Progressive mus atrophy(weakness, atrophy and fasciculations=skeleton hand)
Ultimately resp mus become involved= death.
Rx is symptomatic:
Spasticity; balcofen & tizanidine
Physiotherapy
Riluzone
Hoffman’s infantile amyotrophia:
Autosomal recessive
Characterized by:
Reduced fetal mvmnts during gestation
Weakness and hypotonia at birth
Hypotonic posture(arms and legs abducted and externally rotated)
Contractures, wasting and fasciculations
Death by 18 months
Q16.Bulbar and pseudobulbar paralysis
Bulbar Pseudobulbar
Medical therapy
(a) anti aggregants
(b) anti coagulants
Surgery
Q24. Hereditary ataxias. Friedrich’s ataxia
Dominantly inherited:
ACDAs:
Type 1 Type 2 Type 3
Mild ataxia Expressed ataxia Only ataxia
Opthalmoplegia Expressed retinopathy
Mild dementia Dementia
Optic atrophy Extrapyramidal features
Spasticity
Recessively inherited:
• 1Louis-Barr syndrome characterized by cerebellar ataxia, multisystem disorders and
ocular, cutaneous teleangiectasia due to chromosome 11 gene defect.Purkinje and
granular cell loss occurs
2Friedrich’s ataxia (chromosome 9 defect)
Damage of spinocerebellar tracts, posterior funiculi and pyramidal tracts. Symptoms are caused by the
damage to various systems
Characterized by loss of:
• position sense
• discrimination
• stereognosis
Clinic:
• +ve Romberg’s sign & ataxia
• Pes cavus( Friedrich’s foot)
• Kyphosis/ scoliosis
• Limb weakness
• Abs of abd reflexes
• No Babinski’s sign
• Abs of lower limb reflexes
• dysathria
Diagnostic variants:
Mary’s spastic ataxia + spastic paraparesis
Strumpell- Lorraine syndrome
Q25. Gait infringement. Ataxia diff diagnosis and clinic
1. Ataxic
• Sensory
• Cerebellar
2. Hemiplegic
3. Parkinsonian
4. steppage
5. myopathic
6. frontal lobe
7. hysterical
8. cautious
9. choreic
10. dystonic
11. spastic
12. toe walking
Ataxia
Sensory ataxia:
Unconscious/ disturbed. No proprioception
+ve Romberg sign
Cerebellar ataxia:
Feet set wide apart when standing or walking.
Steps are jerky and unsure. Heel-to-toe walking is impossible.
-ve Romberg sign
Thalamic ataxia:
Contralateral ataxia with tendency to fall backwards or to opposite side of lesion
Spinal ataxia:
Ataxia and disequilibrium with Spasticity
Ataxia teleangiectasia:
(Louis Barr syndrome)
Progressive cerebellar ataxia with cutaneous and ocular teleangiectasia and immunodeficiency
Pts confined to wheel chair
Friedrich’s ataxia:
Damage of spinocerebellar tracts, posterior funiculi and pyramidal tracts, Symptoms are caused by the
damage to various systems
Characterized by loss of:
• position sense
• discrimination
• stereogenesis
Clinic:
• +ve Romberg’s sign
• Pes cavus( Friedrich’s foot)
• Kyphosis/ scoliosis
• Limb weakness
• Abs of abd reflexes
• No Babinski’s sign
• Abs of lower limb reflexes
Peripheral paralysis
Viral infections, vascular disorders, otitis media, mastoiditis, petrous bone fracture may cause this,
leading to flaccid paresis of all mimic musc including forehead musc.
Post recovery, preserved fibres send new axons to the damaged portions leading to faulty innervation wch
may cause:
• Contracture
• Synkinesis
• Crocodile tears( paradoxical gustolacrimal reflex)
Variants of facial nerve lesion
1. contralateral supranuclear lesion: unilateral lower face with normal eye closure
2. ipsilateral nuclear lesion
3. bulbar/ pseudobulbar palsy
4. nuclear lesion: pons, internal auditory meatus, facial canal, peripheral nerve
lesions
Other disorders incl Bell’s palsy, Ramsey Hunt syndrome, hemifacial spasm
Ramsey Hunt syndrome:
Herpes zoster infection with characteristic zoster eruptions in the auditory meatus, it has sudden facial
weakness and pain. Sero sanguinous discharge is present from ear, as well as deafness (damage to CN
VIII)
Hemi facial spasm:
Esp. in mid aged females, characterized by unilateral clonic spasms of m orbicularis oculi. Also ipsilateral
clicking sound (os. Stapedius). Contractions are worsened by stress.
Q35. Hemorrhagic stroke, SAH
Etiology:
Cerebral aneurysms
Small thrombosed aneurysms
Hypertension
Clinic:
Headache
Disturbed consciousness
Nausea & vomiting
Meningeal signs
• neck stiffness
• +ve kernig’s
• coma
• epileptic seizures
• focal signs
• papilledema
• pyrexia
Diagnostics:
CT, LP, MRI, MRA, angiography
Tx:
Evacuation of clots ,endotracheal intubation, stabilize arterial pressure and cardiac rhythm
Medical tx- use triple “h” therapy i.e induce ( inorder to prevent vasospasm) hypertension,
hypervolemia, hemodilution. To maintain perfusion to cerebrum . use calcium blockers
Kleinfelters Dx
Genotype: 47XXY, 48XXXY, 49XXXXY
Or 47XYY, 48XYYY
Typically males
Clinic:
Onset at puberty
Hypogonadism
Sterility
Externally; tall, asthenic, gynecomastia, long limbs
Turner’s syndrome
Genotype: 45XO
Typically females
Clinic:
Short stature
Hypogonadism
Amenorrhea
Sterility
Poor secondary sexual characteristics
Webbed neck, cubitus valgus
Edema of legs
Coarctation of aorta
Q37. Olfactory system, lesion, topical diagnosis
Sup. Regio olfactoria+ nasal septum------bulbus olfactorius -------trigonum olfactorum---- ant perforated
sub------- temporal lobe fila olfactoria
1st neuron= bipolar olfactory cells
2nd neuron= mitral & tuft cells of bulb
3rd neuron= n. amygdaloideus
Lesion of olfaction:
1. Agenesis of olfactory tracts
2. Dx of upper olfactory mucosa
3. Fracture of lamina cribrosa causing tearing of fila olfactoria
4. Destruction of bulb by contusions e.g. falling on back of head
Anosmia (uni or bilateral) may be the only neurological sign of damage. Often patients do not realize
their loss of olfaction and instead complain of loss of taste.
Diff diag:
Impairment maybe temporary or permanent
Extramedullary tumor:
Originate in area of post roots and produce Radicular pain as they grow.
Dorsomedial growth: press on post tracts, roots, and pyramidal tracts
Clinic:
Ipsilateral spastic paralysis with paraesthesia& defects in proprioception, coughing and sneezing incr pain
(rheumatic felt in distal limbs 1st)
• Tumor in foramen magnum= pain, para- and hyperesthesia
• Tumor in area of C2= paresis of SCM & trapezius (accessory nerve)
• Hour glass tumor: neurinoma in intervertebral foramen growing to the outside & sp cord.
Produces compression symptoms and later brown Sequard syndrome.
Hypoesthesia in corresponding dermatome
Bladder and rectal paralysis
Ventral growth: press on ant roots
Clinic:
Flaccid paresis of hands, spastic paralysis if pyramidal tracts r involved
Hyperesthesia if pressure is on ventrolateral funiculus
Incontinence of bladder and rectum
Extrinsic;
Acoustic Schwanoma :( nerve sheath tumors) cerebellopontine angle lesions, bilateral chrct for NF2, they
r benign, damage CN VIII
Tx: translabyrinthine & middle fossa approach
Dermoid cysts: rare, embryogenic cysts. They adhere closely so can’t be removed, evacuation can be
done & steroids) clinic: Depressed corneal reflexes, chemical meningitis (rupture of cholesterol into SA
space)
Q49. Pupillary accommodation
Ciliary contract, rectus contract, pupillary constriction
Aniso conia
Pupil constriction is characteristic of:
Horner’s syndrome:
Ipsilateral mild ptosis, miosis, sweating disturbance depends on site of lesion: if lesion is on proximal site
of fibres btwn int carotid= ipsilateral.
Levels of lesions: break my car I arrest
Brainstem
Mid fossa
Cervical Cord
int carotid artery
ant roots of C8- T1
Congenital familial Horner’s syndrome: loss of iris pigmentation
Preganglionic & post ganglionic lesions differentiation
In preganglionic: Intra ocular cocaine, receptors take up cocaine= dilation (in norm they take up adrenalin
and constrict)
In post ganglionic there r no receptors
Argyle Robertson (ass with syphilis)
Irregular, small pupils unresponsive to light, but can accommodate. May result from mid brain Dx,
diabetic neuropathy. Also motor nuc of Oculomotor is affected.
Diagnosis:
Underlying syphilis
Serological tests
Tx
Para-sympato-mimetic drugs: carbacol, opiates
Q50. Transversal myelitis
Rare, occurs in ass in EBV, small pox, viral infections
Clinic:
Fever, back and limb pain (b4 paralysis)
Paralysis is first flaccid then spastic (after 1-2 wks)
Bladder disturbance
Diagnosis:
LP
Check for underlying cause
Myelography
MRI
Tx
Supportive
Steroids
Q51. Brain – arterial aneurysm
Unusual swelling or dilation of vessels wch may rupture.
Shapes: saccular, fusiform, and mycotic
1. Saccular may be found in
Ant cerebral & communicans: leg weak, incontinence, confusion
Medial cerebral artery: hemiparesis hemiplegia dysphasia
Post inf cerebellar artery & basilar: asymptomatic
In case of rupture severe headache + SAH
2. Mycotic may be traumatic or vascular (carotid, vertebral, basilar)
3. Fusiform maybe atherosclerotic on basilar & int carotid: neuralgia
They rupture easily and cause carotid cavernous fistula (ipsilateral exopthalamus synchronous with pulse,
swelling eyelid paralysis of eye muscles
Aneurysm of intra cranial carotid affects optic chiasma, tract & nerve- visual field disturbance
Post comu artery- Oculomotor palsy
If aneurysm bursts:
SAH, incr ICP, headache, confusion, nausea, neck stiffness
If blood penetrates tissues= intra cerebral hematoma
Diagnostics
CT
Tx
Symptomatic
Surgery: clip neck of aneurysm
Q52. Caudal cranial nerves syndromes
Glossopharyngeal nerve (caused by: med or nerve root lesions norm ass with X-XI lesions= jugular-
foramen syndrome)
1. Glossopharyngeal neuralgia
Short, sharp pain (identical to tic douloreux) affects post pharynx & tonsillar area irradiates to ear, incr by
swallowing. Stimulation of vagus= bradycardia & syncope (Carbamazepine for relief)
2. Vagus disorders
Palatal weakness: uni/ bilateral
Pharyngeal weakness: uni/ bilateral
Laryngeal weakness
3. Accessory disorders
Unilateral paralysis of SCM (can’t turn to opp side); bilateral (can’t hold up head)
Trapezius paralysis (hanging shoulder & caudo lateral scapula)
4. Hypoglossal disorders
Lesion leads to atrophy & deviation of tongue to weak side
Jugular- foramen syndrome: lesion of CN IX-XI
Callet-Sicard syndrome: extra cranial lesion involving CN IX-XII
Villaret’s syndrome: retropharyngeal space lesion involving CN IX-XII & Horner’s syndrome
Q53. Intramedullary tumors
Not common, e.g. astrocytoma
Clinics:
Segmental pain
Loss of pain & temp at level of lesion (interruption of decussation)
Sensory loss
Tumor expansion: involve ant horn cells= l.m.n weakness, Corticospinal tract inv=u.m.n weakness
Diagnosis:
X-ray shows widening of interpedicular distance, Myelography confirms lesion, MRI differentiates from
syringomyelias
Tx: exploratory laminectomy, needle biopsy, aspiration, after OP; radiotherapy
Q54. Structure and classification of chromosomes, Down’s syndrome
Each chromosome contains a single DNA molecule organized into several orders of packaging =
metaphase chromosome.
Chromatin: DNA protein complex made up of histones (5 types), 8 histones = nucleosome. Nucleosomes
are tightly packed together to form a 30nm wide fiber (can be seen under electron microscope)
Special features
Euchromatin has high density coding regions or genes
Heterochromatin devoid or inactive genes maybe constitutive and facultative (inactive X chromosome
remains highly condensed and stains darkly during interphase = Barr bodies)
Centromere constricted area of metaphase chromosome
Telomere DNA sequence at ends of chromosomes
Classification
Normal karyotype = 23 pairs of chromosomes (22 homologous autosomes and 1 sex)
Chromosomes may be metacentric, submentacentric and acrocentric satellites
Bands: Starting at centromere each arm is divided into one or more regions
Down’s syndrome:
Trisomy 21 affects both males and females
Clinic:
Typical Mongolian face (moon face, small mouth, large tongue, close skewed eyes)
Males sterile but females fertile
Prenatal: nuchal thickening, duodenal stenosis and short femur
Infancy: flat nasal bridge, Brush Field spots, flat occiput
Childhood - adulthood: mental retardation, autoimmune disorders, hearing loss, short
Old age: Alzheimer’s pre-senile dementia.
Q55.Oculomotor nerves, Post longitudinal fascicle, lesion of Oculomotor
Motor nuclei- front part of periaqueductal gray matter
Autonomic (Edinger- Westphal) nuclei- poorly myelinated periaqueductal gray matter
PSNS nuclei (Perlia’s) - between the autonomic
INNERVATION:
Motor= mm recti sup, inf, medial, m. inf orbicularis
Autonomic= m. sphincter pupillae, m. ciliare
Longitudinal fascicle:
Constitutes a collection of various fiber systems connecting nuclei abducens, Trochlear and Oculomotor
It passes thru sp cord, pons & mid brain and carries impulses from the vestibular system.
Particulate lesion causes displacement of eye globe in horizontal (divergent strabismus) and vertical
planes (one eye medial and downward; on side of lesion the other is lateral and upward)
Also Nystagmus maybe present.
Lesions:
Complete lesion produces
Ptosis, dilated unresponsive pupil and fixed eye
Partial lesions produce only part f the syndrome e.g. opthalmoplegia
If all mus are paralysed= peripheral nerve damage
If only one mus= lesion in nucleus of Oculomotor nerve
Clinic:
If CBF is maintained, ICP may show symptoms but not neuronal damage.
Headache, vomiting, papilledema, vomiting, brainshift
Diff diag:
Brain abscess, trauma, tumor, shift, MS, hematoma, meningitis, migraine
Tx
Remove expanding mass
ICP level > 30mmHg (norm= 0-10) requires Tx
Methods of decr ICP:
100ml, 20 % mannitol/ 15 mins a bolus
Controlled hyperventilation
CSF withdrawal
Sedatives
Steroids
Q62. Cervical osteochondrosis
Compression, shrinkage, saddle formation
Progressing atrophy of cervical disks and approximation of vertebrae
P/genesis:
Cervical Radicular syndrome is almost always a result of CO
Osteochondrosis of cervical column= C3-T1, the spaces btwn 5th & 6th as well as btwn 6th & 7th most often
involved.
These syndromes consist of Radicular irritation= paraesthesia & pain in segmental distribution, if more
severe; = Radicular sensory and motor loss ass w abnorm reflexes
Syndromology
C4- C5
Pain & numbness radiating to shoulder
Weakness of deltoid
C5- C6
Pain & numbness of top of neck, biceps, lateral arm, dorsal thumb & index
Weak biceps with decr reflex
Weak wrist flexion
C6- C7
Pain in top of neck, triceps, postero lateral arm to middle finger
Weak triceps, decr triceps reflex
C7- T1
Pain & numbness acr neck, arm, to small and ring fing
Wrist flexion and intrinsic arm musc r weak
Diff diag
MS
Trauma
Metastatic cancer of cervical spine
Rheumatoid arthritis
Osteomyelitis
Tx
Steroids
Muscle relaxants (500mg, PO/ 6 hrs)
Physiotherapy
Rest
Soft cervical collar
Microvascular decompression
Q63. Trigeminal neuralgia
Paroxysmal attacks of short, stabbing pain. Always unilateral
Etiology:
Root compression
Demyelination
Diagnosis
CT/MRI
Tx
Drug therapy: Carbamazepine
Pain: analgesic
Surgery:
Nerve block, root section
Q64. Lesion of right internal capsule
Contralateral spastic hemiplegia
Contralateral paralysis of facial and hypoglossal nerves (corticonuclear tracts)
A lesion causing rapid damage produces a contralateral paralysis dat is at first flaccid then it becums
spastic as the extrapyramidal tracts r damaged.
Q65. Lateral amyotrophic sclerosis (ALS)
Chronic progressive degenerating Dx involving motor neurons in sp cord and brain
Etiology
Viruses
Excitotoxins
Genetic predisposition
Toxins
Minerals
Forms
I. Cervical: spastic paresis in legs, flaccid in arms, dysphagia
II. Bulbar: dysarthria, tongue fasciculations, pyramidal symptoms
III. Pseudobulbar form: Dysphagia, pathological laughing and crying, frontal dementia, l.m.n lesion
symptoms
IV. Lumbar form: flaccid paresis in legs, with atrophy & fasciculations
Clinic:
@ Onset: asymmetric weakness & wasting of extremities
Bulbar & pseudobulbar features
Frontal dementia
Pseudobulbar palsy:
• Weak musc of mastication & facial expression
• Incr gag& jaw jerk reflex
• Drooling speech
• Dysphagia
• Immobile pointy tongue
Progressive bulbar palsy:
• Atrophy & fasciculation in mus supplied
• Wasted tongue
• No jaw & gag reflex
Corticospinal involvement:
• Hypertonia
• Hyperreflexia
Ant horn involvement (progressive muscular dystrophy):
• Atrophy, weakness, fasciculations
• ‘Skeleton hand’
• Resp musc failure is normally cause of death
Tx
Riluzone 100mg/day
Symptomatic:
Speech therapy
Nutrition
Drooling- amitryptilin
Musc weakness- physiotherapy
Resp failure mngmnt
Q66. Extramedullary tumors
Meningioma
Localization: thoracic region, intra dural
Clinic:
Esp. in elderly females
Ipsilateral paresis
Sensory disorders
Diagnostics:
MRI or CT Myelography
Surgery: complete removal tho recurrence is possible
Neurinoma
Localization:
Any level in post nerve roots, either within sp canal or in ‘dumbell’
Clinic:
Ass with multiple neurofibromatosis
Café au lait spots
Root pain
Compression symptoms may follow
Diagnostics:
MRI, CT
Oblique X-ray: foraminal enlargement
TX
Complete removal, sacrificial coz other nerves compensate for lost ones
Q67. Lesion of frontal lobe
Lesion leads to:
Defect in each area!!!
Q68. Brain contusion
May occur under or on opposite side of injury, it may be multiple and bilateral
Consciousness is only lost when bleeding into contusion (hematoma)
Clinic
Loss of consciousness, edema, seizures, focal neuro signs, blood into CSF (meningeal irritation &
hydrocephalus)
Diagnosis:
Gen exam:
Injury, basal skull fracture
Consciousness (eye opening, motor & verbal)
CT/MRI
Tx
Extensive contusion w edema= focal mass lesion: surgery
A, B, C
Elevate head & give barbiturates and furosemide
Cx
Post traumatic epilepsy
Intracranial hematoma
Post contusional encephalopathy
CSF leakage
Q69. Main principles of hereditary Dx Tx
1. Amelioration of clinical phenotype: symptomatic Tx and surgery
( correction of congenital anomalies)
2. Amelioration of metabolic abnormalities:
3. Replacement therapy e.g. hemophilia (replacement of factor VIII)
• IDDM insulin therapy
• Organ transplant
3. Modulation of gene expression
4. gene therapy: germ line therapy and somatic therapy
5. diet ethics e.g. galactosemia: removal of milk and diary products
Non specific
• special education
• symptomatic medication
• avoidance of environmental exposure
Surgical
• correction of anomalities e.g. cleft
• organ transplantation
• gene therapy
Q70. Facial nerve
This nerve has 2 subdivisions:
The larger (facial nerve proper) motor nerve that innervates mimic musc of face and the thinner (n.
intermedius) that has autonomic and somatic fibers.
Diagnostics of level of lesion:
Lesion may occur at many levels, there are several branches of the facial nerve along its path;
1. Before and at the internal acoustic porus (2 branches: n Facialis proper & intermedius)
Peripheral motor paralysis of mimic muscles+ hearing impairment/ deafness/ decr vestibular excitability
2. between internal acoustic porus and 1st branch (n. petrosus majoris)
PMP + impaired taste, lacrimation & salivation
Tx
Anticonvulsants (monotherapy)
Surgery
Withdrawal if pt is fit free >3 years
Surgery:
1. Temporal lobectomy
2. hemispherectomy
3. corpus callosal section
4. extra temporal cortical resection
5. selective amygdalo-hippocamectomy
Diff diag
Syncope, hypertensive crisis, panic attack
Q77. Carotid- Arterio anastomosis
A fistulous communication btwn the int carotid and the cavernous sinus may follow trauma, rupture of
small intra cavernous artery
Clinic
Pulsating tinnitus
Prominent facial veins
Pulsatile exopthalamus
Edema of periorbit
Papilledema
Opthalmoplegia
Diagnostics:
Angiography
Fundoscopy
Tx
1. fistule repair
• spontaneous closure
• trapping( ligation)
2. direct op repair
3. Embolisation
Q79. Aphasia, Apraxia, Agnosia
Aphasia:
Loss/impairment of language processing caused by brain damage
Cortical centers for language in dominant hemisphere:
Broca’s area
Temporal lobe receptive area
Parietal lobe receptive area
Etiology: Stroke, focal or diffuse lesions
Clinics:
Nonfluency, auditory incomprehension
Paraphasia (substituting correct words with incorrect ones)
Agraphia, anomia, alexia
Topical diagnosis:
Check content and fluency
Comprehension
Assess spontaneous speech, writing, reading
Motor aphasia: pt understands bt cant speak, is aware of defect
Sensory: not aware, cant understand, inadequate speech
Amnestic: pt understands can speak but forgets names of pple, obj, places etc
Semantic: can understand some words but can’t link i.e. dsnt see relation
Total: severe includes wernicke’s, broca’s area
Agnosia:
Loss of comprehension of auditory, visual other sensations tho sensory sphere is intact
Auditory, colour, finger, optic, tactile, time
Anton’s syndrome: when one is no longer aware of opposite side can’t feel paresis(visual agnosia)
In case of dominant parietal lobe lesion agnosia is of fingers.
Geographical agnosia –non dominant parietal lobe lesion
Finger agnosia-dominant parietal lobe lesion
Apraxia:
Inability to carry out skilled mvmnt despite full understanding of task and full motor power
Constructional (non dominant parietal lobe)
Dressing apraxia (non dom)
Gait apraxia (frontal lobe/ corpus callosum Dx)
Oculomotor (parieto- occipital lobe)
Ideamotor (dom hemisphere) can’t carry out task when asked to but otherwise can perform it
Ideational (frontal lobe Dx) can’t do sequence but can do each action separately
Q80. Early syphilitic meningitis
The initial event in neurosyphilis is meningitis.
Clinic:
Asymptomatic
Aseptic (fever, rash, malaise, neck stiffness)
Acute basal meningitis (hydrocephalus, CN palsies)
Diagnostics:
LP
CSF: leukocytosis, incr protein
Tx
If symptomatic give penicillin
If untreated:
Meningovascular, spinal, optic syphilis, gen paralysis
Tabes dorsalis
Syphilitic gummae
Q82. Muscle tone
It is the resistance of muscle to passive movement of the joint. It depends on degree of muscle contraction
and mechanical properties of muscle and connective tissue. Muscle contraction depends on anterior horn
cells.
Types of disturbance:
Hypertonia, Increased tone. Can be:
Spasticity: Affects different muscle groups to different extents. Resistance increases when passive
movement is initiated. Increased tone is velocity dependent (spasticity is caused by UMN lesion e.g.
stroke)
Rigidity: Increased resistance independent of direction (lead pipe and cog wheel). Rigidity indicates
extrapyramidal dysfunction – lesion of basal ganglia.
Hypotonia:
Excessive floppiness, reduced resistance and easily waved distal limb (involvement of LMN e.g. primary
muscle disorder cerebellar Dx)
Paratonia:
Rigidity when limb is moved rapidly but normal when moved slowly (frontal lobe or diffused cerebral
Dx)
Q84.Brain abscess
Maybe:
Extradural
Subdural
Cerebral
Cerebral abscess
Hematogenous spread from inflammatory Dx
Local spread may be direct (penetration) or indirect
Site depends on source:
Chronic otitis media (temporal lobe)
Mastoiditis
Compound #
Frontal sinusitis
Infected dental caries
Etiology: strep. Pneumonia, s. aureus, proteus, toxoplasma
Clinics:
Toxicity produces- pyrexia, malaise
Raised ICP
Focal signs
Neck stiffness (coz of tonsillar herniation or meningism)
Q85. Pyramidal tract lesion
Sub cortical lesion: contralateral monoparesis of hand/arm wth jacksonian epilepsy
Internal capsule: spastic contralateral hemiplegia & paresis of hypoglossal and facial nerves
Peduncle: alternating syndrome; ipsilateral occulomotor palsy and contralateral spastic hemiplegia
Pons: contralateral/ bilateral spastic hemiplegia & ipsi abducens and trigeminal palsy
Pyramidal lesion-flaccid contralateral hemiparesis
Cervical cord: spastic hemiplegia wc is ipsilateral
Ant horn: ipsilateral flaccid paresis
Thoracic: spastic ipsilateral monoplegia of leg (if bilateral= paraplegia)
Q86. Migraine
Common, familial disorder affecting females mostly; it is a unilateral, throbbing headache.
With aura: warning sign; visual, sensory or motor, with headache worsened by light, noise, relieved by
sleep with vomiting and nausea.
Without aura: headache acc by tension
Mech: either neuronal or vascular
Types:
Basilar: bilateral visual symptoms, dysarthria, unconsciousness, vertigo
Hemiplegic: unilateral paralysis, often mistaken for stroke
Opthalmoplegic: extra ocular nerve palsies
Sometimes may be acc by coma: migraine coma
Enhancing factors: Diet (tyramines), hormones, stress, fatigue
Diagnosis:
Clinical history
Tx
Avoid risk factors
β- Blockers
5HT2 blockers
Anticonvulsants, antidepressants
In case of acute attack: analgesics, Sumatriptan, ergotamine, methlyprednisolone
Q87. Brain Meningioma extrinsic tumourl
Slow growing and arise in subarachnoid granulations, esp. around venous sinuses. Usually benign
Class
By localization
Falcine, convexity, olfactory groove, supra sellar, sphenoid wing, post fossa, tentorial
By histology
Syncytial, transitional, fibroblastic, angioblastic
Clinic
Epilepsy, headache, vomiting, papilledema, foot weakness, homonymous hemianopia, memory and visual
impairment, Foster- Kennedy syndrome (optic atrophy+ papilledema), ptosis, facial pain
Diag
CT, MRI, angiography
Tx
Remove tumor, catheterization and embolization (to ease removal)
Q88. Trigeminal nerve
n. opthalmicus: nn. Frontalis, lacrimalis, naso cilliaris
Ganglion trigeminalis sup orb fissure
n. maxillaries: nn. Pterygopalatini, alveolaris sup, zygomaticus
For. Rotundum
n. mandibularis: supplies masticatory muscles
For ovale
Syndromes:
Neuralgia (tic douloreux): sharp, agonizing, pain in region of supply. Esp. when touched and trigged by
washing, brushing teeth etc
Symptomatic pain: in territory of one branch, pain. Coz of infected tooth, sinusitis, fractures etc
Charlin’s syndrome: severe pain in inner corner of eye, pain in root of nose, lacrimation and nasal
secretion ipsilaterally. (Irritation of ciliary ganglion)
Gradenigo’s syndrome: pain in area of frontal branch, with abducens paresis
Bing- Horton’s: pain during sleep ass with reddening of ipsilateral face, lacrimation, nasal secretion and
Horner’s syndrome
Carotid artery aneurysm
Other Dx (meningitis, cranial dx)
Trismus: strong tension, pt cant open mouth due to encephalitis, tetanus, rabies
Q89. TB meningitis
Etiology:
M.TB, Bovis
TBM is common result of TB infection of NS
After bacteremia, metastatic foci lodge in 1. Meninges 2. Cerebral/ spinal tissue 3. Choroid plexus
Rupture of encapsulated foci leads to spread of infection to SubA space
Clinic:
Stage 1 Stage 2 Stage 3
Non specific symptoms confusion coma
Fever CN paresis
Lethargy meningism
Vasculitis
Seizures, chorea, myoclonus, dementia may occur.
Diag
General exam (anemia, leukocytosis)
Chest X-ray
Tuberculin skin test
CT/MRI
Tx
Anti TB TX (Iso, Rif, Pyr)
Steroid therapy
Q90. Methods of clinical genetics
Genealogical: pattern of inheritance, expressivity& penetrance, probability or abnormal off spring
Statistical: frequency of dominant & recessive alleles, intensity of mutations, distribution of defects and
prediction of their appearance in future
Gemellary: relation btwn genetic and environmental factors in mutation
Cytological: investigation of structure of chromosomes & their possible mutations
Q91. Sleep, fxns & physiology, disturbances
Sleep is produced as a result of stimulation of sleep producing centers in the brain (RF& raphe nuc)
There are two stages: REM & non REM sleep
Tx
Psychotherapy: cognitive, behavior therapy
Minor tranquilizers
Q95. Tunnel median nerve syndromes
C7- C8, lat median cords, supplies the palmar side of radial border of hand.
Causes
Entrapment under carpal ligament
E.g. rheumatoid arthritis, acromegaly, fluid retention, infiltration of ligaments
Symptoms
Pain esp. @ nite
Paraesthesia
Loss of sensory sensation
Mus wasting and weakness in thenar
Tinel’s sign: percussion of nerve at the wrist produces incr paraesthesia
Tx
Treat underlying cause
Diuretics
Surgery: division of ligament
Q96. Torsion dystonia
These are sustained abnormal postures produced by contraction of trunk & limb muscles.
TD has child hood onset, Autosomal dominant
Clinics
Extensive turning and twisting mvmnts of trunk and proximal extremities
May be idiopathic or symptomatic (encephalitis, Wilson’s Dx)
Abnorm posture of head trunk and limbs
Normal during sleep
Diagnostics:
EMG
Tx
L- Dopa
Carbamazepine
Anticholinergics
Stereotactic surgery:
Lesion in ventrolateral thalamus reduces dystonia in contralateral side
Q98. Neurological symptoms of alcoholism: neuropathy
Polyneuropathy is a common result of chronic alcoholism, it may occur alone or with Wernicke’s
encephalopathy etc Polyneuropathy is usually symmetrical, esp. in legs, methanol produces sudden
blindness.
Alcoholic neuropathy: sensorimotor neuropathy affects legs.
Mech: local compression of nerves during alcohol obtundation.
Radial compression in humerus spiral groove= wrist drop, peroneal nerve at head of fibula, ulnar nerve in
medial epicondyle; if deficit dsnt improve within 2mnths surgery or electrodiagnostic tx is indicated.
Clinic:
Progressive
Sensory symptoms: numbness, paraesthesia
Motor signs: foot drop, wrist drop, weakness
Diagnostics:
EMG: axonal damage
Incr liver enzymes
Tx
Stop OH, thiamine diet (100mg/day)
Q100. Subthalamic syndromes, panic attacks
The main structure: corpus luisi. Part of extrapyramidal system, in case of Corpus damage, there is
contralateral hemiballismus (pain attack)m
Borders: red nuc and sub nigra
1. lesion of all sensory pathways:
• Contralateral hemi anesthesia
2. lesion of trigeminal nuc & lat Spinothalamic tract
• Contralateral loss of pain & temp on face & body.
3. lesion of ant Spinothalamic tract& med leminiscus
• contralateral sensory loss with intact pain & temp
4. lesion of spino trigeminal nuc and tract & lat sp thalamic tract
• Loss of temp & pain on ipsilateral face contralateral body.
Q101. Tunnel syndrome of radial and ulnar nerves
Entrapment of a nerve in a tunnel where it passes:
Ulnar
Cubital or guyon’s tunnels
Causes:
Elbow trauma, repetitive mvmnt, and prolonged flexion
Clinical
(Cubital) claw hand
(Guyons)
In case of complete lesion dorsal and palmar sensory loss without fingers. In case of deep lesion motor
loss, in case of superficial only sensory loss.
Radial
Axillar groove
Post interosseous nerve
Sup sensory branch
Causes
(Axillar groove)
Bad use of crutches
Vigorous hand exercises
Clinic:
Weakness of muscles
Sensory loss on dorsal hand, thumb, index & mid finger
Wrist drop (no extension of hand & fingers)
PIN: same as abv
SSB: sensory loss in radial distribution
Q102. Neurofibromatosis
Dominant her Dx wch leads to multiple tumor formation in nerves.
Type 1(NF1)
Embryologic; overgrowth of mesoderm and ectoderm (meninges, skin, viscera, CNS, CVS)
Clinic:
Café au lait spots- demarcated edged spots wch grow with age
Scoliosis
Mental retardation & epilepsy
Neoplasia (intracranial, Intraspinal, peripheral nerves)
Lesch nodules
Mollusca fibrosa
Tx
Remove mass
Decr ICP
Normalize blood gases
Q105. Epilepsy, attacks, status Tx
Successive tonic/clonic seizures with gaps in between, if consciousness does not return during gap=
status epilepticus. It is life threatening, and may be caused by: pregnancy, frontal lobe lesions, metabolic
disorders, head injury. Types: tonic/ clonic, absence seizures, status epilepticus, grand mall, petit mal
e.t.c.
Tx
GENERAL
Oxygen therapy- 10litres /min
IV infusion of 500ml 5%dextrose/0.9N saline
Prevent hyperthermia
THE END!!!!!!!!!!!!!!