SSPE, myasthenia n LETM

transcript

CLINICAL CASE DISCUSSION

UNIT 6

GUIDES: Dr.A.Pauranik

Dr.P.Shivde

Dr.A.Verma

Candidate: Dr.Amith R

Case 1:Purpose of presentation.

Rarity of the case Peculiar clinical picture.

Case Scenario

24 year male, laundry business.

Involuntary jerky movements of extremities and

trunk - 8 months.

Difficulty while walking.

Interruption while speaking.

Involuntary movements are not suppressible voluntarily & are absent during sleep

No stiffness of limbs, weakness or sensory symptoms.

No c/o forgetfulness, abnormal behaviour.

No visual disturbances, dysphagia.

No bowel, bladder incontinence.

Examination

General examination : normal Nervous system :

Conscious oriented to TPP.

Higher mental functions: memory, intelligence is

normal

Speech: fluency is affected by pauses during the

Cranial Nerves: Normal

Contd.. Motor system:

Nutrition – normal

Tone – Normal

Power – Normal. Involuntary movements:

- present at rest

- involves limb and axial musculature,

- abrupt , brief & arrhythmic

- repetitive with frequency of 15-16/min,

- large amplitude with dystonic posturing of

upper limbs

- not stimulus sensitive

Sensory system : Within normal limits Cerebellar signs – absent. Gait – normal except for pauses during the

episodes of jerks.

Bilateral & near symmetrical T2 hyperintense signal in posterior putamen, Slightly larger on the right. Both reveal restriction of diffusion & are hypo on T1.No cortical or white matter changes seen.

Stereotyped periodic complexes with normal backgroundStrongly suggestive of SSPE.

CSF Analysis

Routine: Glucose-64 mg/dl

Protein- 68 mg/dl

Cells- 2/cu.mm Measles IgG antibodies by EIA

Serum IgG 3764 U/ml

CSF IgG 16205 U/ml

CSF/Serum Quotient 2.62 (N- <1.3

E- 1.3-1.5

P- >1.5)

CLINICALCHEMISTRY, Vol.37, No.7, 1991

Subacute Sclerosing Pan Encephalitis(SSPE)

Fatal neurodegenerative disorder- sequel to early childhood measles.

Prevalence in India is 6-8/million population. Virus remains dormant intracellularly & manifests

as SSPE a decade later. Avg interval between measles & SSPE is 8-11 yrs. Increasing age of onset, more aggressive is the

course.

Diagnostic Criteria

Definitive: 3 out of 5 should be positive Clinically progressive, subacute cognitive

deterioration or typical signs like myoclonus. EEG -Periodic, stereotyped, high voltage

discharges. Cerebrospinal fluid raised gammaglobulin or

oligoclonal pattern Measles antibodies raised titre in serum and/or

cerebrospinal fluid. Brain biopsy suggestive of panencephalitis

. Subacute sclerosing panencephalitis. Neurol Clinical (31) 79–95

What’s atypical in our patient?

Age of onset. Absence of other symptoms. MRI abnormality.

Neurology India Sept-Oct 2012/Vol 60/Issue 5Am J Neuroradiol 17:761–772, April 1996

Treatment

No curative therapy. Disease modifying agents

- Isoprinosine

- Levamisole

- Amantidine

- Interferan alpha

- IVIg

Postgrad Med J 2002. 7863–70.70.

Case 2Purpose of presentation Management issues in a common disease

18 yr old female student. 3 months h/o drooping of eyelid, difficulty in

chewing, swallowing & change of voice associated with generalised weakness.

Symptoms worsen as the day progress and improve on resting.

No limb weakness or sensory symptoms. Examination reveals ptosis on Lt side, reduced

elevation of soft palate b’l with preserved gag reflex.

Fatigability of limbs on sustained contraction

Diagnostic tests

Edrophonium (Prostigmin)test. Anti- AchR antibodies. Electrodiagnostic testing. Ocular cooling.

Bradley's Neurology in Clinical Practice- 6th Ed

Prostigmin test

Anti Ach receptor antibodies – Positive

0.65 nmol/L(< 0.25nmol/L) Thyroid functions are normal

Myasthenia Gravis

Incidence of 3-5/1,00,000 in India.

More common in females but in India M>F.

Females in 1-2 decade but in males in 5-6 decade.

Neurology India Jul-Aug 2009/Vol 57/Issue 4

Treatment

Pyridostigmine Prednisolone. Azathioprine. Thymectomy…

Thymectomy in myasthenia Absolutely indicated in cases of

thymoma regardless of age. Nonthymomatous generalised MG :

Early onset Anti Ach receptor positive cases.

Less effective in seronegative Anti Musk Ab positive patients.

Preferred approach is transthoracic sternal splitting procedure

Bradley's Neurology in Clinical Practice- 6th EdNeurology India Jul-Aug 2009/Vol 57/Issue 4

Case 3Purpose of presentation

Clinical syndrome of transverse myelitis is common.

This can occur due to LETM.(post MRI era)

List of causes for LETM is long and requires exhaustive workup.

Even after exhaustive workup cause may not be known.

Case Scenario

55 yr old male . Building contractor

Tingling & numbness of both lower limbs 8 months

Progressive weakness of both lower limbs (L>R).

Hesitancy & dribbling micturition,

No retention or incontinence. Involuntary movements of both lower limbs

(L>R). Band like sensation around the waist,

No backache or weakness of upper limbs. No h/o diminution of vision, breathing difficulty

Examination

Vitals : normal Nervous System:

- Conscious oriented to TPP.

- Higher mental functions : normal

- Findings are confined to motor & sensory

system.

contd….

Motor system examination:

Beevors sign: positive Neck muscles: power 5/5

RUL LUL RLL LLL

Nutrition N N N N

Tone N N ↑↑ ↑↑

Power 5/5 5/5 4-/5 3/5

Coordination

Normal Normal Impaired Impaired

Involuntarymovements

Absent Absent Clonus Clonus

Reflexes : Upper limb DTR were brisk,

Hoffman's & Wartenberg negative

Lower limb reflexes exaggerated with

ankle & patellar clonus. Abdominal and cremastric reflexes absent. Plantars are extensor bilaterally. No cerebellar signs. Romberg's: positive. Sensory system examination:

Pain, temperature, vibration & joint position

sense absent till level of ASIS.

Diagnosis

Anatomical:

Myelopathy

Lower Dorsal (D11- 12)

Pyramidal, Posterior column, Spinothalamic

± Autonomic involvement

Diagnosis

Pathological:

- Inflammation = Transverse myelitis.

- Nutritional = Vit B12 deficiency

- Ischemic = Spinal cord infarction.

- Dural AVM

- Tumour & other causes of compression

Investigations

X RAY L-S SPINE

MRI SPINE

MRI Spine

Evidence of a long segment intramedullary T2 hyperintense signal seen in dorsal cord extending from D7 upto D11/12 segments. Both half of cord appear involved and involved cord appears edematous.

The possibility of inflammatory demyelinating disease like LETM is more likely.

Longitudinally Extensive Transverse Myelitis(LETM)

Spinal cord inflammation extending 3 or more vertebral segments in length.

Can involve entire length of spinal cord, are much rarer.

Associated with greater morbidity than typical transverse myelitis.

European Neurological Journal 2011; 3:(1)

Neuromyelitis Optica(Devic’s disease)

Well recognised cause of LETM. Demyelination of spinal cord and optic nerve. Can have monophasic or relapsing course Diagnosis is by clinical, radiological & serological

criteria. Poorer prognosis than MS

Diagnostic Criteria for NMO

1: Optic Neuritis

2: Myelitis

3: Atleast two of the following

(a) Contiguous spinal cord MRI lesion extending 3 or more segments

(b) Brain MRI not meeting criteria for MS

(c) NMO IgG seropositive status

In our patient

Optic neuritis - Visual evoked potential is normal

Myelitis - Satisfies the criteria. Anti NMO antibody - negative

Multiple Sclerosis

Commonly associated with partial transverse myelitis spanning 2 or less spinal segments.

LETM common in Indians with the optico-spinal variant.

Have coexistent brain lesions.

In our patient

CSF Analysis:

- Opening pressure: 10 cm of H2O.

- Pandy’s : Negative.

- Glucose : 63 mg%(40-70)

- Protein : 101 mg%(15-40)

- Total cells: 2 (100% lymphocytes)

- Oligoclonal bands: No band seen.

- ADA : 5

MRI Brain : No abnormality detected

Systemic Lupus Erythematosis(SLE)

Most severe and rare complication of SLE seen in 1-3% patients.

Majority occurs shortly after SLE is diagnosed

Recurrence is frequent Preceded usually by non specific

prodromal symptoms

Myelitis in the course of SLE- POLSKIE ARCHIWUM 2009,119

Diagnostic Criteria for myelitis in SLE by ACR

Rapid onset of one or more of the following

Bilateral weakness of legs with or without arms, may be asymmetrical

Sensory impairment with cord level similar to that of motor weakness

www.rheumatology.org/publications/1999/499ap10

Exclusion Criteria

Mass lesion causing compression of or within spinal cord

Cauda equina lesion

In our patient

CBC : Normal Urinanalysis : Normal ESR : 10 mm/hr. CRP : 0.6 (N-0.6) ANA -0.649 (positive >1.4) Anti dsDNA – Negative aPTT – 35 sec

Myelopathy seen in 5-10% Most common being vacuolar

myelopathy. Seen mostly in advanced stages Rare after introduction of cART. In our patient- HIV 1 & 2 both were

negative. VDRL -Nonreactive

Intern Med 50: 1615-1617, 2011

Sarcoidosis

Mainly subacute or chronic in presentation.

Most common in males in their early 40s

Lesions most often located in cervical or upper thoracic region.

Associated commonly with respiratory symptoms.

In our patient

Serum ACE – 26mcg/L (N < 40mcg/L)

CRP – normal. S. Calcium – 9.7

Other investigations

Serum B12 – 931.7 (211-911). USG abdomen & pelvis:

enlarged prostate. LFT : Normal. TSH: 1.56

CAUSES OF LETM

Inflammatory

Neuromyelitis Optica

Multiple Sclerosis

Behcet’s syndrome

Sarcoidosis

Sjogren’s syndrome

Infectious

HTLV I & II

Neurosyphilis

Herpes simplex

Varicella zoster

Vascular

Dural AVM

Spinal cord infarction

Intramedullary spinal neoplasms

Ependymoma

Astrocytoma

Radiation myelitis

THANK YOU

SSPE, myasthenia n LETM

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