Motor neuron disease in HIV


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Motor neuron disease in HIV

  1. 1. Interesting Case Presentation Dr. Ankit Raiyani Medicine Dept. LTMMC & GH
  2. 2. History42 yr male married R/O Matunga illiterate tailorPresented with c/o• Difficulty in holding small objects in right hand with loss of dexterity, progressive over 6 months and in left UL since last 2 mths• Difficulty in walking due to tightness in both LL progressive over 6 mths• Difficulty in speaking requiring more effort, more after talking for long time, progressively worsening over 6 mths• Twitching in both UL since 4 mths, in LL since 2 mths• Difficulty in swallowing solids and liquids with nasal regurgitation of liquids• increased frequency of micturition a/w urgency since 2 mths
  3. 3. No complaints of• Diplopia, blurring of vision• Headache, loss of consciousness,• Bowel, bladder incontinence• Loose motions, vomiting, abdominal pain• Tingling, numbness
  4. 4. Past history• k/c/o sero positive status, on ART (ZLN) since 2008, CD4 count- 222 in march 2011• H/O trauma to right knee at the age of 16 year. Unable to bend his right knee since then• Not a K/C/O diabetes, hypertension,• No P/H/O tuberculosisPersonal history- Ex alcoholic, ex tobacco chewerleft since last 6 mthsFamily history- no h/o such illness in family
  5. 5. Examination• Patient conscious oriented avg built, nourished• P- 88/min reg, all pp well felt• BP- 118/74 mm of Hg, supine• No pallor, icterus, clubbing, LNpathy, edema• Poor oral hygiene, no oral candidiasis, no linear bluish discoloration of gums• Skull, spine- NAD
  6. 6. CNS examination-• Conscious oriented co-operative• MMSE- 27/30, spastic dysarthria• Cranial nerves- – II to VIII – normal – IX, X- soft palate movement decreased , gag ++, cough + – XI- SCM, trapezius b/l normal – XII- atrophy +, fasciculations+
  7. 7. • Motor system – Nutrition- wasting of the thenar and hypothenar muscles of both hands – Patient sitting in bed with elbows flexed and wrist palmar flexed – fasciculations seen over both upper limbs, poly mini myoclonus+ – Tone - spasticity in BL LL > UL – Power- • 3/5 in right UL, 4/5 in left UL, • Wasting of thenar and hypothenar muscles, dorsal interossei , abd digiti minimi on both hands Rt > Lt – Reflexes- • DTR- brisk in all 4 limbs • Sup-Abdominal, Cremasteric- absent, Plantar - both extensor
  8. 8. • No sensory deficits. Rhomberg’s –negative• No cerebellar signs• Gait- spastic, scissoringOther systems - NAD
  9. 9. • 35 year male with diffuse mixture of LMN and UMN weakness in both UL and LL with pseudobulbar palsy• Clinical diagnosis- Motor Neuron Disease
  10. 10. Diff. diagnosis• Motor neuron disease- – Paraneoplastic syndrome – Heavy metal poisoning – Paraproteinemia – Metabolic – HIV associated MND• Pure motor polyradiculopathy
  11. 11. Investigations• Hb-12.1 gm%• WBC- 5100cells/cmm• DLC- P74/L24/M2• Platelets- 182000/cmm• MCV- 83 fl• FBS- 94 mg%• T. bili- 0.8 mg%• AST/ALT- 23/28 U/L• TP/Alb/Glob- 6.2/4.0/2.2 gm%• Bun/ Sr creat- 12/0.8 mg%• Na/K- 136/4.2 mmol/L• Ca/PO4- 7.4/3.6 mmol/L• Sr. TG/ Cholesterol- 104/ 158 mg%• Urinary BJP- negative• RBS- 93 mg%• T3/T4/TSH- 3.11/1.03/4.02 pg/dl• CD4- 222cells/µL• Stool R/M - NAD
  12. 12. • X-ray chest- NAD• CSF-WNL – Routine/microscopy- no cells – Prot- 28mg% Sugar- 81.8mg% – Gm stain – no PC, no org, no growth – Cryptococcus- not seen, no growth – Culture – no growth• EMG/NCV- s/o generalized anterior horn cell disease – Spontaneous Fibrillation, as well as polymorphic fasciculation in all groups of muscles – Sensory nerve conduction findings normal – No e/o an underlying generalized peripheral neuropathy• MRI Brain with screening of Cx spine- no significant abnormality
  13. 13. • Diagnosis- HIV associated generalised Motor Neuron Disease• Treatment- – Continue HAART – T. Riluzole (50mg) BD – T. Baclofen (10mg) TDS – IV Vit B12/ Folate supplements – Physiotherapy/ occupational therapy
  14. 14. HIV associated Motor neuron disease• HIV infection has been associated with increased incidence of ALS and other MND(1,2,3)• Possible mechanisms- not completely understood 1. Retroviral myelopathy(4) 2. Through HERV- K reactivation(5,6)- • HIV infection induces reactivation and replication of dormant Human Endogenous Retro Virus-K10. (5) • HERV-K has recently been implicated for pathogenesis of ALS though exact mechanism is not established.HERV-K pol transcripts were shown to be significantly increased in brain biopsy of patients with ALS compared to those with chronic systemic illness. (6)• Whole spectrum of MND ( UMN/LMN/Both) can be seen in different cases• Commonly involves patients not yet started on HAART
  15. 15. Diagnosis of exclusionAll other secondary causes of MND are to be ruled out• Structural- Cx myelopathy• Infections-tetanus, Lyme ds., poliomyelitis• Toxins/drugs- lead, aluminum, phenytion, strychnin• Immunologic- plasma cell dyscrasias, autoimmune polyrediculoneuropathy• Paraneoplastic• Metabolic- hyperthyroidism, hyperparathyroidism, B12/Folate def.• Errors of metabolism- adult onset Tay-Sach’s ds, SOD1 mutation
  16. 16. TreatmentNo specific treatment available• HAART- if patient not on ART. Starting HAART has shown neurological improvement with decreasing viral load with antiretroviral therapy. (1,2)• Supportive measures• Riluzole – may prolong life
  17. 17. References-1. Moulignier A, Moulonguet A, Pialoux G, et al. Reversible ALS-like disorder in HIV infection. Neurology 2001;57:995–10012. Reversal of HIV-associated motor neuron syndrome after highly active antiretroviral therapy. J Neurol 2001;248:233–4.3. Jubelt B, Berger JR. Does viral disease underlie ALS? Lessons from the AIDS pandemic. Neurology 2001;57:945–6.4. Modi G, Ranchhod J, Hari K, Mochan A, Modi M. Non-traumatic myelopathy -the influence of HIV. QJM 2011 Aug;104(8):697-7035. Garrison KE et al. T cell responses to human endogenous retroviruses in HIV-1 infection. PLoS Pathog. 2007 Nov;3(11):e165.6. Douville R, Liu J, Rothstein J, Nath A. Identification of active loci of a human endogenous retrovirus in neurons of patients with amyotrophic lateral sclerosis. Ann Neurol. 2011 Jan;69(1):141-51. doi: 10.1002/ana.22149.