Neurological Symptoms in Right-Handed Man

1. CC • A 55 y. o. right handed man with difficulty at handwriting and with motor problems in his right leg

2. HPI • Starting 5 months prior to admission: • Numbness and sensation of „swelling“ in the feet, without edema>> persisting

3. Three days before admission • Subtle difficulty in steering his car • Awkwardness of handwriting • Right foot felt clumsy on walking • Difficulty in solving simple mathematical problems • Symptoms continued >> admission to hospital

4. PMH • Hypertension: Atenolol 100 mg/ day • Gout: Allopurinol 300 mg/ day • Left nephrectomy with 11 years because of ureteral stricture • Left leg vein stripping recently • Dental abscess two years ago • Three beers nightly • 45 pack-years of smoking, ceased last year

5. No history of: • Optic neuritis or other neurological problems • Bowel or bladder dysfunction • Recent headache • Fever • Speech problems • Dysphagia, nausea, vomiting • Risk factors for HIV

6. No history of: • Illicit drug abuse • Recent immunizations • Weight loss • Travel, except one trip to Bermuda six months ago

7. SH • married • Math teacher

8. Physical examination • Temperature: 36,3°C • Pulse: 53 and regular • BP: 170/ 55 mmHg • Respiratory Rate: 18/ min • Lungs, heart, abdomen: normal

9. Neurological examination • Alert and oriented • Difficulty solving simple math problems • Problems with spelling words backward • Function of cranial nerves: normal • Muscle tone: normal • Motor function: 5/ 5, except a mild footdrop and a questionable limitation in grip strength, both on the right side

10. Neurological examination 2 • Perception of pinprick and light touch: intact • Vibratory sensation: present, except in the great toes • Gait was rather slow and cautious • Romberg questionable positive • Right knee jerk ++, left knee jerk + • No ankle jerks, Biceps jerks bilaterally +++

11. Lab • Urine: within normal limits • Hematocrit: 41, 2% • White cell count: 7 800 / cm² • Platelet count: 250 000/ cm² • Erythrocyte sedimentation rate: 11 mm/ h • Quick and PTT: normal • IgA, G, M: within normal limits

12. Lab 2 • Antibody against Borrellia • Anti- HIV • Anti- Toxoplasmosis IgM • Serologic test for syphilis • ANCA • >>> all negative • Normal serum level of Vit. B12

13. Assessment • A 55 y.o. math teacher with numbness in the right foot • Clumsy walking • Awkwardness of handwriting • Cognitive problems ( solving math problems, spelling backwards...)

14. CT • Multiple round lesions within the subcortical white matter • No evidance of hemorrhage or infarct

16. MRI • Multiple nodular and ringenhancing lesions • Located in the white matter of both cerebral hemispheres

17. Other diagnostic procedures • Chest XR • Abdominal, pelvic and thoracic CT • >> no abnormalities • Transthoracic cardiac ultrasonography: no evidence of valvular vegetations or of valvular regurgitations

18. Management • Administration of thiamine and folic acid • Therapy with atenolol and allopurinol continued

19. Fifth hospital day • Patient reveals confusion • Could not name the date and the year

20. Lumbar puncture • Clear, colorless cerebrospinal fluid • Lightly raised white cells ( 8/ cm² , 80% Lymphos, 20% Monos ) • Glucose level: normal • Total protein and albumin level: lightly raised • IgG-level: lightly raised

21. Lumbar puncture 2 • No acid-fast bacilli or other microorganisms • Agarose gel electrophoresis: no specific banding • No cryptococcal antigene • Results of bacterial, fungal and mycobacterial cultures were pending

22. The same day • Patient began to shiver • Fever: 38,7ºC • But fully oriented

23. Another neurological exam • Grip strength right slightly weaker • Tendon reflexes mildly hyperactive on right side • Right sided Babinski +

24. Management • Metronidazol, Penicillin and Vancomycin • Another CT was unchanged

25. Eigth hospital day • Temperature was normal again: 37,4ºC • All culture specimens remained sterile

26. DD • Bacterial endocarditis • Metastasis from cancer • Fungal infection • Mycobacterial infection • Neurocysticercosis • Neurosarcoidose and SLE • Multiple Sclerosis and ADEM

27. Bacterial endocarditis • Pro: history of dental abscess • Multiple brain abscesses? • Contra: absence of vegetations in echocardiography • Low erythrocyte sedimentation rate • Absence of prominent systemic signs ( weight loss, skin lesions ) • Treatment with antibiotics would not suppress the infection within a day

28. Metastasis from lung cancer • Pro: 45-pack-years history of smoking • Small cell cancer is difficult to identify, even on thoracic CT • Contra: absence of headache • Slow progressions of symptoms over a five month period • No signs of increased intracranial pressure

29. Fungal infection • Contra: no immunocompromise, organ transplantation, diabetes or travel to remote tropical locations • Cryptococcel antigen: - • Glucose level: normal

30. Neurocysticercosis • Contra: absence of scolices inside any of the acute appearing lesions • 60% of the patients present with seizures • No eosinophilia in the periphery or in the cerebrospinal fluid

31. Neurosarcoidose and SLE • Contra: no meningeal involvement on enhanced MRI • ANCA: - ( for SLE )

32. Mycobacterial infection • Not easy to rule out: even not with normal glucose level and normal chest XR

33. Multiple Sclerosis • Most common white matter disease in all age and ethnic groups • Manifestation in a myriad of ways • Appearence either with acute exacerbations with recurrences and progression over years or chronical

34. ADEM ( acute disseminated encephalomyelitis ) • In this case they make a difference between MS and ADEM • ADEM: one time event • Often postvaccinal or parainfectious • Or idiopathic • Often dramatic response to high dose corticosteroids

35. Pathology • Astonishing for me, they made here a verification with stereotactic needle biopsy of the frontal lobe: • Striking perivascular inflammation • Loss of the normal organized appearence of the white matter • Loss of myelin and relative preservation of axons

39. Medication • Methylprednisolone i.V.: 1000 mg for five days • Then 500 mg for three days • And another three days with 250 mg • Then reduction of dose and oral formulation

40. Follow up • For weeks after discharge: • Speech fluency improved dramatically • Patient gestured with both arms during conversation • Gait was broadbased, right leg remained slighty weak • But patient is steady with cane

41. Three months after discharge • Patient can walk without assistance • No weakness in arms and legs • Mental capacity returned close to base line level with exception of ability to perform math calculations, so the patient thinks of early retirement