pls

1. TUESDAY LECTURE
SERIES:
NEUROMUSCULA
R DISORDERS

2. IDENTIFYING DATA:
C.P.
44 yo/ Male
Right-handed
Roman Catholic
Driver/ Mechanic
From Dalumbatan, Teresa Rizal
First admission: May 21, 2010

3. CHIEF COMPLAINT:
Progressive Right-sided weakness of 1
year duration

4. HISTORY OF PRESENT ILLNESS
1 year and 1 month PTA (April 2009):
Mild foot weakness, foot as slow and dropping
Difficulty of putting his shoe to the Right
Difficulty lifting up the foot
No consult was done
1 year PTA (May 2009):
Difficulty lifting his Right leg
Infrequent episodes of buckling of the Right knee
Bumped on to chairs while walking, could not control his Right leg
Described his Right leg as weak
No problems in going up & down the stairs
Still no consult was done

5. HISTORY OF PRESENT ILLNESS
8 months PTA (Sept 2009):
Progressive Right lower extremity weakness
Noticeable instability during ambulation
Initial consult with a Private MD was done
On Consult:
4/5 motor strength on the Right leg
(+) Babinski’s sign
Right ankle clonus
Plain Lumbar MRI and EMG-NCV of both LE was done

6. PLAIN LUMBAR MRI:
mild spondylosis at L2-L3
small right paracentral disc protrusion at L2-L3 causing right lateral
recess stenosis
right ventral thecal sac and the exiting right L3 nerve root
indentations
Posterior disc bulges with annular fissures were similarly noted at L3-
L4 to L5-S1, with mild thecal sac indentation at L3-L4 and L4-L5

7. EMG-NCV (LOWER EXT –
BILATERAL)
Sensory Nerve Conduction:
Nerve and Site Onset Latency Peak
Latency
Amplitude Latency
Difference
Distance Conduction
Velocity
Sural.L
Calf 1.6 ms 2.1 ms 23 V 1.6 ms 92 mm 58 m/s
Sural.R
Calf 1.6 ms 2.0 ms 23 V 1.6 ms 89 mm 57 m/s
Superficial peroneal.L
Ankle 1.6 ms 2.1 ms 21 V 1.6 ms 100 mm 63 m/s
Superficial peroneal.R
Ankle 1.6 ms 2.2 ms 19 V 1.6 ms 102 mm 64 m/s

8. EMG-NCV (LOWER EXT –
BILATERAL)
Motor Nerve Conduction:
Nerve and Site Latency Amplitude Latency
Difference
Distance Conduction
Velocity
Tibial.L
Ankle 4.2 ms 16.6 mV 4.2 ms
Popliteal fossa 12.6 ms 11.7 mV 8.4 ms 375 mm 45 m/s
Tibial.R
Ankle 4.7 ms 15.1 mV 4.7 ms
Popliteal fossa 13.0 ms 9.5 mV 8.3 ms 369 mm 44 m/s
Peroneal.R
Ankle 3.9 ms 5.4 mV 3.9 ms
Fibula (head) 11.3 ms 5.6 mV 7.4 ms 340 mm 46 m/s
Popliteal fossa 12.8 ms 5.1 mV 1.5 ms 66 mm 44 m/s
Peroneal.L
Ankle 4.4 ms 5.5 mV 4.4 ms
Fibula (head) 11.4 ms 5.2 mV 7.0 ms 345 mm 49 m/s
Popliteal fossa 12.7 ms 5.2 mV 1.3 ms 55 mm 42 m/s

9. EMG-NCV (LOWER EXT –
BILATERAL)
F-wave:
Nerve F-Latency
Tibial.L 48.4
Tibial.R 48.6
H-waves:
Nerve Latency
Tibial.L
H-wave: 29.4 ms
Tibial.R
H-wave: 29.2 ms
EMG Studies:
Insertional Spontaneous Activity Volitional MUAPs Max Volitional Activity
Muscle Insertional Fibs +Wave Fasc Duration Amplitude Poly Config Recruitment Amplitude Pattern Effort
Gastrocnemius (Medial
head).L
Increased None None None Normal Normal None Normal Normal
Tibialis anterior.R Normal None None None Normal Normal None Normal Normal
Vastus medialis.R Normal None None None Normal Normal None Normal Normal
Rectus femoris.R Normal None None None Normal Normal None Normal Normal
L5-S1 paraspinal.R Normal None None None Normal Normal None Normal Normal
L4-L5 paraspinal.R Normal None None None Normal Normal None Normal Normal
L3-L4 paraspinal.R Increased None None None Normal Normal None Normal Normal
L2-L3 paraspinal.R Increased None 1+ None Normal Normal None Normal Normal
L1-L2 paraspinal.R Normal None None None Normal Normal None Normal Normal
L5-S1 paraspinal.L Normal None None None Normal Normal None Normal Normal
L3-L4 paraspinal.L Normal None None None Normal Normal None Normal Normal
L2-L3 paraspinal.L Increased None 1+ None Normal Normal None Normal Normal

10. EMG-NCV (LOWER EXT –
BILATERAL)
EMG Studies:
Insertional Spontaneous Activity Volitional MUAPs Max Volitional Activity
Muscle Insertional Fibs +Wave Fasc Duration Amplitude Poly Config Recruitment Amplitude Pattern Effort
Gastrocnemius (Medial
head).L
Increased None None None Normal Normal None Normal Normal
Tibialis anterior.R Normal None None None Normal Normal None Normal Normal
Vastus medialis.R Normal None None None Normal Normal None Normal Normal
Rectus femoris.R Normal None None None Normal Normal None Normal Normal
L5-S1 paraspinal.R Normal None None None Normal Normal None Normal Normal
L4-L5 paraspinal.R Normal None None None Normal Normal None Normal Normal
L3-L4 paraspinal.R Increased None None None Normal Normal None Normal Normal
L2-L3 paraspinal.R Increased None 1+ None Normal Normal None Normal Normal
L1-L2 paraspinal.R Normal None None None Normal Normal None Normal Normal
L5-S1 paraspinal.L Normal None None None Normal Normal None Normal Normal
L3-L4 paraspinal.L Normal None None None Normal Normal None Normal Normal
L2-L3 paraspinal.L Increased None 1+ None Normal Normal None Normal Normal

11. EMG-NCV
Evidence for Bilateral (right more than left) lumbar radiculopathy
affecting L2/L3 roots.

12. HISTORY OF PRESENT ILLNESS
7 months PTA (Oct. 2009)
Persistent Right leg weakness
Prominent Right upper extremity weakness: combing hair, scratching ears and reaching for
objects above head
Difficulty in gripping objects with the Right hand
Changes in his hand-writing
Repeat consult: “Brain Mass” suspected
Plain Cranial MRI and Cervico-Thoracic Contrast MRI was done

13. PLAIN CRANIAL MRI
bilateral frontal and parietal white matter signals
may be seen in microvascular ischemic changes, demyelination or in
migraine headache
no evidence of infarct, mass or hemorrhages.
Cervico-Thoracic Contrast MRI
• Spondylosis at C4-C7
• Circumferential disc bulges at C4-C5 to C6-C7 with mild
subarachnoid space indentation.
• Nothing conclusive was identified at this time.

14. HISTORY OF PRESENT ILLNESS
7 months PTA (Oct. 2009)
Repeat EMG-NCV was done a few weeks after

15. REPEAT
EMG-
NCV
Sensory Nerve Conduction:
Nerve and Site Onset Latency Peak
Latency
Amplitude Latency
Difference
Distance Conduction
Velocity
Sural.L
Calf 1.8 ms 2.2 ms 27 V 1.8 ms 102 mm 57 m/s
Sural.R
Calf 2.1 ms 2.6 ms 25 V 2.1 ms 115 mm 55 m/s
Superficial peroneal.L
Ankle 1.7 ms 2.3 ms 18 V 1.7 ms 99 mm 59 m/s
Superficial peroneal.R
Ankle 1.8 ms 2.4 ms 13 V 1.8 ms 95 mm 53 m/s
Median.R
Wrist 2.5 ms 3.1 ms 45 V 2.5 ms 130 mm 52 m/s
Palm 1.8 ms 2.2 ms 49 V 0.7 ms 36 mm 51 m/s
Ulnar.R
Wrist 2.0 ms 2.6 ms 39 V 2.0 ms 115 mm 58 m/s
Median/Ulnar.R
Comparison (Median) 2.5 ms 3.1 ms 11 V
Comparison (Ulnar) 2.3 ms 2.8 ms 20 V 0.2 ms
Radial.R
Forearm 1.5 ms 1.9 ms 37 V 1.5 ms 90 mm 62 m/s
Median.L
Wrist 2.3 ms 2.9 ms 48 V 2.3 ms 135 mm 59 m/s
Palm 1.5 ms 1.9 ms 40 V 0.8 ms 46 mm 58 m/s
Ulnar.L
Wrist 2.1 ms 2.5 ms 38 V 2.1 ms 120 mm 58 m/s
Median/Ulnar.L
Comparison (Median) 2.4 ms 3.2 ms 24 V
Comparison (Ulnar) 2.2 ms 2.8 ms 26 V 0.2 ms
Radial.L
Forearm 1.5 ms 1.9 ms 38 V 1.5 ms 92 mm 61 m/s

16. REPEAT
EMG-NCV
Motor Nerve Conduction:
Nerve and Site Latency Amplitude Latency
Difference
Distance Conduction
Velocity
Tibial.L
Ankle 4.6 ms 17.0 mV 4.6 ms
Popliteal fossa 12.4 ms 12.7 mV 7.8 ms 380 mm 49 m/s
Tibial.R
Ankle 4.6 ms 15.9 mV 4.6 ms
Popliteal fossa 12.6 ms 10.2 mV 8.0 ms 390 mm 49 m/s
Peroneal.R
Ankle 4.2 ms 6.0 mV 4.2 ms
Fibula (head) 11.7 ms 5.8 mV 7.5 ms 355 mm 47 m/s
Popliteal fossa 13.4 ms 6.7 mV 1.7 ms 75 mm 44 m/s
Peroneal.L
Ankle 3.9 ms 6.2 mV 3.9 ms
Fibula (head) 11.0 ms 6.6 mV 7.1 ms 350 mm 49 m/s
Popliteal fossa 12.3 ms 5.8 mV 1.3 ms 60 mm 46 m/s
Median.R
Wrist 3.8 ms 14.7 mV
Elbow 7.7 ms 13.0 mV 3.9 ms 230 mm 59 m/s
Ulnar.R
Wrist 3.1 ms 18.0 mV 4.1 ms 260 mm 63 m/s
Bel Elbow 7.2 ms 18.9 mV 1.2 ms 75 mm 63 m/s
Abv Elbow 8.4 ms 18.3 mV
Median.L
Wrist 3.3 ms 12.9 mV
Elbow 7.7 ms 13.0 mV 4.4 ms 260 mm 59 m/s
Ulnar.L
Wrist 2.6 ms 17.3 mV 4.6 ms 270 mm 59 m/s
Bel Elbow 7.2 ms 17.2 mV 1.1 ms 65 mm 59 m/s
Abv Elbow 8.3 ms 16.6 mV

17. REPEAT EMG-NCV
F-wave:
Nerve F-Latency
Tibial.L 47.8
Tibial.R 47.6
Median.R 27.9
Ulnar.R 27.3
Median.L 26.9
Ulnar.L 27.0
H-waves:
Nerve Latency
Tibial.L
H-wave: 29.1 ms
Tibial.R
H-wave: 29.7 ms

18. EMG Studies:
Insertional Spontaneous Activity Volitional MUAPs Max Volitional Activity
Muscle Insertional Fibs +Wave Fasc Duration Amplitude Poly Config Recruitment Amplitude Pattern Effort
Biceps brachii.R Normal None None None Normal Normal None Normal Normal
Flexor carpi radialis.R Normal None None None Normal Normal None Normal Normal
Extensor digitorum
communis.R
Normal None None None Normal Normal None Normal Normal
1st dorsal interosseous.R Normal None None None Normal Normal None Normal Normal
Gastrocnemius (Medial
head).R
Normal None None None Normal Normal None Normal Normal
Tibialis anterior.R Normal None None None Normal Normal None Normal Normal
Rectus femoris.R Normal None None None Normal Normal None Normal Normal
L5-S1 paraspinal.R Increased None 1+ None Normal Normal None Normal Normal
L5-S1 paraspinal.L Increased None None None Normal Normal None Normal Normal
L4-L5 paraspinal.R Normal None None None Normal Normal None Normal Normal
L4-L5 paraspinal.L Normal None None None Normal Normal None Normal Normal
C5-C6 paraspinal.R Normal None None None Normal Normal None Normal Normal
C6-C7 paraspinal.R Normal None None None Normal Normal None Normal Normal
C7-C8 paraspinal.R Normal None None None Normal Normal None Normal Normal
T6-T7 paraspinal.R Normal None None None Normal Normal None Normal Normal

19. REPEAT EMG-NCV
Evidence of an acute bilateral lower lumbar radiculopathy affecting
the S1 and/or L5 roots.
There were no findings at that time suggestive of a diffuse motor
neuron disease, myopathy, or neuropathy.
A repeat study was suggested a few months after.

20. SECOND OPINION ON NEUROIMAGING
Few non-specific, non-enhancing signal abnormalities involving the cerebral white matter, a
finding commonly seen in association with chronic migraine headaches and chronic small
vessel ischemic disease.
Mild lumbar spondylosis, demonstrating a small central and right paracentral disc protrusion
at L2-L3 and mild disc bulges from L3-L4 to L5-S1. No evidence of spinal canal/ neural
foraminal narrowing, or nerve root impingement is seen at any level.
Non-specific benign-appearing signal abnormality involving the S1 superior endplate, likely
representing degenerative bony sclerosis
Incidental finding of a moderate-sized simple cyst involving the left kidney.
“many subtle signal abnormalities particularly in the cervical cord. These may be
artifacts…explore the possibility that these may be due to vitamin B12 deficiency (subacute
combined degeneration)”

21. HISTORY OF PRESENT ILLNESS
1 Month PTA (April 2010):
Still ambulatory but noted marked difficulty lifting his Right leg
Limps when he walks and had difficulty walking up stairs
Could not effortlessly get up from a sitting position
Decreased arm swing when ambulating
Imperceptible weak grip on the right hand had now become distinct and
unmistakable.

22. CON’T
Patient was again subjected to an MRI of the cervical spine with Gadolinium.
Review of the images revealed normal findings.
The impression of the physician at that time was Amyotrophic Lateral
sclerosis versus Primary Lateral sclerosis.
Advised admission for further work up of possible ALS vs PLS, including
Lumbar Puncture and analyses of CSF.

23. PAST MEDICAL HISTORY:
No history of hypertension, diabetes, stroke/ TIA, cancer, aneurysm, recent
trauma, surgeries, heart disease, thyroid disease, or hospitalizations
FAMILY HISTORY:
No family history of hypertension, diabetes, cancer, stroke, aneurysm, or
movement disorder

24. PERSONAL AND SOCIAL HISTORY:
A Mechanical Engineer working as a mechanic supervisor at an International
Hotel
Non-smoker, occasional alcoholic beverage drinker (3-5 bottles once a
month), denies history of illicit drug use
He denies exposure to any toxic gas/ chemical, insecticide, or radiation.
Drinking water comes from Maynilad and is filtered and boiled.

25. REVIEW OF SYSTEMS
GENERAL/HEENT: (-) weight loss, (-) colds, (-) sore throat, (-) ear
fullness,
(-) tinnitus
CHEST/LUNGS: (-) difficulty of breathing; (-) shortness of breath, (-)
cough
CARDIAC: (-) palpitations, (-) orthopnea/ dyspnea, (-) chest pain, (-)
diaphoresis, (-) easy fatigability
ABDOMINAL: (-) abdominal pain, (-) nausea/ vomiting, (-) abdominal
enlargement
GENITOURINARY: (-) dysuria, (-) frequency, (-) hesitancy, (-)
incontinence

26. PHYSICAL EXAMINATION
General
Survey
Awake, not in cardio-respiratory distress
Vital Signs BP: 110/70, supine mmHg HR: 74 bpm RR: 19 cpm T: 36.1˚ C
Anthropom
etrics
Ht.: 172 cm Wt: 85 kg BMI:28.7 kg/m2
(Obese I)
HEENT Pink palpebral conjunctivae, anicteric sclerae, no gross head lesions; trachea
midline no palpable cervical lymphadenopathies, no thyroid enlargement or
distended neck veins; no carotid bruits
Chest/Lun
gs
No chest deformity, equal chest expansion, no rales, no wheezing, no
rhonchi
CVS Adynamic precordium, normal rate, regular rhythm, Apex beat and PMI at 4th
ICS LMCL, no heaves, no gallops, no murmur
Abdomen Flat, no lesions, no abrasions, lacerations, hematoma, normoactive bowel
sounds, no guarding, no masses, no organomegaly
Extremities Full and equal pulses, no edema, no cyanosis, good capillary refill
Rectal Intact anal wink, with good sphincteric tone. Empty recal vault

27. NEUROLOGICAL EXAMINATION
Mental Status
Exam
Awake, coherent, good attention, oriented, intact word registration,
can do serial 7’s, intact immediate, recent and remote memory, can
identify watch and pen, can do word repetition, follows 3-step
commands,
No ideational or ideomotor apraxia, no visual or tactile neglect, can do
interlocking fingers
Cranial
Nerves
I Can identify coffee
II Pupils isocoric 2mm BRTL, intact direct and consensual light reflex, no
visual field cuts bilaterally; fundoscopy: (+) ROR, no hemorrhages, AV
ratio 2:3, distinct disc margins; CD ratio 0.3
III, IV,
VI
Full EOM’s, no ptosis
V Intact V1 - V3, to pain, temperature and touch; Brisk corneal reflexes
bilaterally
VII No facial asymmetry, can raise brow, frown, smile
VIII Intact gross hearing, AC > BC, AU, non-lateralizing
IX,X Uvula midline with intact gag

28. 28
Motor
Exam
Θ
Θ
Spastic right upper and lower extremity. Normal tone on the left upper
and lower extremity, normal muscle bulk. No rigidity, no observable
fasciculation
UPPER Right Left
Shoulder Flexion 5/5 5/5
Shoulder Extension 5/5 5/5
Shoulder Abduction 5/5 5/5
Shoulder Adduction 5/5 5/5
Elbow Flexion 5/5 5/5
Elbow Extension 5/5 5/5
Pronation 5/5 5/5
Supination 5/5 5/5
Wrist Extension 4/5 5/5
Wrist Flexion 4/5 5/5
Finger Extension 4/5 5/5
Finger Flexion 4/5 5/5
Grip 4/5 5/5
NEUROLOGICAL EXAMINATION

29. Motor Exam
Θ
LOWER Right Left
Hip Flexion 4/5 5/5
Hip Extension 4/5 5/5
Hip Adduction 4/5 5/5
Hip Abduction 4/5 5/5
Knee Flexion 3/5 5/5
Knee Extension 3/5 5/5
Plantar Flexion 3/5 4/5
Dorsiflexion 3/5 4/5
Eversion 3/5 4/5
Inversion 3/5 4/5
Toe Flexion 3/5 4/5
Toe Extension 3/5 4/5
Cerebellar
Function
No nystagmus, no dysdiodochokinesia, no dysmetria
Pathologic
Reflexes
Θ
Bilateral Babinski’s and Hoffmann’s signs, R>L.
Absent palmo-mental reflex.
Bilateral ankle clonus R>L
NEUROLOGICAL EXAMINATION

30. DTR’s Right Left
Brachioradialis +++ ++
Biceps +++ ++
Triceps +++ ++
Patellar +++ +++
Plantar +++ +++
Sensory Exam No sensory level, intact to pain, temperature, touch, vibration and
position sense, (-) Romberg’s Test
Right Left
Upper extremity 100% intact 100% intact
Lower extremity 100% intact 100% intact
Meninges Supple neck; absent Kernig’s and Brudzinski’s signs
Gait
Θ
Wide-based gait with decreased stride length and decreased arm
swing on the right with absent heal-strike/ toe-off on the right (flat
foot down). Dorsiflexion of the toes on leg swing. (Waddling Gait)
Tilting of the right hip on mid-stance. Swings right leg with tilted
right hip to turn left.
NEUROLOGICAL EXAMINATION

31. COURSE IN THE WARD
Admitted for Lumbar Tap:
 Obtained was 10-15cc of free flowing CSF with slight turbidity.
 Opening pressure was 22 cmH2O
 Closing pressure of 16.5cmH2O
 CSF analysis, routine and CALAS, HSV, oligoclonal bundle IgG, VDRL, TB PCR
 Vitamin B12; thyroid function test
Asymptomatic all through out the hospital stay
Discharged the following day

32. LABS
CBC 5/21
Hgb 141 (N)
Hct 0.42 (N)
RBC 4.71 (N)
WBC 6.50 (N)
MCH 30 (N)
MCHC 0.34 (N)
MCV 88 (N)
RDW 13.5 (N)
Platelet 227 (N)
N 0.74 (N)
L 0.24 (N)
M 0.02 (N)
E 0.01 (N)
Morph Normocytic,
Normochromic
BLOOD
CHEMISTRY
5/21
BUA 6.1mg/dL
(N)
TSH 1.73 µIU/L
(N)
FT3 2.22 pg/mL
(N)
FT4 0.82 ng/mL
(N)
Total PSA 0.48 ng/mL
(N)
RBS 108 mg/dL
(N)
Vitamin B12 409.30
pg/mL (N)
CSF 5/21
Volume 0.0140 L
Appearance Colorless/ Clear
Supernatant Fluid Colorless/ Clear
RBC 60.00
WBC 0.00
Total Cell Count 60.00
DIFFERENTIAL COUNT
N 0.00
L 0.00
M 0.00
Total Protein 32.29 mg/dL
(N)
Glucose 58.72 mg/dL
(N)
MTC PCR Negative
CSF
Immunofixation
Electrophoresis
No oligoclonal
band detected

33. LOCALIZATION OF THE
LESION

34. SALIENT FEATURES
44/M, 1 year duration of right-sided
weakness
Weakness pattern: right foot -> right leg -
> right knee -> right arm -> right hand
Went for a second opinion
No known comorbidities
Occasional alcoholic beverage drinker
Non-smoker
No family history of stroke, movement
disorder
Intact cortical function, Intact cranial
nerves, Intact cerebellar function, Intact
sensory function, Absent meningeal signs
Spastic right upper and lower extremity
Right-sided weakness (⅗-⅘) from wrist,
hands, hips, knees, ankles, to toes; Left-
sided weakness (⅘) from knee to toes
Bilateral Babinski, Bilateral Hoffman,
Bilateral ankle clonus, Hyperreflexia on
right upper extremity and both lower
extremities
Waddling gait, wide-based with decreased
arm swing

35. PERTINENT ANCILLARY FINDINGS
EMG-NCV: Acute bilateral lower radiculopathy affecting L5-S1 roots
MRI:
● Cervical Spine: Spondylosis at C4-C7
● Lumbar Spine: Small right paracentral disc protrusion at L2-L3 causing
stenosis and indentations
● Cranial: Bilateral frontal and parietal white matter signals; non-specific, non-
enhancing signal abnormalities, a finding commonly associated with chronic
migraine headaches and chronic small vessel disease
● Cervical: Heterogeneous marrow signal, compatible with hematopoietic
marrow conversion

36. UMN OR LMN?
Spastic right upper and lower extremity
Right-sided weakness (⅗-⅘) from wrist,
hands, hips, knees, ankles, to toes; ⅘ from
left knee to toes
Bilateral Babinski, Bilateral Hoffman,
Bilateral ankle clonus, Hyperreflexia on
right upper extremity and both lower
extremities
Waddling gait, wide-based with decreased
arm swing

37. UMN OR LMN?
Spastic right upper and lower extremity
Right-sided weakness (⅗-⅘) from wrist,
hands, hips, knees, ankles, to toes; ⅘ from
left knee to toes
Bilateral Babinski, Bilateral Hoffman,
Bilateral ankle clonus, Hyperreflexia on
right upper extremity and both lower
extremities
Waddling gait, wide-based with decreased
arm swing

38. WHERE IS THE LESION?
Intact cortical function
Intact cranial nerves
Intact cerebellar function
Intact sensory function
Absent meningeal signs

39. WHERE IS THE LESION?
Intact cortical function
● Awake, coherent, good attention,
oriented
● Intact word registration
● Can do serial 7s
● Intact immediate recent and remote
memory
● Can identify watch and pen
● Can do word repetition
● Follows 3-step commands
● No apraxia

40. WHERE IS THE LESION?
Intact cortical function
Intact cranial nerves
● Identify coffee
● Pupils BRTL, intact light reflex
● Full EOMs
● Intact V1 to V3 sensory, Brisk corneal
reflexes
● No facial asymmetry
● Intact gross hearing
● Midline uvula with intact gag
● Tongue midline with good articulation

41. WHERE IS THE LESION?
Intact cortical function
Intact cranial nerves
Intact cerebellar function
Intact sensory function
Absent meningeal signs

42. WHERE IS THE LESION?
Spastic right upper and lower extremity
Right-sided weakness (⅗-⅘) from wrist,
hands, hips, knees, ankles, to toes; ⅘ from
left knee to toes
Bilateral Babinski, Bilateral Hoffman,
Bilateral ankle clonus, Hyperreflexia on
right upper extremity and both lower
extremities
Waddling gait, wide-based with decreased
arm swing

43. Localize:
Spinal Cord
prior to AHC
WHERE IS THE LESION?
Spastic right upper and lower extremity
Right-sided weakness (⅗-⅘) from wrist,
hands, hips, knees, ankles, to toes; ⅘ from
left knee to toes
Bilateral Babinski, Bilateral Hoffman,
Bilateral ankle clonus, Hyperreflexia on
right upper extremity and both lower
extremities
Waddling gait, wide-based with decreased
arm swing
Lateralize: Left
Levelize:
Spinal

44. LOCALIZE: SPINAL CORD
Spastic right upper and lower extremity
Right-sided weakness (⅗-⅘) from wrist,
hands, hips, knees, ankles, to toes; ⅘ from
left knee to toes
Bilateral Babinski, Bilateral Hoffman,
Bilateral ankle clonus, Hyperreflexia on
right upper extremity and both lower
extremities
Waddling gait, wide-based with decreased
arm swing

45. PATHOPHYSIOLOGY
Spastic right upper and lower extremity
Bilateral Babinski
Bilateral Hoffman
Bilateral ankle clonus
Hyperreflexia on right upper extremity and
both lower extremities

46. PATHOPHYSIOLOGY
Loss of descending
central inhibitory
control

47. PATHOPHYSIOLOGY
Loss of descending
central inhibitory
control
Hypersensitive muscle
spindles

48. PATHOPHYSIOLOGY
Loss of descending central
inhibitory control
Hypersensitive muscle
spindles
Intrafusal muscle fibers
remain activated
(prestretched)

49. PATHOPHYSIOLOGY
Loss of descending central
inhibitory control
Hypersensitive muscle
spindles
Intrafusal muscle fibers
remain activated
(prestretched)
Disturbed regulatory
circuit

50. PATHOPHYSIOLOGY
Loss of descending
central inhibitory
control
Hypersensitive muscle
spindles
Intrafusal muscle fibers
remain activated
(prestretched)
Disturbed regulatory
circuit
Spastic tone and
hyperreflexia

51. PATHOPHYSIOLOGY
Right-sided weakness (⅗-⅘) from
wrist, hands, hips, knees, ankles, to
toes
Left-sided weakness (⅘) from knee to
toes
Waddling gait, wide-based with
decreased arm swing

52. PATHOPHYSIOLOGY
● Corticospinal and Rubrospinal
tracts
● Project to the distal musculature
● Primarily responsible for
voluntary movements, precise,
highly differentiated, fine motor
control
Lateral Group

53. PATHOPHYSIOLOGY
● Corticospinal and Rubrospinal tracts
● Project to the distal musculature
● Primarily responsible for voluntary
movements, precise, highly
differentiated, fine motor control
● Reticulospinal, Vestibulospinal, and
Tectospinal tracts
● Innervate motor neurons lying more
medially in the AHC
● Primarily responsible for movements
of the trunk and lower limbs
Lateral Group
Medial Group

54. WORKING IMPRESSION:
PRIMARY LATERAL
SCLEROSIS

55. DIFFERENTIAL DIAGNOSIS

56. IS THERE A LESION?
YES

57. Where is the
lesion?
Upper Motor
Neuron
Lower Motor
Neuron

58. Where is
the lesion?
Spinal Supraspinal

59. WHERE IS THE LESION?
Upper Motor Neuron Lesion at the
Spinal Level
What is the lesion?

60. UPPER MOTOR NEURON LESION
Genetic-congenital
Traumatic
Infectious
Psychogenic
Iatrogenic
Toxic
Metabolic (Inherited versus Acquired)
Neoplastic
Inflammatory – immune
Vascular
Degenerative
History
Adult onset -44 years old
Chronic
Progressive
Ascending
Affecting one side of the
body
Unremarkable family history
No history of trauma
No known exposure to toxic
chemicals / occupational
hazards
No previous surgeries
No history of fever, weight
loss
No pain
Laboratory results
Imaging studies
Adams and Victor’s Principles of Neurology 10th
edition
Physical Examination
Stable vital signs, afebrile
No involuntary movements
(+) Babinski, R
(+) Spasticity
(+) weakness of R UE & LE

61. PRIMARY LATERAL
SCLEROSIS
Degenerative upper motor neuron lesion at
the spinal level

62. PRIMARY LATERAL SCLEROSIS
Clinical manifestations Patient’s Case
5th decade of life 44 y.o.
Upper motor neuron lesion (+) Babinski
Progressive spasticity (+)
Clumsiness tripping during practice,
difficulty putting on right shoe,
instability during ambulation
MRI – cortical atrophy and
increased T2 signal in pyramidal
tracts
Absent
Electrodiagnostic test – absent
LMN involvement
Radiculopathy; findings not
suggestive of a diffuse motor
Braddom’s Physical Medicine and Rehabilitation 5th
Ed (2016)

63. MILLS’ SYNDROME
Idiopathic, slow progressive, ascending spastic hemiparesis
Symptoms started in a leg
Mean onset of 59 years
Mildly elevated CSF protein
MRI demonstrated focal T2 hyper-intensity located eccentrically in
the cervical cord ipsilateral to the symptomatic side
Jaiser, S.R., Mitra, D., Williams, T.L. et al. Mills’ syndrome revisited. J Neurol 266, 6
(2019).

64. INITIAL IMPRESSION
to consider Mills’
syndrome

65. DIAGNOSTICS/IMAGING

66. LUMBAR MRI PLAIN
9/9/2009
67

70. LUMBAR MRI IMAGES (PLAIN)
Mild spondylosis, L2-L3.
Small right paracentral disc protrusion, L2-L3, causing right lateral
recess stenosis, right ventral thecal sac and the exiting right L3 nerve
root indentations.
Posterior disc bulges with annular fissures, L3-L4 to L5-S1, with mild
thecal sac indentation at L3-L4 and L4-L5.
Renal cyst, left.
71

71. EMG-NCV
Evidence of bilateral (right more than left) lumbar radiculopathy
affecting L2/L3 roots
72

72. CRANIAL MRI IMAGES (PLAIN)
11/12/2009

74. 75

77. Bilateral frontal and parietal white matter signals, which maybe seen
in microvascular ischemic changes, demyelination or in migraine
headache.
No evident acute infarct, mass or hemorrhage.
Nasal congestion.
78

78. CERVICO-THORACIC MRI
WITH CONTRAST
11/18/2009

82. Spondylosis, C4-C7.
Circumferential disc bulges, C4-C5 to C6-C7 with mild ventral
subarachnoid space indentation
83

83. SECOND OPINION OF
NEUROIMAGING
Few non-specific, non-enhancing signal abnormalities
involving the cerebral white matter, a finding commonly seen
in association with chronic migraine headaches and chronic
small vessel ischemic disease.
Mild lumbar spondylosis, demonstrating a small central and
right paracentral disc protrusion at L2-L3 and mild disc
bulges from L3-L4 to L5-S1. No evidence of spinal canal/
neural foraminal narrowing, or nerve root impingement is
seen at any level.
Non-specific benign-appearing signal abnormality involving
the S1 superior endplate, likely representing degenerative
bony sclerosis
Incidental finding of a moderate-sized simple cyst involving
the left kidney.
“many subtle signal abnormalities particularly in the cervical 84

84. CERVICAL CONTRAST MRI
4/17/2010

87. 88

89. Heterogenous marrow signal, compatible with hematopoietic marrow
conversion
Otherwise no other abnormality seen involving the cervical spinal
column
No evidence of focal disc herniation, spinal canal/ neural foraminal
narrowing or cord compression is seen at any level.
No intrinsic cord signal abnormality identified
90

90. EMG-NCV DATE FINDINGS
Lower Extremities
and back
9/18/20
09
 There is evidence for bilateral (right side more than the left) lumbar
radiculopathy affecting L2/L3 roots.
Upper extremities
and neck
12/18/2
009
 Evidence for an acute bilateral lower lumbar radiculopathy affecting
the S1 and/or L5 roots.
 There are no findings at that time suggestive of a diffuse motor
neuron disease, myopathy, or neuropathy

91. MANAGEMENT

92. There is no cure for PLS
Riluzole, the only FDA approved drug for ALS, has not shown any clear benefit in patients with
PLS.
Spasticity:
 FIRST LINE ORAL AGENTS: baclofen, tizanidine, or valium.
 For patients who achieve some benefit with anti-spasticity drugs, but are limited by sedating side-
effects, a trial of intrathecal baclofen may be useful
Excess oral secretions or drooling:
 Most patients will be first tried on oral anticholinergic medications – amitriptyline, scopolamine,
glycopyrrolate, or atropine drops.
 For drooling unresponsive to oral therapies botulism toxin injections into submandibular glands
may be beneficial
Pseudobulbar affect:
 Combination of dextromethorphan and quinidine (Neudexta) may prove beneficial.
 If Neudexta does not work: Tricyclic antidepressants may prove beneficial.
Primary Lateral Sclerosis, Statland et. al, Neurol Clin. 2015 November ; 33(4):
749–760.

93. REHABILITATION
- Strategies for rehabilitation must be designed to the targeted patient population depending on disease
impact and natural history.
- Patients with primarily spinal involvement tend to progress over six stages with common clusters of
rehabilitation needs in each, although individuals can vary considerably and straddle stages rather
than fall neatly into one.

94. Stage 1
patients who are ambulatory
and fully independent, with
mild weakness or clumsiness
- need to maintain active range of motion, carry on with normal activities of
daily living, but can benefit from strengthening unaffected muscle.
- strengthening all muscles, including affected muscles, with caution for
overwork damage is reasonable
Stage 2
remain ambulatory and
independent but have
moderate weakness
- Functional impairment may be severe in some areas despite preserved
overall function.
- Often benefit from ankle-foot orthoses to compensate for foot drop
- Dorsiflexion assist: should be avoided in patient with plantar flexion
spasticity
- A wrist-hand orthosis to assist with wrist drop is often needed.
- An opponens splint can be used to accommodate lack of thumb opposition
from thenar muscle weakness
- Hand weakness also commonly makes buttoning difficult: assistive devices
to ease this task are helpful.
- Focal weakness of the cervical extensor muscles leading to head drop or
chin-on-chest deformity is a common problem: cervical orthoses to
stabilize the cervical spine may help provide some relief but are sometimes
poorly tolerated.
- Work simplification can be useful at this stage.
- Exercise prescription is similar to stage 1
Stage 3
patients who remain
ambulatory but have severe
weakness in selected muscle
- Common problem: inability to rise from a chair.
- Lift chairs and elevated toilet seats can mitigate this problem.
- Ambulation aids i.e., a walker, can be helpful and a manual wheelchair for

95. Stage 4
patients who are
nonambulatory but remain
independent
- Electrically powered chairs are usually appropriate.
- Exercise is limited to any unaffected muscles
- Utilization of range-of-motion exercises is still useful.
- A hospital bed facilitates bed mobility.
Stage 5
the patient is no longer
independent
- Transfer assistance, with devices such as Hoyer lifts, is generally required.
- Family education is a large part of the care of the patient.
- A wheeled shower chair is often helpful for these patients.
- A lack of mobility makes pressure relief measures in the wheelchair and
bed higher priorities.
Stage 6
completely bedridden,
dependent, and requiring
maximal assistance
- Proper positioning is important to keep patients as comfortable as
possible.
- Pressure relief and skilled caregivers are critical.
- Palliative care becomes the focus of treatment.

96. PROGNOSIS A question of great concern to PLS patients is whether their
condition will convert to ALS.
Patients who do not have lower motor neuron findings after
four years typically remain with clinically pure upper motor
neuron dysfunction with a normal lifespan.
There are, however, a few reported cases of PLS patients
developing late slowly progressive lower motor neurons
and EMG findings, even several decades later
Primary Lateral Sclerosis, Statland et. al, Neurol Clin. 2015 November ; 33(4): 749–
760.

97. THANK YOU!