Clinical Session

Neurology Department

17^th of May 2016, 12:00H

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Ovidiu C Banea, Clinical Neurophysiology Unit

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Reason for medical consultation

17.12.2015 11:56 Læknasetrið Viðtakandi,

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Taugarannsóknadeild, LSH Fossvogi�Innihald: Beiðni um taug vöðvarit.

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40 year old women

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Mikið verkjavandamál og straum tilfinning í útlimi

Einnig síðustu 2-3 mánuði kippir í ganglimi meira vi megin

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Pain and disestesia (electric current) in extremities, more in the lower limbs

Also in the last 2-3 months contractions and spasms more in the left side

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Reason for medical consultation

WHAT TO DO?

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??? RADICULOPATHY, POLYNEUROPATHY, 2 MN

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NCS, Needle EMG… and stop?

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Findings

Findings

Findings

Findings

Mixed SP

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Cutaneous SP

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Indirect assessment of myelinated A delta small fibers (onset Latency)

EDX didn´t show radiculopathy or PNP

What to do?

Extended anamnesis showed episodes of dizziness, general weakness and chronic pain...

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New set of tests: RNS, SFEMG, VEMPc

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Findings

Findings

Findings

SFEMG

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showed increased jitter in more than 10% of single muscle fibers

History (1)

Extended anamnesis:

40 year old female, no smoke, with alcoholism (disabled 20 years ago), hypersensitivity to anesthetics, great intolerance to drugs and foods, which cause a variety of symptoms such as dizziness and nausea. Urinary tract infections as a child and went to the widening of the urethra twice (saga). Mother of 4 children born in '95, '97, '98 and '08. An abortion and miscarriage '05 and '06 (G6, P4). In 2008 tubar surgery sterilization. For this last surgery she signed for spinal anesthesia and avoided general anesthesia.

• Weakness and episodes of muscle tone losses since she was a child
• When she was 5-7 year old she remembers first episode of falling without reason
• When she was 10 year old she suffered a surgery (maxilo-facial), a correction of a tooth in left maxilar sinus, consequent weakness
• In 2004 she was diagnosticated with MS: neurological numbness, urinary symptoms..ocb, MRI negative Copaxone (Glatiramer Acetate), Lyrica, Symetrol. Severe worsening, diagnostic of psychiatric illness.�

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History (2)

Extended anamnesis:

• Patient with episodes of general weakness occurred when she was going to scool in several times in the 1990s, with dizziness and general weakness episodes which started in 1996, she describes that she lived 5 years in Denmark and often she went out through forests before starting with dizziness.
• Also she described sleep paralysis episodes.
• With diagnostic of MS in 2003 for which she received treatment with Lyrica, glatiramer acetate, Amantadine, Magnesium i.v. supplements. �Discribes that everytime after surgical intervention she felt very weak and sometimes with loss of motor control, with anal and urine incontinence which required many rehabilitation exercices, intermittent episodes of facial erythema with tingling in her face and her body which is intermittent manifestation, with memory lapses and loss in the last 2 years. �Actually with weakness which occurred in the afternoon sometimes after brief period of exercises (fatigability) or normal activity and which improves after few hours of sleep.

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Anamnesis

Presumptive diagnosis

Neuromuscular Junction Dysfunction (myastenic syndrome, MG) ?

Myalgic Encephalomyelitis / Chronic Fatigue Syndrome (ME/CFS) ?

Multiple Sclerosis ?

Lyme disease?

Neurosarcoidosis?

Amyotrophic Lateral Sclerosis (ALS)?

Sleep disorder?

Other...

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Fatigue or fatigability ?

FATIGUE = a subjective lack of physical and/or mental energy that is perceived by the individual or caregiver to interfere with usual or desired activities

FATIGABILITY = characteristic describing an individual’s susceptibility to experiencing fatigue for a given quantifiable demand (physical or cognitive exercise); measure of fatigue.

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NOT sleepiness, depression or apathy

Epworth Sleepiness Scale (Johns Murray, 1991)

MG

“Use of muscle relaxants for one week in a child resulted in a six week course of recovery. Similarly, long-term weakness from vecuronium use is reported to occur in adults. Paralysis lasted eight weeks after use of the neuromuscular blocker atracurium besilate “

Our patient experienced this weakness after every surgery

SFEMG had increased jitter

?Triggered by surgeries, Copaxone (Glatiramer acetate, Magnesium i.v. treatment ?)

Myalgic Encephalopathy (ME/CFS)

• Myalgic Encephalopathy or “ME” (a term which The ME Association feels is more appropriate than the original, Myalgic Encephalomyelitis)
• Chronic Fatigue Syndrome or “CFS”
• Post-Viral Fatigue Syndrome or “PVFS”
• Chronic Fatigue Immune Dysfunction Syndrome or “CFIDS”

Tiredness more than 6 months, acute onset, fatigue resilient to sleep

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Myalgic Encephalopathy (ME/CFS)

ALS, Lyme disease, neurosarcoidosis

ALS

-UMN (cognitive)

-LMN (fasciculations)

Needle EMG (-)

-Sensory symptoms (+)

-S-ACE (-)

Follow up

1. Antibodies to Ach (21.05.2016)

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• PSG and MSLT (sleep disorder, narcolepsy) to schedule with the neurologist and Sleep Unit

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• Lyme disease (Borrelia burgdorferi ELISA and specific Western Blot or PCR?)

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• If not psychiatric disorder or other medical condition... ME/CFS

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Narcolepsy

PSG 2008

The structure and pattern of the sleep stage characterized by superficial sleep and it lacks a deep sleep. High proportion of REM sleep.

Considerable örvökur, 17.8 / h., sometimes as a result of leg movements. PLM index 7.5 / hour.�Conclusion: disrupt sleep patterns and 13 of awakenings and sleep effeciency 89.9% and PLM.

Discuss these results and decide to wait with the medication during pregnancy. Sleep hygiene advice and will contact sleep profesionals if symptoms worsen.

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ME / CSF (Fernádez et al BMC, Psychiatry 2009)

Excluding other medical conditions caracterized by fatigue

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psychiatric disorders, such as major depression, schizophrenia, eating disorders (anorexia, bulimia), bipolar disorder, alcohol or other substance abuse, in addition to morbid obesity, and active medical diseases, either non-treated or without a completely established resolution.

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Conclusions

• The patient has disability (dizziness, chronic pain, fatigue, sleepiness, tingling, limb movement) for 24 years
• Narcolepsy, NMJ dysfunction(MG), MS, Lyme, pshychiatric disorder? ME/CFS?
• There are cases in literature of MG development due to Glatiramer acetate tt of MS; Magnesium supplements i.v. and neuromuscular blockade… can also unmask and trigger MG
• Fatigue should be differentiated of fatigability
• Sleepiness, depression or apathy are not fatigue
• MS, Narcolepsy, MG and ME/CFS have overlapping symptoms
• ME/CFS should be avoided at the beginning of the diagnostic process

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