Neuro Packets- Neuromuscular Disease
Pablo O. Medina
1.a. The most likely diagnosis in this patient is a mononeuropathy due to compression of the
median nerve as it passes through the carpal tunnel (carpal tunnel syndrome). Symptoms of
carpal tunnel syndrome can be instigated by prolonged flexion or extension of the wrist, which is
a common position when driving. During pregnancy, fluid accumulates in the carpal tunnel and
CTS becomes a common complaint. Carpal tunnel entrapment involves parasthesias in the
median distribution, most commonly affecting the first 3 or 4 digits and lateral hand. It also
involves pain in the palm, wrist, forearm, and sometimes arm and shoulder. Physical exam signs
to look for in this patient would be median sensory loss, weakness of thumb opposition, thenar
atrophy, and possibly a Tinel’s and/or Phalen’s sign. Other alternative diagnoses to consider
would be ulnar neuropathy, a polyneuropathy, multiple mononeuropathy, a radiculopathy, a
polyradiculopathy, or a brachial plexopathy. Ulnar neuropathy would have weakness and
wasting of the intrinsic muscles of the hand and parasthesias of the 4th and 5th digits, usually due
to ulnar nerve compression as it passes through the cubital tunnel. A multiple mononeuropathy
would have an acute onset of sharp pain and development of focal numbness and weakness.
Multiple mononeuropathies are commonly found in areas where nerve compression is
uncommon, and this finding usually suggests a systemic vasculitis. Polyneuropathies more
commonly affect the long nerves first and are associated with metabolic or toxic disorders.
Symptoms typically start distally and progress proximally in the characteristic stocking/glove
distribution. Patients may experience tingling or burning sensations, decreased light touch and
pinprick sensation, loss of vibratory sensation, muscular atrophy of the feet, and decreased ankle
reflexes. A radiculopathy would present with pain radiating from the neck to the arm, especially
during movement of the neck and/or arm. Sensory deficits caused by a radiculopathy would be
confined to that nerve root’s distribution. Polyradiculopathy presents with weakness that is just
as likely to be proximal as distal and sensory deficits do not conform to single nerves. Brachial
plexopathy usually presents with pain and weakness confined to one entire extremity.
1.b. As discussed above, pregnancy predisposes a woman to fluid accumulation within the carpal
tunnel, which leads to compression of the median nerve. Other predisposing conditions for
median nerve entrapment are rheumatoid arthritis, myxedema secondary to Grave’s disease, and
acromegaly. Repetitive movements (chronic trauma) or acute trauma can also be a cause of this
patient’s symptoms. Certain conditions, such as diabetes mellitus, Vitamin B12 deficiency, or
sarcoidosis, can lead to a mononeuropathy that could mimic CTS.
1.c. Tinel’s test and Phalen’s test are two commonly-used tools that can help a physician in
diagnosing CTS. Tinel’s test elicits pain and/or parasthesias in the hand(s) when the median
nerve in the wrist is tapped just proximal to where it enters the carpal tunnel. Phalen’s test elicits
these same symptoms when dorsal surfaces of the hands are pressed together in prolonged
flexion. Light touch sensation should also be assessed for in the distribution of the median nerve.
1.d. If a diagnosis cannot be definitively made by history and PE, electrophysiologic studies can
be used to determine if conduction abnormalities are present. Nerve conduction studies can be
used to determine if entrapment is present and if so, how severe the nerve damage is. If
metabolic abnormalities/toxicity is suspected, then labs should be performed to evaluate.
1.e. Therapeutic options for carpal tunnel syndrome include wrist immobilization, avoidance of
instigating activities, and rest. Lidocaine and steroid injections can also be administered into the
carpal tunnel to relieve inflammation and pain. As a last resort, decompression via release of the
flexor retinaculum can be performed.
2.a. Spinal cord lesions often present with little to no atrophy, increased tone, sluggish initiation
of movements, posturing, brisk reflexes, Babinski signs, and central patterns of sensory loss.
These lesions could also show segmental signs at the level of the lesion and autonomic
dysfunction such as bowel or bladder incontinence. Radicular disease often presents with
radiating pain from the lower back down into the leg that is precipitated by movement. Pain is
found in a radicular distribution and sensory loss is confined to a dermatomal distribution.
Autonomic dysfunction is not present with radicular lesions. Peripheral nerve lesions present
with distal lower extremity numbness, decreased deep tendon reflexes, down-going toes, muscle
atrophy and fasciculations, and sensory loss. Like radicular lesions, automonic dysfunction is not
common with peripheral nerve lesions either.
2.b. This patient’s symptoms are most likely due to a metabolic/toxic polyneuropathy. This
patient’s symptoms are characteristic of this deficit: symptoms initially in the feet that progress
proximally up the legs and eventually affecting the hands and arms last. Other polyneuropathy
symptoms present in our patient include tingling/burning sensations and loss of vibration
sensation in the toes, atrophy of the intrinsic foot muscles, and reduced or absent ankle jerk
reflexes, all of which are present in our patient. Sensory loss in a stocking/glove pattern is also
common in these patients.
2.c. This patient’s ED is likely associated with the same disorder that is causing his peripheral
nerve symptoms. Diabetics with peripheral vascular disease and polyneuropathy often present
with ED, as erections are achieved and maintained via the parasympathetic nervous system.
Other signs/symptoms of autonomic dysfunction include incontinence, GI dysmotility,
gastroparesis, and orthostatic changes.
2.d. The differential diagnosis in this patient includes diabetic or uremic polyneuropathy
(secondary to endocrine or liver dysfunction), toxic or drug-related polyneuropathy (from
alcohol, heavy metals, NO, or organophosphate poisoning), nutritional deficiencies (B12, folate,
thiamine, or B6 deficiency) and inherited or autoimmune causes (CVDs or Charcot-marie-tooth
disease). The diagnostic work-up should include a CBC to look for anemias, B12 and folate
deficiency, ESR and CRP, TSH, SPE, and CMP to look for metabolic abnormalities such as
uremia. An OGTT should be performed and a HbA1c should be obtained to assess for diabetes,
and a review of alcohol, drugs, and medication use should be done.
3.a. This patient’s most likely diagnosis is Guillain-Barre Syndrome, which is a type of
autoimmune polyradiculoneuropathy. It is preceded by infection or stressors in over half of all
cases, and some causes such as Campylobacter, CMV, and EBV have been identified. As in our
patient, GBS is characterized by rapid development of generalized weakness, loss of DTRs, and
minor sensory loss. The differential diagnosis includes the Miller-Fisher variant of GBS in
which there is prominent sensory loss, chronic inflammatory demyelinating
polyradiculoneuropathy, which is a chronic form of GBS, multiple myeloma, Waldenstrom’s
macro-globulinemia or other benign monoclonal gammopathies.
3.b. Due to the rapid development of muscle weakness in GBS, the infirmary physician should
have sent the student immediately to the ER where she could have been seen by neurology. GBS
progresses rapidly over just a few days and can be fatal if respiratory depression or circulatory
collapse (shock) develops unexpectedly.
3.c. During the patient’s hospital stay, her respiratory function should be monitored closely as
respiratory failure secondary to muscle weakness can occur rapidly and unexpectedly. Vital
signs, ABGs, and PFT’s can all be used to monitor respiratory function, and if it deteriorates,
intubation can be performed. Patients with autonomic instability can be monitored in the ICU.
3.d. Treatment for GBS most commonly consists of supportive treatment for up to 4 weeks to
prevent the possibly fatal consequences mentioned above. IVIG or plasma exchange can be of
some benefit in severe cases.
4.a. If considered individually, most of the patient’s symptoms can be localized to a specific
nerve. The patient’s symptoms in the anterior compartment of his left leg consisting of pain,
numbness, foot-drop, atrophy, and weakness of dorsiflexion and eversion are evidence of
peroneal neuropathy. The patient’s weakness and pain in the lateral 3 fingers and thumb,
numbness of the palmar surface of the thumb, middle, and index finger, and weakness of the
thenar muscles are all consistent with median neuropathy. The patient’s numbness in the medial
2 fingers, weakness of finger spreading, thumb adduction, flexion of the pinky and ring fingers,
and wrist extension are suggestive of ulnar neuropathy. The sequential development of these
neuropathies is suggestive of multiple mononeuropathy, which is most commonly due to a
4.b. Carpal tunnel syndrome is unlikely to be causing the RUE symptoms because it is a gradual
process most commonly due to overuse (chronic trauma) and is often instigated by prolonged
wrist flexion or extension. This usually occurs over the course of many years, unlike the acute
presentation in our patient. Also, this patient presents with a left ulnar neuropathy which further
suggests the diagnosis of multiple mononeuropathy.
4.c. The most likely cause of this patient’s neurologic symptoms is a systemic necrotizing
vasculitis such as polyartertitis nodosa or Wegener’s granulomatosis. This usually manifests as a
sharp pain which represents infarction of the nerve, followed by focal weakness and numbness
and is of acute onset and in sites not common for compression. Our patient presents with this
clinical picture. These disorders are also auto-immunologic in nature, which coincides with this
patients RA history.
4.d. Early recognition and initiation is key to the treatment of these vasculitides. First-line
treatment usually includes high-dose corticosteroids and/or other immuno-suppressive agents.
Early treatment with antiviral agents in hepatitis C-associated vasculitis are also beneficial.
5.a. This patient’s presentation is most suggestive of an aquired disorder of the muscle fibers
themselves, i.e. a myopathy. This patient exhibits progressive proximal muscle weakness by
having difficulty arising from chairs and climbing stairs. He also has normal sensation and
relatively normal reflexes along with normal CNs and mental status. All of this is characteristic
of a myopathy. A CNS lesion would be manifested by little or no atrophy, increased tone,
slowness in initiating movements, posturing, brisk reflexes, Babinski signs, and central patterns
of sensory loss. A PNS lesion would present with decreased deep tendon reflexes, down-going
toes, muscle atrophy and fasciculations, and sensory loss, most often in a stocking/glove
distribution. Lesions of the neuromuscular junction often exhibit muscle weakness without
sensory or autonomic deficits. These lesions also impair proximal extremity and extraocular
5.b. DDx for this patient includes acquired disorders of muscles such as polymyositis,
dermatomyositis, sarcoidosis, viral trichinosis, HIV-related myositis, and drug-induced myositis.
Also causing these disorders could be hyper- or hypothyroidism, acromegaly, and parathyroid
disorders. Inherited myopathies should also be on the differential, however, they are unlikely as
they usually manifest themselves in the childhood or teenage years.
5.c. Lab studies useful for diagnosis would include a serum CK, TSH, T4, CBC, ESR, cortisol
levels, and an HIV test.
5.d. Nerve conduction studies and EMG studies would definitely be of use in diagnosing this
patient. Both Polymyositis and dermatomyositis have abnormal EMG findings. In distinguishing
the locations pointed out in question 5a, nerve conduction studies will be normal with
myelopathies and CNS lesions, and abnormal with GBS. In disorders with PNS involvement,
nerve conduction studies can show some conduction delay. Nerve conduction studies can also be
useful in identifying primary demyelinating disorders and the severity of peripheral nerve
entrapment. In disorders of the NMJ, repetitive stimulation and EMG studies can help with
diagnosis. EMGs are useful for identifying specific types of myopathies as well as neurogenic
causes of muscle weakness and denervation causes.
5.e. Two examples of treatable myopathies include endocrine-related and drug-induced
myopathies. Hyper- and hypothyroidism can be treated with thyroid hormone supplements,
radio-iodine ablation, thyroidectomy, or other medications and can improve once the thyroid
disorder is under control. Accordingly, drug-induced myopathies can be cured by discontinuing
the offending agent.
Neuro Packets- Spine Disease/Myelopathy
Pablo O. Medina
1.a. Other historical information would include more pertinent details concerning the pain, such
as what the patient was doing when the pain began, if it was associated with physical activity, if
he has experienced any recent trauma, or if it is provoked by coughing or straining. I would also
want to know if the patient has ever had pain like this before, whether the patient’s occupation or
daily activities could cause repetitive injury, or whether or not the patient has a history of
obesity, which could predispose this patient to a degenerative disease. I would also want to
know if the patient has a history of cancer. I would like to know what alleviates and aggravates
the pain, if the patient has tried anything to relieve the pain, and if the patient has bowel/bladder
incontinence or gait disturbances.
1.b. I would suggest the straight leg raise test in which the patient should experience radicular
pain. I would also suggest the Lasegue maneuver, which stresses the sciatic nerve and provokes
a radicular pain. If the injury is in the hip joint, pain would not be present using this maneuver.
Another maneuver to distinguish between radicular and hip joint pain is the Patrick’s maneuver
which would elicit pain in the hip joint with a hip joint injury. Accordingly, having the patient
stand on their tiptoes and then drop back heavily onto the heels should elicit pain in a disk
herniation injury. A complete neurologic exam should be performed, specifically assessing gait,
reflexes, and a complete musculoskeletal exam of the back and legs. Given this patient’s
suspected history of a herniated disk, this patient should have a positive straight leg test at less
than 60 degrees. The patient would also have a negative Patrick’s test, and either a positive or
negative Lasegue depending on whether or not the sciatic nerve root is involved.
1.c. Since the neurologic exam is normal, the most like etiologies of this patient’s symptoms are
nerve root or peripheral nerve lesions.
1.d. Since this patient has no neurologic abnormalities on exam, diagnostic studies are not
warranted, and a long weekend of bed rest and conservative therapy should be instituted. In this
type of injury, diagnostic imaging studies are usually not warranted until surgery is being
considered an option for treatment.
1.e. For this patient’s treatment, I would first institute complete bed rest for several days, which
will allow the herniated fragment to move back into place. I would also begin an NSAID for its
analgesic and anti-inflammatory properties. As time passes the patient should be instructed to
gradually increase activity and participate in exercises to help improve strength in the back and
abdominal musculature. Physical therapy may also be helpful in this patient.
2.a. The etiology of this patient’s presentation is most likely cord compression of the cervical
spinal cord at the C6-C7 levels. This is most likely true because of the decreased DTRs in the
biceps, triceps, brachioradialis, and finger flexors. If the lesion were at C5-C6 level, then the
patient would have hyperactive triceps and finger jerk reflexes. His lesion is clinically
significant due to his iliopsoas weakness and narrow-based gait. The decreased ankle and
plantar reflexes may be explained by a diabetic neuropathy, which can mask the expected hyper-
reflexia below the level of the lesion, and the patient’s history of smoking could lead one to
believe that metastatic cancer to the spine could also be the cause. His symptoms came on quite
rapidly over weeks vs. years, which is more indicative of spinal cord compression than a
2.b. This patient’s bladder function should be tested as his history suggests a spinal cord
compression, and not just a nerve root compression. Cord compression can lead to autonomic
dysfunction, which can manifest as acute urinary retention or as chronic incontinence with
urinary urgency. Bladder function can be tested with urinary studies testing different volumes
and pressures, and can be assessed with different maneuvers such as coughing or a Valsalva
2.c. The ankle jerk reflexes in this patient may be diminished rather than hyper-reflexic
secondary to a peripheral neuropathy due to the patient’s diabetic status.
2.d. Diagnostic studies for this patient could include a plain AP/lateral spine film, which can
detect erosion of a vertebral body or pedicle. However, MRI is the definitive study because it can
define the extent metastasis, which is highly probably given this patient’s 100 pack-year history.
We should also obtain a chest x-ray to evaluate for primary lung cancer.
2.e. Suspected spinal metastasis with cord compression should be treated immediately on the day
the patient’s disease is first discovered with both steroids and radiation therapy. One can begin
with 100mg of Decadron IV immediately then tapering down to 2 mg PO over the next few
weeks. Surgery is only recommended if the patient has spinal instability and the lesion should be
biopsied if the primary is unknown.
2.f. If this patient had a lumbosacral plexopathy due to his diabetes, he would most likely have
unilateral deficits and would have marked limb weakness and atrophy in his iliopsoas and quads.
The patient may exhibit femoral nerve deficits with decreased sensation in the femoral area of
the leg and weakness with leg extension. Lastly, the patient may complain of pain in the hip and
thigh that is more severe at nighttime.
3.a. This patient is exhibiting signs and symptoms of degenerative disease of the cervical spine.
One important findings from the neurologic exam that suggest this process is pain beginning in
the right side of her posterior neck and radiating down her arm. She also exhibits difficulty and
pain when rotating her head, with an associated sense of grinding or popping, which is
characteristic of this disease process. The tingling down her lateral arm most likely represents a
radicular impingement. Also important in the neurologic exam would be assessment of trapezius
and cervical paraspinous tenderness, atrophy or muscle wasting, and depressed DTRs. Optimal
treatment should include medical management with NSAIDS and benzodiazepines. Surgery
should be considered if there are severe neurologic deficits and/or functional impairment, but it
may not be successful in relieving pain. Surgery is also recommended if there are gait problems,
spasticity, or spinal instability.
3.b. If the neurologic exam is completely normal, further diagnostic studies are not warranted.
Medical management would be implicated and diagnostic studies would only be of use if surgery
were being considered. Conservative treatment for degenerative cervical spine disease consists of
NSAIDS for pain and inflammation, benzodiazepines qHS for muscle spasms, and spine
stabilization with a soft collar.
3.c. EMG/NCVs are not indicated in the patient because this patient’s symptoms clearly exhibit a
neurodegenerative disease. These studies would not alter the course of conservative treatment
that we would start with (as described above). If the diagnosis of this patient was uncertain, these
tests could more decisively identify an etiology.
4.a. Given this patient’s case history, he does not appear to have a neurological lesion. If a
neurologic(spinal cord) lesion were present this patient could exhibit symptoms of urinary
retention or dysfunction, sensory symptoms, muscle atrophy, spasticity, gait disorder, abnormal
DTRs, or a Babinski sign. Lumbar stenosis or a herniated disk could cause this patient to
experience some neurogenic claudication in the legs, which would be relieved by position
4.b. If this patient experienced neurogenic claudication, i.e. radicular-like pain precipitated by
prolonged standing, sitting, or walking, that is relieved within 5 minutes of ceasing of the
activity, I would consider the diagnosis of lumbar stenosis, a condition caused by hypertrophic
changes in the spinal canal.
4.c. Diagnostic studies for suspected lumbar stenosis should not be performed until surgery has
been decided upon as the treatment of choice, in which case an MRI would be the study of
choice. Also, since lumbar stenosis is caused by hypertrophic changes, the condition is often not
resolved by conservative treatment alone. Although studies are not necessarily warranted in this
patient with his presentation, certain maneuvers such as the Lasegue maneuver and straight leg
raise test can be used to clarify the diagnosis. Also, Waddell signs for symptom magnifiers
should be used to clarify the authenticity of this patient’s pain.
4.d. The most likely diagnosis in this patient is chronic low back pain secondary to this
gentleman’s aging process and history of back trauma.
4.e. My treatment plan would consist of encouraging the patient to partake in physical therapy,
including back and abdominal strengthening exercises to help reduce spinal stress and lose
weight. Water exercises would also be useful in this patient. Further treatment would include
daily NSAID and muscle relaxant treatment, if needed. Narcotic analgesics should be avoided, if
possible, in this patient.