ACUTE QUADRIPLEGIA: AN EXERCISE IN DIFFERENTIAL

DIAGNOSIS:

DR.RAM

(E-mail: mraman@pc.jaring.my).

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Summary.

This report deals with a 25-year old woman who presented with acute
muscle weakness. She generated a gamut of possibilities at the time of
presentation. By exclusion, her weakness was found to be related to diabetes
mellitus. The rare presentation of a common problem is highlighted as it
appears to be potentially reversible with appropriate treatment.

Key Words: acute muscle weakness, diabetic neuropathy, amyotrophy.

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Introduction.

Diabetes mellitus can involve every segment of the peripheral and

autonomic nervous system (1, 2). The diagnosis may be straight forward
when one encounters the classical peripheral neuropathy in a patient known
to suffer from diabetes mellitus. On the other hand one might easily miss the
diagnosis in a patient in whom a neurological emergency heralds the onset
of diabetes mellitus for the first time.

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Case report:

A 25-year old university student was admitted to the hospital because of

muscle weakness. She was well till 2 weeks earlier when she developed
fever with chills and rigors. There was little response to the various
antibiotics and supportive measures she received from her doctors. She had
no cough, urinary symptoms or bowel changes. Her appetite was poor and
had lost some weight.

A week later she noticed her legs were getting weak. Soon she was not able
to lift her arms as well. Initially she could walk with assistance from her
parents. But two days before entry, she was completely bedridden which led
to the admission.

She did not have any sensory symptoms. There was no muscle pain. She had
no double vision, difficulty in swallowing or in breathing. She did not notice
any diurnal variation in her symptoms. There was no involvement of her
sphincters. But she had to resort to diapers after she was bedridden.

There was no history of drug, tobacco or alcohol abuse. The family history
was negative for any form of muscle weakness.

Later on when told of the possibility of diabetes mellitus in her, she admitted
that she was experiencing polydypsia for the past week and has been
consuming a lot of canned drinks. On direct questioning, her father admitted
being a diabetic himself.

On examination she was febrile with a temperature of 390C. She was toxic
and dehydrated. Her pulse was 120 beats per minute and regular. Blood
pressure was 110/70 mm Hg. The findings were otherwise localised to her
nervous system. She was exhausted with a short attention span but was able
to cooperate with some coaxing. Her higher functions were normal. The
cranial nerves and sensation were intact. The power in her lower limbs was
0/5 proximally and 2/5 distally. In the upper limbs it was 0/5 proximally and
3/5 distally. The deep tendon reflexes were absent. The plantar response was
normal. There was no wasting.

As the initial impression was one of Guillain Barre Syndrome, the patient
was advised on the need for a lumbar puncture (LP) to sample the cerebral
spinal fluid (CSF). Despite repeated requests, she refused the procedure.

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The pulse oximeter on admission showed her oxygen saturation to be 100%.
The resting electrocardiogram (ECG) showed sinus tachycardia with a heart
rate of 116. An arterial blood gas analysis was normal.

Her blood was withdrawn for routine blood tests and culture. She was then
started on a normal saline infusion and IV Ceftriaxone 2 gm daily while
waiting for the laboratory results to return.

Her blood sugar was 63.9 mmol/L. The other laboratory results are shown in
Table 1. The liver and thyroid function tests were normal. Her urinalysis
confirmed heavy glycosuria with traces of ketones and protein. The
microscopic examination of the urine showed numerous pus cells. The blood
and urine cultures were positive for Escherichia coli sensitive to the
antibiotic prescribed earlier.

Her intravenous normal saline infusion was stepped up once the laboratory
results were known. She was also started on an insulin infusion. Potassium
chloride was added to the saline infusion. Her renal profile and glucose
levels were monitored regularly and adjustments were made to her treatment
regime accordingly.

She became more alert within a few hours of commencing her on treatment.
The power in her limbs showed gradual improvement and she was able to sit
up without much support on the third day. She was able to walk normally
and brush her teeth on her own a week later. Her fever too settled quickly.

As she refused to go on regular insulin, her diabetes was managed with oral
hypoglycaemic agents in addition to nutritional therapy and regular exercise.
She was discharged well from the hospital on the tenth day. Although her
limb weakness resolved quickly, it took another two weeks before her
reflexes became normal. She has since then returned to the university to
complete her studies.

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Discussion.

Several possibilities were considered in this patient at admission (Table 2). It

should be noted that diabetes mellitus was not on the card!

The acute weakness in a febrile patient did raise the possibility of spinal
cord problems, viz epidural abscess and transverse myelitis. An acute flaccid
paralysis is a known presentation in transverse myelitis. But it tends to
evolve into a hyperreflexive paresis during the course of the illness. Further
the absence of a sensory level and sphincter involvement were against
transverse myelitis in her.

Diabetes mellitus is a known predisposing factor for the development of

spinal epidural abscess which is usually due to staphylococcus aureus
infection (3). The absence of neck or radicular pain, tenderness over the
spine (3), bladder disturbance and upper motor neurone signs in the lower
limbs spelt against an epidural abscess at the cervical region as the cause of
her malady.

The patient’s classical acute motor polyneuropathy in her limbs with no

sensory or sphincter involvement was highly suggestive of Guillain Barre
Syndrome (GBS). In fact, GBS was the provisional diagnosis. But patients
with GBS are usually afebrile at presentation. As the patient refused an LP
further avenues to confidently exclude GBS were closed. If the patient had
not shown such dramatic improvement with the treatment for her diabetes
mellitus, it would have been difficult to ignore the possibility of GBS in her.

The sparing of the ocular and bulbar muscles with loss of deep tendon
reflexes deleted myasthenia gravis from the list.

Polymyositis (PM) was ticked off on clinical grounds. It tends to occur in a

more insidious manner in the fifth decade of life. The normal creatine
phosphokinase (CPK) level virtually excluded PM from this patient.

In the absence of a relevant history with normal CPK levels the possibility
of drug or alcohol induced rhabdomyolysis leading to acute muscle
weakness was erased off the slate quickly.

Familial Periodic Paralysis (FPP) was thought to be unlikely in the absence

of family history, similar previous episodes and normal potassium levels.

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Furthermore, the weakness in FPP tends to resolve spontaneously within 24
hours.

Although thyrotoxic periodic paralysis (TPP) has been classically described

as a problem of oriental males, it has been reported among females as well
(4). TPP may be the only manifestation of thyrotoxicosis. The normal serum
potassium level with no hypokalemic changes in her ECG as well as the
results of the thyroid function tests conclusively excluded TPP in her.

Diabetes mellitus was not thought of on admission. It was the routine blood
sugar estimation which alerted us to reassess her problem! Most reviewers
have stressed the protean neurological manifestations one can encounter in a
diabetic patient (1, 2). In this patient it was difficult to classify the exact type
of neuropathy as neurophysiological studies were not possible for want of
facilities.

On clinical grounds she could not be classified as a case of diabetic

amyotrophy. Diabetic amyotrophy is a problem of the elderly. But it has also
been reported in young patients (5). The symmetrical proximal weakness of
both upper and lower limbs in the absence of pain is rather unusual in
diabetic amyotrophy. Moreover the rapid resolution of her weakness is not
typical of the condition (1).

An acute onset of predominantly a motor polyneuropathy with full recovery

after a few weeks, albeit rare, has been reported in diabetic ketoacidosis (2).
The possible mechanism postulated for this rapidly reversible
polyneuropathy includes a metabolic derangement and/or a transient
haemodynamic change in nature (2). Whether the same hypothesis holds true
for this patient remains a speculation.

Whatever the pathophysiology might be, this patient’s experience reminds us

of the importance of determining the blood glucose in the assessment of an
acute neurological emergency.

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 Table 1.

Initial Laboratory Results.

Haemoglobin: 130g/L.
Red cell indices: Normal.
Total White cell count: 22.0x109/L.
Differential Count:
Neutrophils 93%,
Lymphocytes 5%,
Monocytes 2%.

Platelets: 285x109/L.

ESR: 92mm/hour.

Renal Profile
Blood urea: 15.8 mmol/L (2.5-8.0).
Creatinine: 0.163 mmol/L (0.050-0.110).
Uric acid: 0.79 mmol/L. (0.15-0.45)
Calcium: 2.20 mmol/L (2.10-2.60).
Phosphate: 1.05 mmol/L (0.65-1.45).

Serum Electrolytes.
Sodium: 122 mmol/L (135-145).
Potassium: 4.2 mmol/L (3.5-5.1).
Chloride: 73 mmol/L (95-110).

Lipids.
Total cholesterol: 3.5 mmol/L (< 5.2).
Triglycerides: 5.90 mmol/L (<1.68).
HDL cholesterol: 0.37 mmol/L (>1.03).

Enzymes.
Creatine phosphokinase (CPK): 98 U/L (<201).
Lactate Dehydrogenase (LDH): 376U/L (110-240).

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 Table 2.

Differential Diagnosis on admission:

1. Spinal cord problems:

Transverse myelitis.
Epidural abscess.

2. Polyneuropathies.
Guillain Barre Syndrome.

3. Neuromuscular Junction disease:

Myasthenia gravis.

4. Muscle disorders:
Polymyositis.
Drug/alcohol induced rhabdomyolysis.
Familial Periodic Paralysis.
Thyrotoxic Periodic Paralysis.

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References:

1. Gooch C, Podwall D. The diabetic neuropathies. The

Neurologist 2004; 10:311-322.

2. Sinnreich M, Taylor BV, Dyck PJB. Diabetic neuropathies:

classification, clinical features and pathophysiological basis.
The Neurologist 2005; 11: 63-79.

3. Soehle M, Wallenfang T. Spinal epidural abscess: clinical

manifestations, prognostic factors and outcomes. Neurosurgery
2002; 51: 79-87.

4. Lin SH. Thyrotoxic periodic paralysis. Mayo Clin Proc 2005;

80: 99-105.

5. Varthakavi PK, Meisheri YV, Nihalani KD. Amyotrophy in

young diabetics: clinical profiles. J Assoc Phy Ind.1990; 38:
206-10.

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