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Basedow paraplegia: a possible misnomer.

Common neurological complications of untreated Graves' disease include cognitive dysfunction, tremor, ophthalmopathy, myopathy and polyneuropathy. Myasthenia gravis and seizures are uncommon associations, while thyrotoxic periodic paralysis, stroke and chorea occur only rarely. [1]

We present a patient with Graves' disease and the acute motor axonal neuropathy (AMAN) variant of Guillain-Barre syndrome, which masqueraded as so-called Basedow paraplegia. [1-5] Had the diagnosis of Basedow paraplegia been adhered to, the patient would have been denied the opportunity of receiving gammaglobulin therapy.

Case report

An 18-year-old female was admitted in November 2014 with acute onset of severe global leg and arm weakness that had started 4 days before her admission. There was a background history (over the previous 11 months) of proptosis, dyspnoea on exertion, palpitations, irritability and forgetfulness. General examination revealed tachycardia and a symmetrical diffusely enlarged goitre. Proptosis and lid lag were also present. Neurological examination revealed motor weakness, with her legs more affected than her arms. There was global hypotonia and deep tendon areflexia, with sparing of all sensory modalities. Blood tests confirmed the clinical suspicion of hyperthyroidism. Serum potassium levels were repeatedly normal (Table 1), urine porphobilinogen screening was negative and antiganglioside antibodies were absent.

The electroneuronographic studies of 11 and 25 November 2014 showed significantly decreased compound muscle action potential (CMAP) amplitudes and preserved sensory nerve action potential (SNAP) amplitudes, with normal distal latencies and conduction velocities, favouring a diagnosis of an AMAN variant of the Guillain-Barre syndrome (Tables 2 and 3). Needle examination of the tibialis anterior muscle on 25 November 2014 showed the presence of fibrillation potentials and clear neurogenic polyphasic motor units.

A 5-day course of intravenous immunoglobulins, 24 g/day, was administered. Carbimazole was prescribed at 20 mg 8-hourly, and on the development of a skin reaction the dose was decreased to 10 mg 8-hourly. Propranonol was administered at a dose of 20 mg 6-hourly. The thyrotoxicosis gradually improved, but the patient's neurological condition had only marginally improved at the time of discharge.


Joffroy coined the term Basedow paraplegia in 1894, after Charcot had referred to 'paraplegia like weakness' in severe hyperthyroidism in 1889. [3] Joffroy went on to describe the concept as follows: 'A flaccid paraplegia with absent reflexes, minimal or no sensory disturbance, and absent sphincter disturbances,' [3] which Pandit in 1998 commented 'could very well fit the description of acute post infective polyneuritis'. [3] Feibel and Campa [2] used the term 'thyrotoxic neuropathy (Basedow paraplegia)' in 1976 and Pandit, [3] in 1998, published a report 'Acute thyrotoxic neuropathy--Basedow's paraplegia revisited,' with the suggestion of an implicit causal relationship between the hyperthyroidism and the neuropathy. However, on closer scrutiny this association may well be fortuitous.

Descriptions of Basedow paraplegia [1-5] appear to conform to the development of an 'acute flaccid paraplegia with absent reflexes' against the background of hyperthyroidism. It is, however, possible that this clinical presentation may reflect the occurrence of an acute idiopathic polyneuritis, [6] possibly associated with an underlying predisposition to autoimmune diseases. [1]

The association between hyperthyroidism and acute flaccid areflexic neuropathy receives little credence in the following well-known clinical textbooks: Dyck and Thomas' Peripheral Neuropathy [7] comments on its uncertain association and the difficulty to distinguish it from acute idiopathic polyneuritis; Bradley's Neurology in Clinical Practice [8] refers to its association as fortuitous; and Williams's Textbook of Endocrinology [9] and Harrison's Internal Medicine [10] do not even mention the association.


It is important to consider the occurrence of other treatable causes of motor paralysis in patients with Graves' disease, such as acute idiopathic polyneuritis presenting with a rapid onset of flaccid paralysis. The entity of Basedow paraplegia as a diagnosis, per se, was found to be misleading.

Acknowledgements. We thank Mr Sagren Naidoo for his valuable assistance.


[1.] Rubin DI, Amin off MJ, Ross DS, Wilterdink JL. Neurologic manifestations of hyperthyroidism and Graves' disease. In: Basow, ed. UpToDate. Waltham, MA: UpToDate, 2013.

[2.] Feibel JH, Campa JF. Thyrotoxic neuropathy (Basedow's paraplegia). J Neurol Neurosurg Psychiatry 1976;39(5):491-497.

[3.] Pandit L, Shankar SK, Gayathri N, Pandit A. Acute thyrotoxic neuropathy -Basedows paraplegia revisited. J Neurol Sci 1998; 155(2):211-214. [http://dx.dolLorg/10.1016/S0022-510X(97)00313-4]

[4.] Sanghvi LM, Gupta KD, Bauerjee K, Bose K. Paraplegia, hypokalaemia and nephropathy, with muscle lesions of potassium deficiency, associated with thyrotoxicosis. Am J Med 1959;27:817-823.

[5.] Fridberg DL, Egart FM. 1A case of Basedow's paraplegia.! Probl Endokrinol (Mosk) 1970;16:38-40.

[6.] Bronsky D, Kaganiek GI, Waldstein SS. An association between the Guillain- Barre syndrome and hyperthyroidism. Am J Med Sci 1964;247(2):196-200.

[7.] Pollard JD. Neuropathy in diseases of the thyroid and pituitary glands. In: Dyck PJ, Thomas PK, eds. Peripheral Neuropathy. 4th ed. Philadelphia: Elsevier Saunders, 2012:2043-2044.

[8.] Aminoff MJ, Josephson SA. Neurological complications of systemic disease: Adults. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, eds. Bradley's Neurology in Clinical Practice. 6th ed. Philadelphia: Saunders Elsevier, 2012: 894-915.

[9.] Davies TF, Larsen PR. Thyrotoxicosis. In: Kronenberg HM, Melmed S, Polonsky KS, Larson PR, eds. William's Textbook of Endocrinology. 11th ed. Philadelphia: Saunders Elsevier, 2008:333-375.

[10.] Amato AA, Brown RH. Muscular dystrophies and other muscle diseases. In: Longo DL, Kasper DL, Jameson JL, Fauci AS, Hauser SL, Loscalzo J, eds. Harrison's Principles of Internal Medicine. 18th ed. New York: McGraw-Hill, 2012:3487-3509.

L Smith, (1) MB ChB, DOH&M, MMed (Int Med), Cert Endo & Metab (SA) Phys; T Kemp, (1) MB ChB, MMed (Int Med), Cert Endo & Metab (SA) Phys, MSc (Clin Epi); C H van der Meyden, (2) MB BCh, FCP, MD; C-M Schutte, (2) MB ChB, MMed (Neurol), MD

(1) Division of Endocrinology, Department of Internal Medicine, Faculty of Health Sciences, University of Pretoria, South Africa

(2) Department of Neurology, Faculty of Health Sciences, University of Pretoria, South Africa

Corresponding author: L Smith (
Table 1. Laboratory results

                                                       1 week after
                                        On admission   admission

TSH (mIU/L) (0.48-4.26)                 0.08
Free thyroxine (pmol/L) (7.6-16.1)      55             30.4
Potassium (mmol/L) (3.5-5.1)            4.9            4.0
TSH receptor antibody (U/L) (< 1.75)    36.26
Cerebrospinal fluid protein (g/L)       0.86

                                        2 weeks after

TSH (mIU/L) (0.48-4.26)
Free thyroxine (pmol/L) (7.6-16.1)      27.5
Potassium (mmol/L) (3.5-5.1)
TSH receptor antibody (U/L) (< 1.75)
Cerebrospinal fluid protein (g/L)

TSH = thyroid-stimulating hormone.

Table 2. Electroneuronographic study, 11 November 2014

             Motor nerve responses

                      CMAP           Normal
Nerve        Muscle   amplitude      values

R median     APB      2.6 mV         > 4.0 mV
R ulnar      ADM      530 [micro]V   > 6 mV
R peroneal   EDB      127 [micro]V   > 2 mV
R tibial     AH       1.7 mV         > 4 mV
R sural

             Sensory nerve responses

             Peak      SNAP            Normal
Nerve        latency   amplitude       value

R median     Palmar    50.7 [micro]V   > 50 [micro]V
R ulnar      Palmar    34.5 [micro]V   > 15 [micro]V
R peroneal
R tibial
R sural      Point B   24.0 [micro]V   > 6 [micro]V

R = right; CMAP = compound muscle action potential; SNAP =
sensory nerve action potential; APB = abductor pollicis brevis;
ADM = abductor digiti minimi; EDB = extensor digitorum brevis; AH
= abductor hallices. The R median, ulnar, peroneal and tibial
nerve latencies were 2.8 ms, 2.4 ms, 3.0 ms and 3.5 ms,
respectively (within normal limits), and their conduction
velocities 54.4 m/s, 70.3 m/s, 43.4 m/s and 55.3 m/s,
respectively, were also normal. The sensory peak latencies of the
R median (palmar), R ulnar (palmar) and R sural (point B) nerves
were 1.9 ms, 2.0 ms and 3.3 ms, respectively (within normal
limits), and their conduction velocities 56.3 m/s, 51.3 m/s and
51.5 m/s, respectively (within normal limits).

Table 3. Electroneuronographic study, 25 November 2014

             Motor nerve responses

                      CMAP             Normal
Nerve        Muscle   amplitude        values

L median     APB      2.2 mV           > 4.0 mV
L ulnar      ADM      339.0 [micro]V   > 6 mV
L peroneal   EDB      195.0 [micro]V   > 2 mV
R peroneal   EDB      92 [micro]V      > 2 mV
L tibial     AH       No response      > 4 mV
R tibial     AH       853 [micro]V     > 4 mV
L sural

             Sensory nerve responses

             Peak      SNAP            Normal
Nerve        latency   amplitude       value

L median     Palmar    52.3 [micro]V   > 50 [micro]V
L ulnar      Palmar    17.9 [micro]V   > 15 [micro]V
L peroneal
R peroneal
L tibial
R tibial
L sural      Point B   14.1 [micro]V   > 6 [micro]V

L = left; R = right; CMAP = compound muscle action potential;
SNAP = sensory nerve action potential. The L median, L ulnar, L
peroneal, R peroneal and R tibial nerve distal latencies were 2.6
ms, 2.6 ms, 4.4 ms, 4.9 ms and 3.8 ms, respectively (within
normal limits), and their conduction velocities 46.8 m/s, 56.6 m/
s, 43.2 m/s, 50.0 m/s and 52.2 m/s, respectively, were also
essentially' normal. The sensory peak latencies of the R median
(palmar), R ulnar (palmar) and R sural (point B) nerves were 2.0
ms, 2.2 ms and 3.8 ms, respectively (within normal limits), and
their conduction velocities 54.6 m/s, 50.6 m/s and 46.1 m/s,
respectively, were also 'essentially' normal.
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Article Details
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Title Annotation:CASE REPORT
Author:Smith, L.; Kemp, T.; van der Meyden, C.H.; Schutte, C.-M.
Publication:South African Medical Journal
Article Type:Clinical report
Date:Oct 1, 2015
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