|Year : 2000 | Volume
| Issue : 3 | Page : 263--5
Hypoglycaemic neuropathy : a case report.
V Puri, N Garg, N Kumar, M Tatke
Departments of Neurology and Pathology, G.B. Pant Hospital, New Delhi, 110002, India., India
Departments of Neurology and Pathology, G.B. Pant Hospital, New Delhi, 110002, India.
A 53 years old male, a known case of ankylosing spondylitis having recurrent attacks of hypoglycaemia, developed symmetrical distal sensorimotor neuropathy. The neuropathy was axonal with secondary demyelination. Evidence of vasculopathy was also noted on histopathology of the nerve. Serum C-peptide level was low, a feature reported with autoimmune hypoglycaemia with antireceptor antibodies. The patient showed spontaneous recovery.
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Puri V, Garg N, Kumar N, Tatke M. Hypoglycaemic neuropathy : a case report. Neurol India 2000;48:263-5
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Puri V, Garg N, Kumar N, Tatke M. Hypoglycaemic neuropathy : a case report. Neurol India [serial online] 2000 [cited 2020 Sep 27 ];48:263-5
Available from: http://www.neurologyindia.com/text.asp?2000/48/3/263/1524
Neurologic manifestations are common in patients with symptomatic hypoglycaemia. These vary from obtudation, stupor, behavioural abnormalities to seizures and transient paresis. Hypoglycaemic neuropathy almost always occurs in association with recurrent central nervous system symptomatology related to hypoglycaemia or may follow a severe attack.,, Peripheral neuropathy secondary to hypoglycaemia is rare and about 30 such cases have since been reported. A case of peripheral neuropathy associated with hypoglycaemia is being presented.
A 53 years old veterinary surgeon was admitted with history of recurrent episodic behavioural abnormalities and alteration of sensorium for about two years and wasting, numbness of hands and feet for two months. The episodes of abnormal behaviour consisted of confusion, irrelevant talk and delusions associated with aggression and violence. The patient had 3-4 such episodes in four months. The EEG reportedly showed left temporal spike and waves. He was diagnosed as having complex partial seizures and was put on carbamazepine (CBZ) 600 mg/day. A symptom free period of one year was followed by recurrent spells of loss of consciousness. These spells were of variable duration and the longest was upto an hour. The dose of CBZ was increased to 1200 mg/day and phenytoin (300 mg/day) was added to the regime. Despite this, the episodes continued and became more frequent (about 4-5/week). These episodes were noticed to be more in the mornings, and blood sugar estimation done during one such episode was 31 mg/dl.
In addition to above mentioned episodes, the patient developed weakness and wasting of hands and feet along with numbness. This progressed over two months and he became bed ridden. He had iridocyclitis in the right eye about 10 years back. The patient had nuchal pain for the past twenty years along with backache and restriction of truncal movements. There were no systemic symptoms like malaise, anorexia or weight loss. He was nonalcoholic and there was no history of exposure to any toxin. No other family member had any neurologic disease. He had an exaggerated thoracic kyphosis and obliterated lumbar lordosis. The chest expansion was 1.5 cm. Spinal mobility was markedly restricted in all directions. Neurologic examination revealed his mental state and cranial nerves to be normal. He had evidence of distal weakness and wasting of both hands and feet with bilateral foot drop. The power of both extensors and flexors of wrist was 2/5 while at ankle it was 0/5. No fasciculations were observed. No limb or truncal ataxia was present. Bilateral ankle jerks were absent. There was glove and stocking type of sensory loss, in all four limbs, to all modalities.
The investigations showed haemoglobin 9 gm%, and ESR 15 mm first hour. Biochemical parameters for liver function tests, blood urea, serum creatinine, electrolytes, calcium and phosphorus were normal. Blood sugar estimation done on multiple occasions revealed values ranging from 24-42 mg%. Plasma insulin was 13.2 micro IU/ml (normal 0-3 micro IU/ml) with corresponding sugar value of 24 mg%. The insulin glucose ratio was 0.55 (normal<0.4). Serum C-peptide level was 0.7 ngm/ml (normal 0.8-4.0 ngm/ml). ANF, anti ds DNA antibodies, LE cell phenomenon and rheumatoid factor were negative. EEG was normal. Roentgenogram of chest, abdomen, skull were normal. X-rays of spine (cervical, dorsal and LS) were consistent with the diagnosis of ankylosing spondylitis. Slit lamp examination revealed old synechiae in the right eye with no evidence of active iridocyclitis. Pulmonary function tests showed a moderately severe restrictive defect. Ultrasound abdomen, CT scan of head and abdomen, celiac axis angiogram and barium meal follow through were normal.
On electrophysiological testing both median nerves, right common peroneal (CP) and right posterior tibial (PT) nerves could not be stimulated while both left PT and CP had prolonged distal latencies (11.0 msec; 17.8 msec) and delayed conductions (28.0 m/sec; 18.9m/sec respectively). SNAPs were not recordable in any of the nerves. BAEPs and VEPs were normal on either side. The right sural nerve biopsy [Figure. 1] and 2) showed decrease in number of myelinated fibres and endoneural fibrosis. The Schwann cell nuclei were increased in number. There was evidence of remyelination with onion bulb formation, thickening of arterial and capillary walls and focal loss of axons. However, no inflammatory cells were seen. Reassessment of patient after three months revealed marked improvement in wasting and weakness.
The hypoglycaemic neuropathy is predominantly distal and symmetrical. Rarely proximal muscle weakness may also be seen., Motor conduction velocities may be normal or slightly prolonged.,, The present case had features of sensorimotor neuropathy on the background of recurrent episodic neuropsychiatric manifestations, secondary to hypoglycaemia. The electrophysiological studies were suggestive of primary axonal neuropathy with evidence of secondary demyelination. The pathologic changes in hypoglycaemic neuropathy may include axonal neuropathy, anterior horn cells destruction in cervical spinal cord with normal dorsal and ventral roots and dorsal root ganglia, or even a normal nerve. Neuropathologic findings in the present case were suggestive of axonal damage with evidence of demyelination along with vasculopathy.
Investigations to rule out insulinoma including ultra sound, CT scan abdomen and celiac angiogram were all normal. Recurrent hypoglycaemia in the absence of insulinoma in the present case could be immune mediated. Two types of antibodies have been found to be associated with hypoglycaemia i.e. antireceptor, or against insulin. Antireceptor antibodies are mostly found in association with autoimmune disease e.g. systemic lupus scleroderma, primary biliary cirrhosis and Hashimoto's thyroiditis. They mimic the action of insulin or may be associated with insulin resistance. The hypoglycaemia in these cases may be associated with high levels of available insulin. The C-peptide levels are low and the titre of antireceptor antibodies usually falls with time. The symptoms generally remit. The second type of antibodies are those associated with insulin auto-immune syndrome (IAS) and are against insulin. The IAS has been reported with autoimmune disease or drugs like hydralazine, methitmazole, penicillamine and glutathione. C-peptide levels are normal in these patients., Hypoglycaemia caused by these antibodies causes axonal degeneration and microvascular abnormalities. Hypoglycaemia has been shown to produce axonal damage in experimental animals as a result of generalised deficiency of energy substrate. The peripheral neuropathy in cases of insulinoma is perhaps due to hyperinsulinaemia rather than due to hypoglycaemia.,
The present case had an autoimmune disease, ankylosing spondylitis with recurrent hypoglycaemia, low level of C-peptide and showed spontaneous recovery. He was not on any drugs before the onset of symptoms. The profile is like insulin receptor antibody induced hypoglycaemia. The evidence of vasculopathy on histopathology of nerve is again a pointer towards the existence of an autoimmune mechanism.
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