Severe Axonal Sensorimotor Polyneuropathy Research Articles

Severe axonal motor neuropathy after heat stroke

A 22-year-old healthy man experienced heat stroke while hiking and collapsed, resulting in multiorgan failure, cerebral edema, and prolonged intensive care unit stay. He developed flaccid tetraplegia, facial weakness, and dysphagia, and was diagnosed with severe axonal sensorimotor polyneuropathy. Rehabilitation improved dysphagia, but he remained dependent on daily activities and required assistance for transfers. The prognosis of critical illness polyneuropathy is typically good in young, healthy individuals. Peripheral neuropathy is a rare complication of heat stroke due to hyperthermia-induced neuronal injury. As global temperatures continue to rise, it is essential to recognize the potential impact of climate change on the incidence of heat stroke and to implement public health interventions to mitigate its effects.

Peripheral neuropathy caused by severe hypothermia

ObjectiveTo report follow-up data in the evaluation of peripheral neuropathy in a 29-year old female after accidental deep hypothermia (13.7°C) in 1999. MethodsNerve conduction studies (NCS) and electromyography (EMG) were performed 20days after the accident and again after 5months and 1, 3, 5 and 11years. Macro EMG was performed after 3, 5 and 11years. To evaluate small fiber function, RR-interval, sympathetic skin response, quantitative sensory testing and skin biopsy for quantification of intra-epidermal nerve fiber density were performed in 2009. ResultsIn the intensive care unit sensory and motor responses were absent except for the tibial nerves, and EMG showed profuse denervation. Improvement of amplitudes and conduction velocities was seen during the first 5years. Muscular atrophy of hand muscles persisted. Large fibers were involved more extensively than small fibers. ConclusionsA severe axonal sensorimotor polyneuropathy developed in the intensive care unit following severe hypothermia. The mechanism was most likely cold injury to peripheral nerves. SignificanceThe clinical picture and the laboratory findings indicate that even multi-organ dysfunction and, of specific interest in this study, a severe axonal degeneration may come to a good restitution after long time.

Acute onset distal symmetrical vasculitic polyneuropathy associated with acute hepatitis B

Hepatitis B can have varied extrahepatic manifestations involving the skin, renal, haematological and nervous systems. Neurological manifestations in hepatitis B may take the form of Guillain-Barré syndrome and secondary systemic vasculitis-related mononeuritis multiplex. The clinical course of hepatitis B-related, vasculitis-related neuropathy is usually subacute to chronic and clinical evolution is relatively benign. To our knowledge, acute hepatitis B-associated vasculitis manifesting as acute distal symmetric polyneuropathy has not been reported. We report a 60-year-old man who presented with fever, mild hepatomegaly, skin lesions in the form of non-palpable purpura and acute onset distal symmetric sensorimotor polyneuropathy. Serum transaminase levels were raised and viral serological markers revealed acute hepatitis B. The patient remained anicteric throughout his clinical course. Nerve conduction studies showed severe axonal sensorimotor polyneuropathy and histopathological examination of sural nerve biopsy was suggestive of vasculitic neuropathy. The patient was first given a course of intravenous immunoglobulin with the antiviral drug entecavir. The fever subsided after 1week of treatment. The patient was started on prednisolone in addition to the entecavir, and showed significant improvement in motor power and marked resolution in paresthesia after 2weeks of treatment. Thus, acute onset distal symmetric sensorimotor polyneuropathy of vasculitic etiology can be a manifestation of acute hepatitis B.

Slowly Progressive Ataxia, Neuropathy, and Oculomotor Dysfunction

A 54-year-old white man presented with slowly progressive incoordination and weakness. He had normal motor development until, at 16 years of age, he noted difficulty walking and difficulty reading despite normal visual acuity. By the fourth decade of life, he developed poor coordination and balance, as well as inability to walk. In subsequent years, he developed progressive, painless sensory loss, weakness, and atrophy in his distal arms and legs. His vision problems progressed and he also developed dysarthria without dysphagia. Family history was negative except for an uncle who was described as "clumsy." Results of an oculomotor examination were notable for increased square-wave jerks, persistent bilateral gaze-evoked nystagmus with saccadic pursuit, intact vestibulo-ocular reflex, and saccadic dysmetria. He had a mixed dysarthria with flaccid and ataxic characteristics and severe weakness and atrophy in the distal limb muscles. Sensation was diminished to the midforearms and midthighs in all modalities. Deep tendon reflexes were absent throughout, with no response to plantar stimulation. He had marked appendicular ataxia with mild axial ataxia. Magnetic resonance imaging of the brain revealed severe cerebellar atrophy. Results of an electrodiagnostic study suggested a severe axonal sensorimotor polyneuropathy with active and chronic denervation. The differential diagnosis in a patient with ataxia, neuropathy, and oculomotor features is discussed; a methodical approach to the diagnostic workup is suggested; and the final diagnosis is revealed.

Progressive Weakness With Respiratory Failure in a Patient With Sarcoidosis

A 29-year-old African American woman with an 8-year history of biopsy-proven renal sarcoidosis and end-stage renal disease requiring hemodialysis was admitted to the hospital with progressive weakness and shortness of breath for 2 months. Eight months prior to admission, she was prescribed 15 mg of prednisone twice a day and 200 mg of hydroxychloroquine sulfate twice a day for hypercalcemia and elevated angiotensin-converting enzyme level. As her laboratory abnormalities improved, the prednisone dose was gradually decreased, and hydroxychloroquine was continued. Six months earlier, she noticed numbness in her feet and progressive loss of muscle bulk in her feet and hands. She also noticed difficulty reaching overhead, getting out of a chair, and climbing stairs. She denied any pain or muscle cramps. Results of electrophysiological tests at that time, which included nerve conduction studies and needle electromyography, revealed moderately severe axonal sensorimotor polyneuropathy. Her weakness worsened and so she was admitted to the hospital and subsequently transferred to our facility for further management.

Periorbital Ecchymoses Are Not Pathognomonic of the Light-chain Type of Amyloidosis

CASE REPORT A 52-year-old man presented with a progressive 16month course of severe sensorimotor polyneuropathy. He had no personal or familial medical past history. Clinical manifestations appeared in July 2003 and were characterized by decreased sensation and painful paraesthesia of the upper extremities, followed 4 months later by distal weakness. Then, decreased sensation, painful paraesthesia and distal weakness of the lower limbs progressively appeared. Alternating diarrhoea and constipation, Raynaud’s syndrome and erectile dysfunction were noted at the same time. The patient also complained of effort dyspnoea and orthopnoea. Electromyography demonstrated a severe axonal sensorimotor polyneuropathy involving all 4 limbs. Investigations revealed a normal blood count and serum glucose, creatinine, and urea, electrolytes and liver function test. There were no inflammatory biological markers. Classical causes of axonal polyneuropathy, such as alcoholism, vitamin deficiency, diabetes melli tus, dysthyroidea and auto-immune diseases, were ruled out. The suspected dysautonomia was confirmed by an abnormal heart-rate variability on electrophysiological tests. Echocardiography showed thickened ventricular walls with a granular sparkling appearance and impaired diastolic dysfunction. The patient developed periorbital and scleral ecchymoses after vomiting (Fig. 1). Since periorbital ecchymoses are classically considered as pathognomonic of AL amyloidosis, we performed a salivary gland biopsy and a sural nerve biopsy. Amyloid deposits were observed in both figures, but immunofluorescence with anti-light-chain (lambda or kappa) antibodies did not reveal any immunoreactivity. There was no evidence of monoclonal gammopathy in spite of an exhaustive work-up comprising blood and urinary immunoelectrophoresis, nephelometric assay of light chain in serum and an osteomedullar biopsy. Since the AL type of amyloidosis was not confirmed, a molecular analysis of the TTR gene was performed and revealed a heterozygous Ser77Tyr mutation, resulting in a diagnosis of familial TTR type amyloid polyneuropathy. Immunohistochemistry confirmed the transthyretin nature of the amyloid deposits (Fig. 2).

Atypical rat cerebellar immunoreactivity in a patient with familial amyloid polyneuropathy

Objective: To describe an atypical immunoreactivity against Purkinje cells and glia of rat cerebellum in a case of transthyretin (TTR)‐related familial amyloid polyneuropathy (FAP). Background: TTR‐FAP is an autosomal dominant disease, usually presenting as a progressive sensory or sensorimotor polyneuropathy with autonomic disturbances. Case report: A 68‐year‐old man complained of a two‐year history of severe orthostatic hypotension, diarrhea and progressive sensorimotor polyneuropathy. The neurological examination showed a severe distal weakness at the four limbs, areflexia and distal impairment of pin‐prick sensation and proprioception. The patient was bed‐ridden because of the severe hypotension. Routine laboratory studies, autoantibodies, neoplastic markers and immunoelectrophoresis were negative; thyroid function was impaired with reduced levels of TSH, T3 and high level of anti‐tireoglobulin antibodies. An unusual reactivity against Purkinje cell membrane and glial cells of rat cerebellum white matter was found on immunohistochemistry. Western blot for the detection of onconeural antibodies (Hu, Ri, Yo and Amphiphysin) was negative. Electrophysiological studies showed a severe axonal sensorimotor polyneuropathy. Chest x‐ray and chest and abdomen CT scans did not reveal any malignancies. Genetic analysis revealed a Phe 64 Leu mutation on TTR gene. Discussion: The unusual immunoreactivity found in our patient has not been previously described. Our case report may be a warning to clinicians to suspect a genetic origin in patients with polyneuropathy and atypical pattern of neuronal immunoreactivity.

Chronic inorganic mercury induced peripheral neuropathy

We report the clinical features, electrophysiological studies, and morphometric analysis of sural nerve pathology in a patient with polyneuropathy due to inorganic mercury intoxication. He developed slowly progressive generalized paralysis of all limbs after 3 months ingestion of herb drugs which contained mercuric sulfate. Electrophysiologic studies revealed axonal polyneuropathy involving both motor and sensory fibers. Sural nerve biopsy demonstrated axonal degeneration with demyelination and a predominant loss of large myelinated fibers. His muscle strength showed only mild improvement after 2 years' follow-up. We concluded that inorganic mercury exposure may induce severe axonal sensorimotor polyneuropathy in humans and that neurological deficits may persist in severe cases.