Nerve Root Enhancement Research Articles

Brainstem abnormalities and vestibular nerve enhancement in acute neuroborreliosis: a case report

BackgroundBorreliosis is a widely distributed disease. Neuroborreliosis may present with unspecific symptoms and signs and often remains difficult to diagnose in patients with central nervous system symptoms, particularly if the pathognomonic erythema chronica migrans does not develop or is missed. Thus, vigilance is mandatory in cases with atypical presentation of the disease and with potentially severe consequences if not recognized early. We present a patient with neuroborreliosis demonstrating brain stem and vestibular nerve abnormalities on magnetic resonance imaging.Case presentationA 28-year-old Caucasian female presented with headaches, neck stiffness, weight loss, nausea, tremor, and gait disturbance. Magnetic resonance imaging showed T2-weighted hyperintense signal alterations in the pons and in the vestibular nerves as well as bilateral post-contrast enhancement of the vestibular nerves. Serologic testing of the cerebrospinal fluid revealed the diagnosis of neuroborreliosis.ConclusionPatients infected with neuroborreliosis may present with unspecific neurologic symptoms and magnetic resonance imaging as a noninvasive imaging tool showing signal abnormalities in the brain stem and nerve root enhancement may help in establishing the diagnosis.

Cauda equina involvement in Susac's syndrome

Susac's syndrome is a rare autoimmune microangiopathy characterized by the clinical triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss. In many cases, the clinical triad is not fully present at the onset of symptoms. MRI studies often show characteristic punched out lesions of the central fibers of the corpus callosum, and leptomeningeal enhancement and deep gray matter lesions may also be seen. Here we present a case of Susac's syndrome in a middle aged man with the unique clinical finding of cauda equina syndrome and spinal MRI showing diffuse lumbosacral nerve root enhancement. Biopsy specimens of the brain, leptomeninges, and skin showed evidence of a pauci-immune endotheliopathy, consistent with pathology described in previous cases of Susac's syndrome. This case is important not only because it expands the clinical features of Susac's syndrome but also because it clarifies the mechanism of a disorder of the endothelium, an important target for many disorders of the nervous system.

Vertebral Body Infarct and Ventral Cauda Equina Enhancement: Two Confirmatory Findings of Acute Spinal Cord Infarct

Two valuable confirmatory MRI findings of acute spinal cord infarct are highlighted and discussed: concomitant vertebral body infarct and ventral cauda equina nerve root enhancement.

Chronic inflammatory demyelinating polyneuropathy of childhood: clinical and neuroradiological findings

This study aims to report on serial magnetic resonance imaging (MRI) studies and clinical features in a cohort of children with chronic inflammatory demyelinating polyneuropathy (CIDP). Clinical, neuroradiological, and statistical investigations performed on nine children with CIDP were retrospectively reviewed. Pathological nerve root enhancement was categorized according to severity, extension, and morphology. A MRI score was thus obtained, and correlations with the clinical picture and disease course were explored. Intrathecal nerve root enhancement (NRE) of varying degrees was seen in a high percentage of patients. There was no significant correlation between the total MRI score at the first MRI study and either severity or course of the disease. However, we found a significant difference (p = 0.002) in NRE of patients with improving CIDP with respect to those with stable or progressing disease at the time of follow-up MRI. Contrast-enhanced MRI plays a pivotal role in children with CIDP, both for the initial diagnosis as well as a biomarker of clinical evolution, and should be performed in all children with suspected CIDP both at initial presentation and during follow-up. Further multicenter studies on larger cohorts are awaited to determine the ideal timing for follow-up MRI.

Unusual Case of West Nile Virus Flaccid Paralysis in a 10-Year-Old Child

West Nile virus infection is asymptomatic in most cases. West Nile virus neuroinvasive disease includes encephalitis, meningitis, and/or acute flaccid paralysis. In children, acute flaccid paralysis as the solo presentation of West Nile virus disease is rare. It develops abruptly and progresses rapidly early in the disease course. We report on a 10-year-old child who presented with a slowly progressive left leg flaccid paralysis over 4 weeks. He tested positive for West Nile virus in both blood and cerebrospinal fluid. Spinal MRI showed enhancement of the ventral nerve roots. This was also supported by electrophysiological studies. One week after the plateauing of his left leg paralysis, he was readmitted to the hospital with left hand weakness. Complete recovery of his recurrent weakness was observed after prompt 5-day course of intravenous immunoglobulin G therapy. However, no improvement was noticed in the left foot drop. To our knowledge, this is the first case report of West Nile virus disease in children presented with a slowly progressive flaccid paralysis, and a recurrent weakness recovered after intravenous immunoglobulin G administration.

An Unusual Case of Recurrent Guillain-Barre Syndrome of a Different Subtype Five Years after Initial Diagnosis

We present a case of a previously healthy 17-year-old girl with history of Guillain-Barre Syndrome 5 years after initial presentation who presented with bilateral lower extremity pain, worsening dysphagia, subsequent weakness, and decreased reflexes. Cerebrospinal fluid analysis had a prominent lymphocytic pleocytosis. MRI of spine showed significant anterior nerve root enhancement. Electromyogram demonstrated a mild axonal greater than demyelinating motor polyneuropathy and intact sensory responses, with no evidence of conduction block or temporal dispersion, unlike her first presentation that revealed a demyelinating polyneuropathy. The patient recovered with mild subjective weakness following 5 days of intravenous immunoglobulin treatment. This case represents a recurrence of a predominantly motor variant polyradiculoneuropathy distinct from the initial presentation with a lymphocytic predominant CSF pleocytosis, nerve root enhancement on MRI spine, and rapid recovery following treatment with intravenous immunoglobulin.

Nerve ultrasound in a case of chronic inflammatory demyelinating neuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common acquired immune-mediated inflammatory disorder of the peripheral nervous system. The diagnosis is based mainly on the clinical presentation and electrophysiological detection of demyelination. Several MRI studies have demonstrated hypertrophy and abnormal enhancement of spinal nerve roots or brachial plexus in CIDP, but there have been only anecdotal reports of similar sonographic findings. This article reports the sonographic findings of a CIDP case and includes a review of the literature and previously reported cases. This case report highlights the importance of sonography in the localization and recognition of focal nerve enlargements in patients with CIDP. This method could be a helpful tool in the diagnosis of conduction block in CIDP, especially in cases where a nerve segment cannot be explored easily with the inching technique. Systematic data are needed to confirm this observation.

Acute ataxic neuropathy with hyperreflexia

Dear Editor, The ataxic form of Guillain-Barre syndrome (GBS), characterized by acute profound ataxia with negative Romberg sign and no (or minimal) ophthalmoplegia [1], is considered as an incomplete form of Fisher syndrome [2]. However, its nosological relationship to acute sensory ataxic neuropathy was not discussed [3]. A recent comprehensive study shows how ataxic GBS and acute sensory ataxic neuropathy share similar clinical and laboratory features, forming a continuous spectrum under the term: acute ataxic neuropathy without ophthalmoplegia (AAN) [4]. AAN typically presents with areflexia or hyporeflexia. Preserved or brisk reflexes are seen in GBS or Bickerstaff brainstem encephalitis [5, 6], but not in AAN. We report the first case of AAN with hyperreflexia. A 45-year-old male developed unsteady gait, 5 days after experiencing fever and sore throat. He had no prior medical history and abstained from alcohol. On day 2, he developed diplopia at all extremes of gaze, with left hand and foot numbness, and a broad-based gait. He demonstrated bilateral dysmetria, and abnormal left heel-shin test. Eye pursuit, vertical and horizontal eye movements were full, except for convergence. He had no nystagmus. Pupils were 5 mm bilaterally with normal light and accommodation reflexes. Cranial nerves were otherwise intact with no facial or oropharyngeal weakness. Limb power and tone were normal. He had hyperreflexia in all 4 limbs, but with normal jaw jerk and down-going plantar responses. Despite complaining of left-sided distal dysesthesias, objective sensory testing of all modalities was normal, other than an abnormal Romberg’s test. There was no autonomic involvement. His brain MRI showed no abnormalities or Wernicke’s encephalopathy. MRI spine had no myelopathy or ventral nerve root enhancement. His vitamin B1 levels were normal and lumbar puncture on day 3 was slightly traumatic with 181 red blood cells/lL, protein 1.2 g/dL (normal, \0.6 g/dL) and 45 cells/lL of predominantly lymphocytic white blood cells. We diagnosed AAN with unusually brisk reflexes. As he was ambulatory, we treated him conservatively. Median, ulnar, tibial and peroneal nerve conduction studies on day 4 showed normal amplitudes of the compound motor action potentials and normal distal motor latencies. Sensory nerve action potential amplitudes and latencies were normal. F-waves had normal latencies and were not dispersed. However, his somatosensory evoked potentials, recording from bilateral median and posterior tibial nerves, showed prolonged cerebral evoked N18 and P37 latencies (Table 1). Nerve conduction studies, including peripheral sensory nerve potentials on day 10 remained normal, whereas N18 and P37 latencies normalized by day 15. Anti-GQ1b antibodies were absent, but IgG anti-GM1b antibodies were positive in his serum. Upon discharge on day 12, eye convergence and gait had improved while hyperreflexia and left-sided limb dysmetria remained. His dysesthesia had resolved but Romberg’s test remained positive. He remained alert throughout the illness and his reflexes normalized on day 45. L. L. L. Yeo K. Ng Division of Neurology, Department of Medicine, National University Hospital, Singapore, Singapore

Paraneoplastic lower motor neuronopathy associated with Hodgkin lymphoma

Paraneoplastic lower motor neuronopathies have been reported rarely with Hodgkin lymphoma. We report a case of rapidly progressive motor neuronopathy preceding the diagnosis of Hodgkin lymphoma. A 31-year-old woman developed subacute rapidly progressive quadriparesis. Electrodiagnostic studies revealed a severe diffuse disorder of motor neurons and their axons. Symmetric enhancement of the cauda equina motor nerve roots was notable on magnetic resonance imaging scan. Further imaging demonstrated an enlarged supraclavicular lymph node, and biopsy revealed Hodgkin lymphoma. A final diagnosis of paraneoplastic motor neuronopathy was made after investigations for alternative causes of motor neuronopathy were unrevealing. Neurological improvement was seen with combined treatment of the underlying malignancy and intravenous immunoglobulin. Paraneoplastic causes should be considered in the differential diagnosis of subacute motor neuronopathy, as the neurological presentation may precede cancer detection. Combinations of lymphoma treatment and immunotherapy may result in a favorable outcome.

Cauda equina syndrome secondary to intravascular lymphoma

Intravascular lymphoma (IVL) is an extranodal B-cell lymphoma that involves the lumina of small and medium size vessels.1 Neurologic presentations are varied and include encephalopathy, myelopathy, and polyradiculoneuropathy, among other features.1,2 Cauda equina syndrome (CES) with IVL has been identified late, either at autopsy or after complications such as flaccid paraplegia.2⇓–4 Early diagnosis and treatment before disease dissemination is difficult but increases progression-free survival and remission.1 We describe a patient with isolated CES and subtle non-nodular MRI nerve root enhancement, who was identified to harbor IVL by cauda equina biopsy. A 70-year-old man presented with a 4-week history of perineal, patchy lower limb numbness and urinary retention. Neurologic examination disclosed absent ankle reflexes and sensory alterations in the back of his thighs and perineal region in an S2-S5 distribution. He had an absent anal wink. Normal investigations included complete blood count, electrolytes, creatinine, urea, thyroid-stimulating hormone, B12, hepatic enzymes, serology for hepatitis B and C and HIV 1 and 2, and paraneoplastic antibodies. Lactate dehydrogenase was elevated (460 U/L: normal 100–235). Serum protein electrophoresis identified an immunoglobulin G (IgG) κ monoclonal protein (1.3 g/L). CSF had an …

Subacute inflammatory demyelinating polyneuropathy disclosed by massive nerve root enhancement in CMT1A

Subacute inflammatory demyelinating polyneuropathy disclosed by massive nerve root enhancement in CMT1A

A case of segmental zoster paresis with enhanced anterior and posterior spinal roots on MRI

Herpes zoster is an infectious disease caused by reactivation of the varicella zoster virus (VZV) in the dorsal root ganglia [2]. Neurological complications other than sensory abnormalities such as encephalitis, myelitis, and various lower motor neuron diseases such as polyradiculoneuritis as segmental radiculitis have been reported [7]. Segmental zoster paresis is characterized by focal motor weakness that appears in the same segment where the skin eruptions, neuralgia, and sensory symptoms occur, and is a relatively common complication [1, 3, 6, 7]. We report a patient in whom the radiological findings were consistent with aberration of anterior and posterior spinal roots with zoster paresis. A 73-year-old man developed right shoulder pain and herpes zoster eruptions over the C5 dermatome. Two days later, he found it impossible to lift up the right arm. He was admitted to our hospital with 2-week history of shoulder pain and progressive muscle weakness around the right shoulder. Physical examination on admission showed weakness of the upper extremity in the distribution of the right C5 myotome and sensory disturbance in the right C5 dermatome, but no clinical symptoms of myelitis. Laboratory findings showed VZV-IgM level of 4.30 mg/dl (normal \0.8 mg/dl). Examination of cerebrospinal fluid (CSF) showed 102.8 cells/mm, protein content of 66.8 mg/dl, and elevated varicella zoster virus IgG titer (antibody index [4). Polymerase chain reaction (PCR) of CSF fluid was positive for VZV DNA, but no search for the number of copies was performed. Needle electromyography showed neurogenic changes in the right deltoid and biceps brachii muscles. T1-weighted magnetic resonance imaging (MRI) showed contrast enhancement with gadolinium in the right anterior and posterior C5 spinal roots below the 4th cervical vertebra (Fig. 1b), but no abnormal contrast enhancement of the roots of the 4th and 6th spinal nerves (Fig. 1a, c). Furthermore, T2-weighted MRI showed no abnormal intensity in the cervical spinal cord. The patient was treated with intravenous 1,500 mg acyclovir for 7 days together with rehabilitation. Examination about 1 month later showed improvement of muscle weakness and disappearance of the shoulder pain. The MRI findings suggested VZV reactivation in the dorsal root ganglia, which was localized to the anterior and posterior C5 spinal roots, based on the motor and sensory symptoms of C5 dermatome and myotome. The spread of VZV from the posterior spinal root, or anterior and posterior horn, to the anterior spinal root could have resulted from inflammation. CSF analysis indicated pleocytosis and serologically confirmed VZV. Previous studies reported abnormalities of CSF in 61% of patients with VZV and subclinical extension of viral inflammation into the central nervous system [4]. The clinical symptoms and MRI findings in our patient were not confirmative for myelitis. We postulate the spread of VZV either directly or through the CSF, rather than myelitis, from the posterior spinal root to the anterior spinal root in our patient. The cause of the nerve root enhancement was considered to be due to either viral activation itself or associated inflammation. Previous MRI findings in zoster paresis showed myelitis [3], posterior root with anterior horn [9] and posterior horn [8], suggesting myelitis and anterior root [5], suggesting disruption of the blood–brain barrier. Post mortem M. Yoshioka (&) Y. Kurita M. Hashimoto M. Murakami M. Suzuki Department of Neurology, Aoto Hospital, The Jikei University School of Medicine, 6-41-2 Aoto, Katsushika-ku, Tokyo 125-8506, Japan e-mail: m.yoshioka@jikei.ac.jp

Miller Fisher syndrome: brief overview and update with a focus on electrophysiological findings

Miller Fisher syndrome (MFS), a variant of the Guillain-Barré syndrome (GBS), is characterized by ophthalmoplegia, ataxia, and areflexia. The annual incidence is around one patient per one million population. The antiganglioside anti-GQ1b IgG antibody has a role in the pathogenesis of the syndrome, especially of ophthalmoplegia. The presence of this antibody in the serum can be identified in over 80% of the patients, peaking in the first week, whereas albuminocytological dissociation in the cerebrospinal fluid (CSF) appears later. The most consistent electrophysiological findings in MFS are reduced sensory nerve action potentials and absent H reflexes. More variability is seen with F waves and various investigations involving cranial structures. Although there are usually no abnormalities in MFS by routine neuroimaging, in a few cases, contrast enhancement of nerve roots and signs of central nervous system involvement were described supporting the hypothesis of an anti-GQ1b-syndrome, a continuum involving GBS, MFS, and Bickerstaff's brainstem encephalitis. Owing to the lack of randomized trials, treatments used for GBS (intravenous immunoglobulin and plasmapheresis) are usually applied, although from retrospective analyses, the outcome was similar between treated and untreated subjects. The outcome of MFS is usually good with case fatality of < 5%. In the few autopsy cases, macroscopic abnormalities were generally not seen in the nervous system. Microscopic examination of the peripheral nervous system (including cranial nerves) showed segmental demyelination with minimal perivascular infiltration with normal spinal cord and brain stem.

West Nile virus neuroinvasive disease presenting with acute flaccid paralysis and bilateral sensorineural hearing loss

West Nile virus (WNV), is an emerging virus [1]. Most human infections are subclinical or manifest as a mild febrile illness, while fewer than 1% develop a neuroinvasive disease (WNND), including meningitis, encephalitis, and/or an acute flaccid paralysis (AFP) [2–4]. We describe a case of sensorineural hearing loss (SNHL) secondary to WNV infection. A 55 year-old previously healthy and normoacousic man, was admitted (in September 2010) for bilateral hearing loss and leg weakness. He complained, about 2 weeks before, of malaise, fever, generalized fatigue, and diffuse myalgias, initially attributed to a flu-like syndrome. After a few days he noted a mild weakness in both of his legs evolving over a week into a flaccid paraparesis. At the same time, he also developed a sudden bilateral hearing loss. He was afebrile, with stable vital signs. Routine and immunological laboratory data were within the normal range. Neurologic examination was remarkable for a flaccid areflexic paraparesis, weakness being more pronounced in the right leg and proximally (3/5 strength in the right leg, 4/5 strength in the left leg), with flexor plantar responses. No cranial nerve involvement, vestibular, cerebellar, brainstem or meningeal signs were noted. MRI of the brain and whole spine was normal without meningeal, parenchymal or ventral nerve root enhancement. Audiological tests, including the Weber and Rinne tuning fork tests, pure tone and speech audiometry, tympanic admittance, auditory evoked potentials (ABRs) and a screening test for acoustic emissions (OAEs), demonstrated a bilateral, down-sloping SNHL—mild on the left side, severe on the right—with normal acoustic admittance, and impaired OAEs, which were absent in the right ear. Weber test was lateralized to the left side, and the Rinne test was not significant. ABR with click at maximum output level (125 dB SPL) was not evocable by stimulating the right ear and normal for the left ear after latencies correction for the hearing threshold elevation. On the whole, the data were compatible with a bilateral asymmetric cochlear damage. Electrodiagnostic studies showed normal sensory and motor nerve conductions, and moderate decrease of compound motor action potential amplitudes. Late responses were normal. Concentric needle electromyography showed active denervation with a neurogenic recruitment pattern in all four limbs, with a clear predominance in legs. The findings fulfilled the diagnostic criteria for WNV AFP [4, 5]. Neurophysiological examination of cranial nerves and brainstem reflexes studies gave normal results. Since the symptoms began during a work-related stay in Romania where a WNV infection outbreak was occurring [6, 7], we considered the above infection in the differential diagnosis. Serum IgM and low-avidity IgG WNV-specific antibodies were detected (titre:1:1,600; 1:51,200, respectively). The presence of IgMand low avidity IgG WNV-specific I. Casetta (&) E. Cesnik Section of Neurology, Department of Medical and Neurological Sciences of Communication and Behaviour, University of Ferrara, Corso della Giovecca 203, 44121 Ferrara, Italy e-mail: cti@unife.it

A case of neurosarcoidosis with swelling and gadolinium enhancement of spinal nerve roots on magnetic resonance imaging

An 80-year-old woman was admitted to our hospital because of developed sense of constriction in the trunk and gradually progressive numbness and muscle weakness in the upper and lower extremities. Cerebrospinal fluid analysis showed increased cell count and protein level. Gadolinium enhanced magnetic resonance imaging (MRI) of spine showed the enhancement and swelling of bilateral nerve root in the cervical and lumbar segments. Although chest computed tomography showed neither bilateral hilar lymphoadenopathy nor lung lesions and serum angiotensin converting enzyme and lysozyme (ACE) were normal, tuberculin skin test was negative and cell count and CD4/CD8 elevated in bronchoalveolar lavage fluid. Biopsy specimen of scalene lymph node showed noncaseating granuloma. The patient was treated with oral predonisolone, which improved her symptoms and abnormalities on MRI. It is important to consider neurosarcoidosis in the differential diagnosis of polyradiculopathy with swelling and gadolinium enhancement of spinal nerve roots.

A case of Staphylococcus aureus meningitis-associated quadriparesis with its successful treatment with adrenocorticosteroid

A 44-year-old woman was admitted to our hospital because of meningitis, with symptoms of an altered mental state and flaccid quadriparesis. Neurological examination revealed nuchal rigidity, flaccid quadriparesis without tendon reflexes, septic rash and urinary retention. Nerve conduction studies showed diminished F-wave ratios. However, the amplitudes and conduction velocities for bilateral motor and sensory nerves of the upper (medial and ulnar nerves) and lower (posterior tibial and sural nerves) limbs were all normal. Spinal MRI showed gadolinium enhancement of the bilateral sacral nerve roots, indicating radiculitis. In addition, T2*-weighed MRI of the brain revealed multiple microbleeds. Infectious endocarditis was detected on admission, and Staphylococcus aureus infection was confirmed by blood culturing. The patient was diagnosed with meningoradiculitis caused by S. aureus. Although antibiotic therapy did not improve quadriparesis, administration of dexamethasone led to a marked amelioration of the quadriparesis with a resultant complete recovery of the limb muscle powers in three months. Furthermore, as the quadriparesis improved, F-wave ratios gradually returned to normal and hearing loss remained as the only sequela. Therefore, adrenocorticosteroid therapy attenuated radiculitis-induced quadriparesis. Although radiculitis due to S. aureus is extremely rare, it should be considered because delayed treatment can lead to permanent injury and impairment.

Nerve Root Enhancement on Spinal MRI in Pediatric Guillain-Barré Syndrome

Guillain-Barré syndrome diagnosis is based on clinical presentation and supportive diagnostic testing. In its early stage, no single, reliable diagnostic test is available. However, a finding of nerve root enhancement on spinal magnetic resonance imaging may be useful. We evaluated the frequency of nerve root enhancement on spinal magnetic resonance imaging in children with Guillain-Barré syndrome. At a single tertiary pediatric center, we conducted a retrospective chart review of children with Guillain-Barré syndrome who had complete spinal or lumbosacral spinal magnetic resonance imaging with gadolinium administration from January 2002-January 2009. Twenty-four consecutive patients were identified. Spinal nerve root enhancement with gadolinium was present in 92% (22/24) of children with Guillain-Barré syndrome on initial spinal magnetic resonance imaging (95% confidence interval, 0.745-0.978). This finding increased to 100% of patients, after two patients underwent repeat spinal magnetic resonance imaging that did reveal nerve root enhancement. Patterns of enhancement were variable, but involved the thoracolumbar nerve roots in all patients. Enhancement of nerve roots with gadolinium on initial spinal magnetic resonance imaging was frequently present in these children with Guillain-Barré syndrome. Spinal magnetic resonance imaging is a sensitive diagnostic test and should be considered an additional diagnostic tool in select cases.

50 Years Ago in T he J ournal of P ediatrics: Polyneuronitis: Guillain-Barré Syndrome

Blattner R. J Pediatr 1960;57:625-8.This review is called “Polyneuronitis: The Guillain-Barré Syndrome.” The first line then reads “The Landry-Guillain-Barré” syndrome. As a pediatric neurologist who trained in the early 1990s, I began to wonder why Landry's name is typically left off, considering that the prominent clinical features of patients with this disorder were actually described first by him in 1859. Was there something vital that was discovered later that significantly changed the diagnosis or treatment of this disorder? Most believe it is related to the fact that Landry did not describe the classic cerebrospinal fluid (CSF) finding of albuminocytologic disassociation, described later by Guillain-Barré and Strohl. During World War I they found patients similar to Landry's; this added CSF finding helped distinguish it from diseases such as syphilis and tuberculosis, which were prevalent during this time. Were these findings so vital that those who described it were deserving of this disorder being named after them? Although the CSF abnormality is important to this day, this finding takes time to develop. During the first 2 days of the illness, 85% of CSF will be normal, about 30% will still be normal in first week. In addition, although the use of electromyography has made the diagnosis of Guillain-Barré syndrome more definitive, these changes can take over a week to develop.Also the early postulation that this was due to an “allergy” holds true today because this is a postinfectious antibody–mediated inflammation of the myelin sheaths or nerves. The recent swine flu pandemic and quick need for a vaccine brought concern about this disease to the forefront again considering the swine flu vaccine–related cases in the 1970s.Guillain-Barré syndrome is still considered associated with multiple causes, and not a great deal more is known now than was known then. Diagnosis is still delayed because spinal fluid and electromyography changes typically do not develop until after the first week. Despite magnetic resonance imaging findings of enhancement of nerve roots and discovery of specific antibodies, the “Landry, Guillain-Barré, Strohl syndrome” (as it should probably be called) to this day, remains mostly a clinical diagnosis as it was when Landry first described it in 1859. Blattner R. J Pediatr 1960;57:625-8. This review is called “Polyneuronitis: The Guillain-Barré Syndrome.” The first line then reads “The Landry-Guillain-Barré” syndrome. As a pediatric neurologist who trained in the early 1990s, I began to wonder why Landry's name is typically left off, considering that the prominent clinical features of patients with this disorder were actually described first by him in 1859. Was there something vital that was discovered later that significantly changed the diagnosis or treatment of this disorder? Most believe it is related to the fact that Landry did not describe the classic cerebrospinal fluid (CSF) finding of albuminocytologic disassociation, described later by Guillain-Barré and Strohl. During World War I they found patients similar to Landry's; this added CSF finding helped distinguish it from diseases such as syphilis and tuberculosis, which were prevalent during this time. Were these findings so vital that those who described it were deserving of this disorder being named after them? Although the CSF abnormality is important to this day, this finding takes time to develop. During the first 2 days of the illness, 85% of CSF will be normal, about 30% will still be normal in first week. In addition, although the use of electromyography has made the diagnosis of Guillain-Barré syndrome more definitive, these changes can take over a week to develop. Also the early postulation that this was due to an “allergy” holds true today because this is a postinfectious antibody–mediated inflammation of the myelin sheaths or nerves. The recent swine flu pandemic and quick need for a vaccine brought concern about this disease to the forefront again considering the swine flu vaccine–related cases in the 1970s. Guillain-Barré syndrome is still considered associated with multiple causes, and not a great deal more is known now than was known then. Diagnosis is still delayed because spinal fluid and electromyography changes typically do not develop until after the first week. Despite magnetic resonance imaging findings of enhancement of nerve roots and discovery of specific antibodies, the “Landry, Guillain-Barré, Strohl syndrome” (as it should probably be called) to this day, remains mostly a clinical diagnosis as it was when Landry first described it in 1859.

Weakness after foreign travel in a 40-year-old man

The diagnosis of hepatitis E was made from abnormal liver function tests, a recent history of foreign travel and serology (anti-HEV IgM antibodies identified in serum) consistent with acute hepatitis E virus (HEV) infection and could not be verified by imaging. Guillain–Barre syndrome is an acute inflammatory demyelinating polyneuropathy [1–4] first described in 1916 by three French neurologists: Georges Guillain, Jean-Alexandre Barre and Andre Stohl [3]. There are at least four described subtypes, the most common of which is AIDP: acute inflammatory demyelinating polyradiculoneuropathy [3]. The clinical diagnosis is usually established based on clinical criteria, electrophysiological studies and CSF analysis [1]. AIDP is an auto-immune disease postulated to be triggered by a preceding viral or bacterial infection, the most common of which are thought to be Campylobacter jejuni, Epstein–Barr virus (EBV), Mycoplasma pneumoniae and cytomegalovirus (CMV) [5]. Magnetic resonance imaging of the lumbar spine in this case demonstrated diffusely abnormal homogeneous low T1 signal intensity of vertebrae throughout the spine on sagittal T1-weighted imaging, which was related to anaemia. Non-contrast-enhanced imaging revealed no discrete cord abnormality or plaque (see Fig. 1a). Following contrast medium administration there was diffuse enhancement of the conus and cauda equina with enhancement and thickening of the filum terminale (see Fig. 1b). The cauda equina enhances after gadolinium in patients with Guillain–Barre syndrome (GBS) [1] because of a breakdown of the blood–nerve barrier that should be present [4]. Gorson et al. in a prospective evaluation of MRI findings in patients with GBS concluded that prominent enhancement of the cauda equina was 83% sensitive for GBS, present in 95% of typical presentations and was associated with an increased GBS disability score [1]. Byun et al. in a retrospective review of MRI findings in patients with GBS concluded that while enhancement of the intrathecal spinal nerve roots is not specific to GBS, enhancement of the anterior spinal nerve roots in isolation is highly suggestive of GBS [4]. Guillain–Barre syndrome has been described is post hepatitis E infection and it is postulated that this association might be due to molecular mimicry [5]. Molecular mimicry describes the situation where the host mounts an immune response to the infectious organism, in this case HEV, because HEV has epitopes homologous to the host’s own peripheral nerves [5]. The case presentation can be found at doi:10.1007/s00256-010-0905-5

Gadolinium enhancement of the lumbar roots in a case of ALS

We present a single case of clinically definite amyotrophic lateral sclerosis (ALS) associated with modest gadolinium enhancement of the lumbar nerve roots without thickening or nodularity and with CSF protein elevation (80 mg/dl). These findings should not exclude a clinical diagnosis of ALS and suggest that the enhancement of nerve roots and protein elevation were related to rapid neuronal degeneration, not inflammation.