Pearls &amp; Oy-sters: Guillain-Barré syndrome

Ponciano, Ana; Carvalho, João Nuno; Gala, Diana; Leite, José P.; Fernandes, C.

doi:10.1212/wnl.0000000000009838

Cited by 4 publications

(6 citation statements)

References 9 publications

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“…The precise mechanism of acute neuropathic porphyria is poorly understood. However, it is related to porphyrin and porphyrin precursor accumulation [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

“…Both entities can have autonomic dysfunction, making a diagnosis challenging. However, GBS patients have a symmetrical distal weakness with associated CSF evidence of cytoalbumin dissociation [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

“…The hyponatremia is hypothesized to be attributed to SIADH or damage to the supraoptic nucleus of the hypothalamus [ 13 ]. Diagnosis during an acute attack is based on laboratory findings, such as raised urinary and serum porphobilinogen, in the setting of high clinical suspicion [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

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Acute Intermittent Porphyria Labeled Initially As Guillain-Barre Syndrome: Challenging Diagnosis

Alayafi,

Aljumah,

Alluhayyan

et al. 2023

Cureus

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Acute intermittent porphyria (AIP) is an autosomal, dominant, rare metabolic disturbance that results from a defect in the activity of the heme biosynthesis. It has a heterogeneous presentation, making a prompt diagnosis challenging. We report a case of acute intermittent porphyria in a young female who underwent in vitro fertilization (IVF) and presented with recurrent abdominal pain and posterior reversible encephalopathy syndrome (PRES), progressing to acute progressive quadriparesis post-delivery.

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“…The precise mechanism of acute neuropathic porphyria is poorly understood. However, it is related to porphyrin and porphyrin precursor accumulation [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Acute Intermittent Porphyria Labeled Initially As Guillain-Barre Syndrome: Challenging Diagnosis

Alayafi,

Aljumah,

Alluhayyan

et al. 2023

Cureus

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“…The best management of these disorders is preventing acute attacks via identifying and avoiding triggers (Table 2 ). Porphyric neurovisceral attacks are often triggered by stress, alcohol, smoking, fasting or severe carbohydrate-restrictive diets, drugs (especially cytochrome P450-inducing agents), infectious diseases, or cyclical hormonal changes [ 24 ]. Identifying the inciting factor is critical in the prevention of further progression of the disease.…”

Section: Reviewmentioning

confidence: 99%

Acute Intermittent Porphyria: A Review and Rehabilitation Perspective

Valbuena Valecillos,

Yatham,

Alderman

et al. 2023

Cureus

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Acute intermittent porphyria (AIP) is an uncommon metabolic disease that impacts multiple organs and can manifest in many ways. It is often misdiagnosed due to its nonspecific symptoms. Neurovisceral signs and symptoms should alert physicians to consider AIP in the differential after excluding more common causes. Identifying the underlying cause is critical in preventing acute attacks, and trigger avoidance is the optimal approach to managing AIP. Medications that are contraindicated should be reviewed thoroughly. Prompt intravenous hematin administration is the primary treatment for acute attacks, and additional pharmacological therapies may be necessary to treat concurrent symptoms. A severe neurological manifestation of AIP is flaccid paralysis or severe motor weakness, which can develop into total quadriplegia and respiratory insufficiency. A comprehensive rehabilitation program is an integral aspect of the treatment plan. Since the incidence of this disease is low, functional prognosis is not well-known. As a result, it is challenging to determine the most appropriate structure, intensity, and duration of rehabilitation therapy. By extending the treatment plan, individuals with tetraplegia due to AIP can continue to make functional gains years after the onset of weakness. Understanding the disease’s functional prognosis will aid in coordinating resources and improving healthcare expenditures.

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“…Cerebrospinal fluid (CSF) assessment in porphyric neuropathy typically reveals normal cell count, glucose and protein; however, a few cases of elevated CSF protein (up to 100 mg/dL) have been reported. 63,64,71,86,[100][101][102]…”

Section: Cerebrospinal Fluidmentioning

confidence: 99%

Porphyric neuropathy

et al. 2021

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Acute hepatic porphyrias are inherited metabolic disorders that may present with polyneuropathy, which if not diagnosed early can lead to quadriparesis, respiratory weakness, and death. Porphyric neuropathy is an acute to subacute motor predominant axonal neuropathy with a predilection for the upper extremities and usually preceded by a predominantly parasympathetic autonomic neuropathy. The rapid progression and associated dysautonomia mimic Guillain‐Barré syndrome but are distinguished by the absence of cerebrospinal fluid albuminocytologic dissociation, progression beyond 4 wk, and associated abdominal pain. Spot urine test to assess the porphyrin precursors delta‐aminolevulinic acid and porphobilinogen can provide a timely diagnosis during an acute attack. Timely treatment with intravenous heme, carbohydrate loading, and avoidance of porphyrinogenic medications can prevent further neurological morbidity and mortality.

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Pearls & Oy-sters: Guillain-Barré syndrome

Cited by 4 publications

References 9 publications

Acute Intermittent Porphyria Labeled Initially As Guillain-Barre Syndrome: Challenging Diagnosis

Acute Intermittent Porphyria Labeled Initially As Guillain-Barre Syndrome: Challenging Diagnosis

Acute Intermittent Porphyria: A Review and Rehabilitation Perspective

Porphyric neuropathy

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